The Value of Intravenous Immunoglobulin Therapy in Idiopathic Inflammatory Myositis in the Current Transformed Era of Biologics
Identifieur interne : 000940 ( Pmc/Corpus ); précédent : 000939; suivant : 000941The Value of Intravenous Immunoglobulin Therapy in Idiopathic Inflammatory Myositis in the Current Transformed Era of Biologics
Auteurs :Source :
- Cureus [ 2168-8184 ] ; ????.
Abstract
The understanding of etiology and pathogenesis of idiopathic immune myositis is fast evolving, and so is the classification of myositis subtypes. The diversity in genetics, major histocompatibility complex expressions, immunohistochemical, and specific and associated autoantibodies not only explains the individual variability in response to therapies but also begs for subtype-specific treatments. With the evolution of the new biological therapies, the treatment of idiopathic immune myositis (IIM) has greatly transformed in recent years. This article appraises the current therapeutic value of intravenous immunoglobulin (IVIg) in idiopathic immune myopathy patients in the era of transformed treatment options. This article argues why the IVIg therapy still retains its value as an unreplaceable treatment option in certain specific subtypes of idiopathic immune myositis patients as well as in certain specific clinical idiopathic immune myositis scenarios.
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DOI: 10.7759/cureus.7049
PubMed: 32128294
PubMed Central: 7034746
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PMC:7034746Le document en format XML
<record><TEI><teiHeader><fileDesc><titleStmt><title xml:lang="en">The Value of Intravenous Immunoglobulin Therapy in Idiopathic Inflammatory Myositis in the Current Transformed Era of Biologics</title></titleStmt><publicationStmt><idno type="wicri:source">PMC</idno><idno type="pmid">32128294</idno><idno type="pmc">7034746</idno><idno type="url">http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7034746</idno><idno type="RBID">PMC:7034746</idno><idno type="doi">10.7759/cureus.7049</idno><date when="????">????</date><idno type="wicri:Area/Pmc/Corpus">000940</idno><idno type="wicri:explorRef" wicri:stream="Pmc" wicri:step="Corpus" wicri:corpus="PMC">000940</idno></publicationStmt><sourceDesc><biblStruct><analytic><title xml:lang="en" level="a" type="main">The Value of Intravenous Immunoglobulin Therapy in Idiopathic Inflammatory Myositis in the Current Transformed Era of Biologics</title></analytic><series><title level="j">Cureus</title><idno type="eISSN">2168-8184</idno><imprint><date when="????">????</date></imprint></series></biblStruct></sourceDesc></fileDesc><profileDesc><textClass></textClass></profileDesc></teiHeader><front><div type="abstract" xml:lang="en"><p>The understanding of etiology and pathogenesis of idiopathic immune myositis is fast evolving, and so is the classification of myositis subtypes. The diversity in genetics, major histocompatibility complex expressions, immunohistochemical, and specific and associated autoantibodies not only explains the individual variability in response to therapies but also begs for subtype-specific treatments. With the evolution of the new biological therapies, the treatment of idiopathic immune myositis (IIM) has greatly transformed in recent years. This article appraises the current therapeutic value of intravenous immunoglobulin (IVIg) in idiopathic immune myopathy patients in the era of transformed treatment options. This article argues why the IVIg therapy still retains its value as an unreplaceable treatment option in certain specific subtypes of idiopathic immune myositis patients as well as in certain specific clinical idiopathic immune myositis scenarios.</p></div></front><back><div1 type="bibliography"><listBibl><biblStruct><analytic><author><name sortKey="Dalakas, Mc" uniqKey="Dalakas M">MC Dalakas</name></author></analytic></biblStruct><biblStruct><analytic><author><name sortKey="Hornung, T" uniqKey="Hornung T">T Hornung</name></author><author><name sortKey="Wenzel, J" uniqKey="Wenzel J">J Wenzel</name></author></analytic></biblStruct><biblStruct><analytic><author><name sortKey="Roifman, Cm" uniqKey="Roifman C">CM Roifman</name></author><author><name sortKey="Schaffer, Fm" uniqKey="Schaffer F">FM Schaffer</name></author><author><name sortKey="Wachsmuth, Se" uniqKey="Wachsmuth S">SE Wachsmuth</name></author><author><name sortKey="Murphy, G" uniqKey="Murphy G">G Murphy</name></author><author><name sortKey="Gelfand, Ew" uniqKey="Gelfand E">EW Gelfand</name></author></analytic></biblStruct><biblStruct><analytic><author><name sortKey="Toyoda, M" uniqKey="Toyoda M">M Toyoda</name></author><author><name sortKey="Pao, A" uniqKey="Pao A">A Pao</name></author><author><name sortKey="Petrosian, A" uniqKey="Petrosian A">A Petrosian</name></author><author><name sortKey="Jordan, Sc" uniqKey="Jordan S">SC Jordan</name></author></analytic></biblStruct><biblStruct><analytic><author><name sortKey="Samuelsson, A" uniqKey="Samuelsson A">A Samuelsson</name></author><author><name sortKey="Towers, Tl" uniqKey="Towers T">TL Towers</name></author><author><name sortKey="Ravetch, Jv" uniqKey="Ravetch J">JV Ravetch</name></author></analytic></biblStruct><biblStruct><analytic><author><name sortKey="Dalakas, Mc" uniqKey="Dalakas M">MC Dalakas</name></author><author><name sortKey="Illa, I" uniqKey="Illa I">I Illa</name></author><author><name sortKey="Dambrosia, Jm" uniqKey="Dambrosia J">JM Dambrosia</name></author></analytic></biblStruct><biblStruct><analytic><author><name sortKey="Saito, E" uniqKey="Saito E">E Saito</name></author><author><name sortKey="Koike, T" uniqKey="Koike T">T Koike</name></author><author><name sortKey="Hashimoto, H" uniqKey="Hashimoto H">H Hashimoto</name></author></analytic></biblStruct><biblStruct><analytic><author><name sortKey="Cherin, P" uniqKey="Cherin P">P Cherin</name></author><author><name sortKey="Piette, Jc" uniqKey="Piette J">JC Piette</name></author><author><name sortKey="Wechsler, B" uniqKey="Wechsler B">B Wechsler</name></author></analytic></biblStruct><biblStruct><analytic><author><name sortKey="Dalakas, Mc" uniqKey="Dalakas M">MC Dalakas</name></author><author><name sortKey="Koffman, B" uniqKey="Koffman B">B Koffman</name></author><author><name sortKey="Fujii, M" uniqKey="Fujii M">M Fujii</name></author><author><name sortKey="Spector, S" uniqKey="Spector S">S Spector</name></author><author><name sortKey="Sivakumar, K" uniqKey="Sivakumar K">K Sivakumar</name></author><author><name sortKey="Cupler, E" uniqKey="Cupler E">E Cupler</name></author></analytic></biblStruct><biblStruct><analytic><author><name sortKey="Walter, Mc" uniqKey="Walter M">MC Walter</name></author><author><name sortKey="Lochmuller, H" uniqKey="Lochmuller H">H Lochmuller</name></author><author><name sortKey="Toepfer, M" uniqKey="Toepfer M">M Toepfer</name></author></analytic></biblStruct><biblStruct><analytic><author><name sortKey="Recher, M" uniqKey="Recher M">M Recher</name></author><author><name sortKey="Sahrbacher, U" uniqKey="Sahrbacher U">U Sahrbacher</name></author><author><name sortKey="Bremer, J" uniqKey="Bremer J">J Bremer</name></author><author><name sortKey="Arndt, B" uniqKey="Arndt B">B Arndt</name></author><author><name sortKey="Steiner, U" uniqKey="Steiner U">U Steiner</name></author><author><name sortKey="Fontana, A" uniqKey="Fontana A">A Fontana</name></author></analytic></biblStruct><biblStruct><analytic><author><name sortKey="Binns, El" uniqKey="Binns E">EL Binns</name></author><author><name sortKey="Moraitis, E" uniqKey="Moraitis E">E Moraitis</name></author><author><name sortKey="Maillard, S" uniqKey="Maillard S">S Maillard</name></author></analytic></biblStruct><biblStruct><analytic><author><name sortKey="Valiyil, R" uniqKey="Valiyil R">R Valiyil</name></author><author><name sortKey="Casciola Rosen, L" uniqKey="Casciola Rosen L">L Casciola-Rosen</name></author><author><name sortKey="Hong, G" uniqKey="Hong G">G Hong</name></author><author><name sortKey="Mammen, A" uniqKey="Mammen A">A Mammen</name></author><author><name sortKey="Christopher Stine, L" uniqKey="Christopher Stine L">L Christopher-Stine</name></author></analytic></biblStruct><biblStruct><analytic><author><name sortKey="Pinal Fernandez, I" uniqKey="Pinal Fernandez I">I Pinal-Fernandez</name></author><author><name sortKey="Parks, C" uniqKey="Parks C">C Parks</name></author><author><name sortKey="Werner, Jl" uniqKey="Werner J">JL Werner</name></author></analytic></biblStruct><biblStruct><analytic><author><name sortKey="Huber, Am" uniqKey="Huber A">AM Huber</name></author><author><name sortKey="Kim, S" uniqKey="Kim S">S Kim</name></author><author><name sortKey="Reed, Am" uniqKey="Reed A">AM Reed</name></author></analytic></biblStruct><biblStruct><analytic><author><name sortKey="Al Mayouf, Sm" uniqKey="Al Mayouf S">SM Al-Mayouf</name></author><author><name sortKey="Laxer, Rm" uniqKey="Laxer R">RM Laxer</name></author><author><name sortKey="Schneider, R" uniqKey="Schneider R">R Schneider</name></author><author><name sortKey="Silverman, Ed" uniqKey="Silverman E">ED Silverman</name></author><author><name sortKey="Feldman, Bm" uniqKey="Feldman B">BM Feldman</name></author></analytic></biblStruct><biblStruct><analytic><author><name sortKey="Imataka, G" uniqKey="Imataka G">G Imataka</name></author><author><name sortKey="Arisaka, O" uniqKey="Arisaka O">O Arisaka</name></author></analytic></biblStruct><biblStruct><analytic><author><name sortKey="Lam, Cg" uniqKey="Lam C">CG Lam</name></author><author><name sortKey="Manlhiot, C" uniqKey="Manlhiot C">C Manlhiot</name></author><author><name sortKey="Pullenayegum, Em" uniqKey="Pullenayegum E">EM Pullenayegum</name></author><author><name sortKey="Feldman, Bm" uniqKey="Feldman B">BM Feldman</name></author></analytic></biblStruct><biblStruct><analytic><author><name sortKey="Diot, E" uniqKey="Diot E">E Diot</name></author><author><name sortKey="Carmier, D" uniqKey="Carmier D">D Carmier</name></author><author><name sortKey="Marquette, D" uniqKey="Marquette D">D Marquette</name></author><author><name sortKey="Marchand Adam, S" uniqKey="Marchand Adam S">S Marchand-Adam</name></author><author><name sortKey="Diot, P" uniqKey="Diot P">P Diot</name></author><author><name sortKey="Lesire, V" uniqKey="Lesire V">V Lesire</name></author></analytic></biblStruct><biblStruct><analytic><author><name sortKey="Marie, I" uniqKey="Marie I">I Marie</name></author><author><name sortKey="Menard, Jf" uniqKey="Menard J">JF Menard</name></author><author><name sortKey="Hatron, Py" uniqKey="Hatron P">PY Hatron</name></author></analytic></biblStruct><biblStruct><analytic><author><name sortKey="Marie, I" uniqKey="Marie I">I Marie</name></author><author><name sortKey="Hatron, Py" uniqKey="Hatron P">PY Hatron</name></author><author><name sortKey="Dominique, S" uniqKey="Dominique S">S Dominique</name></author><author><name sortKey="Cherin, P" uniqKey="Cherin P">P Cherin</name></author><author><name sortKey="Mouthon, L" uniqKey="Mouthon L">L Mouthon</name></author><author><name sortKey="Menard, Jf" uniqKey="Menard J">JF Menard</name></author></analytic></biblStruct><biblStruct><analytic><author><name sortKey="Kawasumi, H" uniqKey="Kawasumi H">H Kawasumi</name></author><author><name sortKey="Gono, T" uniqKey="Gono T">T Gono</name></author><author><name sortKey="Kawaguchi, Y" uniqKey="Kawaguchi Y">Y Kawaguchi</name></author><author><name sortKey="Yamanaka, H" uniqKey="Yamanaka H">H Yamanaka</name></author></analytic></biblStruct><biblStruct><analytic><author><name sortKey="Hirakata, M" uniqKey="Hirakata M">M Hirakata</name></author><author><name sortKey="Nagai, S" uniqKey="Nagai S">S Nagai</name></author></analytic></biblStruct><biblStruct><analytic><author><name sortKey="Ji, Sy" uniqKey="Ji S">SY Ji</name></author><author><name sortKey="Zeng, Fq" uniqKey="Zeng F">FQ Zeng</name></author><author><name sortKey="Guo, Q" uniqKey="Guo Q">Q Guo</name></author></analytic></biblStruct><biblStruct><analytic><author><name sortKey="Wang, Pz" uniqKey="Wang P">PZ Wang</name></author><author><name sortKey="Guan, Jl" uniqKey="Guan J">JL Guan</name></author><author><name sortKey="Han, Xh" uniqKey="Han X">XH Han</name></author></analytic></biblStruct><biblStruct><analytic><author><name sortKey="Fathi, M" uniqKey="Fathi M">M Fathi</name></author><author><name sortKey="Barbasso Helmers, S" uniqKey="Barbasso Helmers S">S Barbasso Helmers</name></author><author><name sortKey="Lundberg, Ie" uniqKey="Lundberg I">IE Lundberg</name></author></analytic></biblStruct><biblStruct><analytic><author><name sortKey="Suzuki, Y" uniqKey="Suzuki Y">Y Suzuki</name></author><author><name sortKey="Hayakawa, H" uniqKey="Hayakawa H">H Hayakawa</name></author><author><name sortKey="Miwa, S" uniqKey="Miwa S">S Miwa</name></author></analytic></biblStruct><biblStruct><analytic><author><name sortKey="Fujisawa, T" uniqKey="Fujisawa T">T Fujisawa</name></author><author><name sortKey="Suda, T" uniqKey="Suda T">T Suda</name></author><author><name sortKey="Nakamura, Y" uniqKey="Nakamura Y">Y Nakamura</name></author></analytic></biblStruct><biblStruct><analytic><author><name sortKey="Ye, S" uniqKey="Ye S">S Ye</name></author><author><name sortKey="Chen, Xx" uniqKey="Chen X">XX Chen</name></author><author><name sortKey="Lu, Xy" uniqKey="Lu X">XY Lu</name></author></analytic></biblStruct><biblStruct><analytic><author><name sortKey="Gono, T" uniqKey="Gono T">T Gono</name></author><author><name sortKey="Kaneko, H" uniqKey="Kaneko H">H Kaneko</name></author><author><name sortKey="Kawaguchi, Y" uniqKey="Kawaguchi Y">Y Kawaguchi</name></author></analytic></biblStruct><biblStruct><analytic><author><name sortKey="Takada, K" uniqKey="Takada K">K Takada</name></author><author><name sortKey="Nagasaka, K" uniqKey="Nagasaka K">K Nagasaka</name></author><author><name sortKey="Miyasaka, N" uniqKey="Miyasaka N">N Miyasaka</name></author></analytic></biblStruct><biblStruct><analytic><author><name sortKey="Sonies, Bc" uniqKey="Sonies B">BC Sonies</name></author></analytic></biblStruct><biblStruct><analytic><author><name sortKey="Maugars, Ym" uniqKey="Maugars Y">YM Maugars</name></author><author><name sortKey="Berthelot, Jm" uniqKey="Berthelot J">JM Berthelot</name></author><author><name sortKey="Abbas, Aa" uniqKey="Abbas A">AA Abbas</name></author><author><name sortKey="Mussini, Jm" uniqKey="Mussini J">JM Mussini</name></author><author><name sortKey="Nguyen, Jm" uniqKey="Nguyen J">JM Nguyen</name></author><author><name sortKey="Prost, Am" uniqKey="Prost A">AM Prost</name></author></analytic></biblStruct><biblStruct><analytic><author><name sortKey="Mugii, N" uniqKey="Mugii N">N Mugii</name></author><author><name sortKey="Hasegawa, M" uniqKey="Hasegawa M">M Hasegawa</name></author><author><name sortKey="Matsushita, T" uniqKey="Matsushita T">T Matsushita</name></author></analytic></biblStruct><biblStruct><analytic><author><name sortKey="Miller, Lc" uniqKey="Miller L">LC Miller</name></author><author><name sortKey="Michael, Af" uniqKey="Michael A">AF Michael</name></author><author><name sortKey="Kim, Y" uniqKey="Kim Y">Y Kim</name></author></analytic></biblStruct><biblStruct><analytic><author><name sortKey="Mccann, Lj" uniqKey="Mccann L">LJ McCann</name></author><author><name sortKey="Garay, Sm" uniqKey="Garay S">SM Garay</name></author><author><name sortKey="Ryan, Mm" uniqKey="Ryan M">MM Ryan</name></author><author><name sortKey="Harris, R" uniqKey="Harris R">R Harris</name></author><author><name sortKey="Riley, P" uniqKey="Riley P">P Riley</name></author><author><name sortKey="Pilkington, Ca" uniqKey="Pilkington C">CA Pilkington</name></author></analytic></biblStruct><biblStruct><analytic><author><name sortKey="Taieb, A" uniqKey="Taieb A">A Taieb</name></author><author><name sortKey="Guichard, C" uniqKey="Guichard C">C Guichard</name></author><author><name sortKey="Salamon, R" uniqKey="Salamon R">R Salamon</name></author><author><name sortKey="Maleville, J" uniqKey="Maleville J">J Maleville</name></author></analytic></biblStruct><biblStruct><analytic><author><name sortKey="Mccann, Lj" uniqKey="Mccann L">LJ McCann</name></author><author><name sortKey="Juggins, Ad" uniqKey="Juggins A">AD Juggins</name></author><author><name sortKey="Maillard, Sm" uniqKey="Maillard S">SM Maillard</name></author><author><name sortKey="Wedderburn, Lr" uniqKey="Wedderburn L">LR Wedderburn</name></author><author><name sortKey="Davidson, Je" uniqKey="Davidson J">JE Davidson</name></author><author><name sortKey="Murray, Kj" uniqKey="Murray K">KJ Murray</name></author><author><name sortKey="Pilkington, Ca" uniqKey="Pilkington C">CA Pilkington</name></author></analytic></biblStruct><biblStruct><analytic><author><name sortKey="Chiu, Sk" uniqKey="Chiu S">SK Chiu</name></author><author><name sortKey="Yang, Yh" uniqKey="Yang Y">YH Yang</name></author><author><name sortKey="Wang, Lc" uniqKey="Wang L">LC Wang</name></author><author><name sortKey="Chiang, Bl" uniqKey="Chiang B">BL Chiang</name></author></analytic></biblStruct><biblStruct><analytic><author><name sortKey="De Inocencio, J" uniqKey="De Inocencio J">J de Inocencio</name></author><author><name sortKey="Enriquez Merayo, E" uniqKey="Enriquez Merayo E">E Enríquez-Merayo</name></author><author><name sortKey="Gonzalez Granado, Li" uniqKey="Gonzalez Granado L">LI Gonzalez-Granado</name></author><author><name sortKey="Casado, R" uniqKey="Casado R">R Casado</name></author></analytic></biblStruct><biblStruct><analytic><author><name sortKey="Marie, I" uniqKey="Marie I">I Marie</name></author><author><name sortKey="Hachulla, E" uniqKey="Hachulla E">E Hachulla</name></author><author><name sortKey="Levesque, H" uniqKey="Levesque H">H Levesque</name></author></analytic></biblStruct><biblStruct><analytic><author><name sortKey="Ko, Eh" uniqKey="Ko E">EH Ko</name></author><author><name sortKey="Rubin, Ad" uniqKey="Rubin A">AD Rubin</name></author></analytic></biblStruct><biblStruct><analytic><author><name sortKey="Jones, K" uniqKey="Jones K">K Jones</name></author><author><name sortKey="Pitceathly, Rd" uniqKey="Pitceathly R">RD Pitceathly</name></author><author><name sortKey="Rose, Mr" uniqKey="Rose M">MR Rose</name></author><author><name sortKey="Mcgowan, S" uniqKey="Mcgowan S">S McGowan</name></author><author><name sortKey="Hill, M" uniqKey="Hill M">M Hill</name></author><author><name sortKey="Badrising, Ua" uniqKey="Badrising U">UA Badrising</name></author><author><name sortKey="Hughes, T" uniqKey="Hughes T">T Hughes</name></author></analytic></biblStruct><biblStruct><analytic><author><name sortKey="Cherin, P" uniqKey="Cherin P">P Cherin</name></author><author><name sortKey="Belizna, C" uniqKey="Belizna C">C Belizna</name></author><author><name sortKey="Cartry, O" uniqKey="Cartry O">O Cartry</name></author></analytic></biblStruct><biblStruct><analytic><author><name sortKey="Patwa, Hs" uniqKey="Patwa H">HS Patwa</name></author><author><name sortKey="Chaudhry, V" uniqKey="Chaudhry V">V Chaudhry</name></author><author><name sortKey="Katzberg, H" uniqKey="Katzberg H">H Katzberg</name></author><author><name sortKey="Rae Grant, Ad" uniqKey="Rae Grant A">AD Rae-Grant</name></author><author><name sortKey="So, Yt" uniqKey="So Y">YT So</name></author></analytic></biblStruct><biblStruct><analytic><author><name sortKey="Sontheimer, Rd" uniqKey="Sontheimer R">RD Sontheimer</name></author></analytic></biblStruct><biblStruct><analytic><author><name sortKey="Lam, C" uniqKey="Lam C">C Lam</name></author><author><name sortKey="Vleugels, Ra" uniqKey="Vleugels R">RA Vleugels</name></author></analytic></biblStruct><biblStruct><analytic><author><name sortKey="Dalakas, Mc" uniqKey="Dalakas M">MC Dalakas</name></author></analytic></biblStruct><biblStruct><analytic><author><name sortKey="Shahani, L" uniqKey="Shahani L">L Shahani</name></author></analytic></biblStruct><biblStruct><analytic><author><name sortKey="Orandi, Ab" uniqKey="Orandi A">AB Orandi</name></author><author><name sortKey="Baszis, Kw" uniqKey="Baszis K">KW Baszis</name></author><author><name sortKey="Dharnidharka, Vr" uniqKey="Dharnidharka V">VR Dharnidharka</name></author><author><name sortKey="Huber, Am" uniqKey="Huber A">AM Huber</name></author><author><name sortKey="Hoeltzel, Mf" uniqKey="Hoeltzel M">MF Hoeltzel</name></author><author><name sortKey="Carra Juvenile Myositis, Subgroup" uniqKey="Carra Juvenile Myositis S">subgroup CARRA Juvenile Myositis</name></author></analytic></biblStruct></listBibl></div1></back></TEI><pmc article-type="review-article"><pmc-dir>properties open_access</pmc-dir>
<front><journal-meta><journal-id journal-id-type="nlm-ta">Cureus</journal-id><journal-id journal-id-type="iso-abbrev">Cureus</journal-id><journal-id journal-id-type="issn">2168-8184</journal-id><journal-title-group><journal-title>Cureus</journal-title></journal-title-group><issn pub-type="epub">2168-8184</issn><publisher><publisher-name>Cureus</publisher-name><publisher-loc>Palo Alto (CA)</publisher-loc></publisher></journal-meta><article-meta><article-id pub-id-type="pmid">32128294</article-id><article-id pub-id-type="pmc">7034746</article-id><article-id pub-id-type="doi">10.7759/cureus.7049</article-id><article-categories><subj-group subj-group-type="heading"><subject>Pediatrics</subject></subj-group><subj-group><subject>Allergy/Immunology</subject></subj-group><subj-group><subject>Rheumatology</subject></subj-group></article-categories><title-group><article-title>The Value of Intravenous Immunoglobulin Therapy in Idiopathic Inflammatory Myositis in the Current Transformed Era of Biologics</article-title></title-group><contrib-group><contrib contrib-type="editor"><name><surname>Muacevic</surname><given-names>Alexander</given-names></name></contrib><contrib contrib-type="editor"><name><surname>Adler</surname><given-names>John R</given-names></name></contrib></contrib-group><contrib-group><contrib contrib-type="author" corresp="yes"><name><surname>Patwardhan</surname><given-names>Anjali</given-names></name><xref ref-type="aff" rid="aff-173775">1</xref></contrib></contrib-group><aff id="aff-173775"><label>1</label> Child Health, University of Missouri, Columbia, USA</aff><author-notes><corresp id="cor1">Anjali Patwardhan <email>doctoranjali@hotmail.com</email></corresp></author-notes><pub-date date-type="pub" publication-format="electronic"><day>19</day><month>2</month><year>2020</year></pub-date><pub-date date-type="collection" publication-format="electronic"><month>2</month><year>2020</year></pub-date><volume>12</volume><issue>2</issue><elocation-id>e7049</elocation-id><history><date date-type="received"><day>15</day><month>5</month><year>2019</year></date><date date-type="accepted"><day>29</day><month>1</month><year>2020</year></date></history><permissions><copyright-statement>Copyright © 2020, Patwardhan et al.</copyright-statement><copyright-year>2020</copyright-year><copyright-holder>Patwardhan et al.</copyright-holder><license license-type="open-access" xlink:href="http://creativecommons.org/licenses/by/3.0/"><license-p>This is an open access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.</license-p></license></permissions><self-uri xlink:href="https://www.cureus.com/articles/19888-the-value-of-intravenous-immunoglobulin-therapy-in-idiopathic-inflammatory-myositis-in-the-current-transformed-era-of-biologics">This article is available from https://www.cureus.com/articles/19888-the-value-of-intravenous-immunoglobulin-therapy-in-idiopathic-inflammatory-myositis-in-the-current-transformed-era-of-biologics</self-uri><abstract><p>The understanding of etiology and pathogenesis of idiopathic immune myositis is fast evolving, and so is the classification of myositis subtypes. The diversity in genetics, major histocompatibility complex expressions, immunohistochemical, and specific and associated autoantibodies not only explains the individual variability in response to therapies but also begs for subtype-specific treatments. With the evolution of the new biological therapies, the treatment of idiopathic immune myositis (IIM) has greatly transformed in recent years. This article appraises the current therapeutic value of intravenous immunoglobulin (IVIg) in idiopathic immune myopathy patients in the era of transformed treatment options. This article argues why the IVIg therapy still retains its value as an unreplaceable treatment option in certain specific subtypes of idiopathic immune myositis patients as well as in certain specific clinical idiopathic immune myositis scenarios.</p></abstract><kwd-group kwd-group-type="author"><kwd>biologics</kwd><kwd>juvenile dermatomyositis</kwd><kwd>interstitial lung disease</kwd><kwd>rituximab</kwd><kwd>dysphagia</kwd><kwd>idiopathic inflammatory myopathy</kwd><kwd>polymyositis</kwd><kwd>recalcitrant</kwd></kwd-group></article-meta><notes><p content-type="disclaimer">The content published in Cureus is the result of clinical experience and/or research by independent individuals or organizations. Cureus is not responsible for the scientific accuracy or reliability of data or conclusions published herein. All content published within Cureus is intended only for educational, research and reference purposes. Additionally, articles published within Cureus should not be deemed a suitable substitute for the advice of a qualified health care professional. Do not disregard or avoid professional medical advice due to content published within Cureus.</p></notes></front><body><sec sec-type="intro"><title>Introduction and background</title><p>Idiopathic immune myositis (IIM) syndromes are considered complement-mediated microangiopathies that involve small vessels of muscles, skin, and internal organs and cause ischemic damages. Several pieces of evidence, such as the presence of myositis-specific and associated autoantibodies (MAS, MSA), infiltration of tissues with immune cells, and the overexpression of major histocompatibility complex (MHC, class I and II) on myofibrils, point to the autoimmune origin of IIM [<xref rid="REF1" ref-type="bibr">1</xref>]. Recent evidence also points to the inappropriate stimulation of the innate immune system (interferons and IFN-regulated proteins), leading to the dysregulation of the adaptive immune retort through dendritic cells. The interferons and IFN-regulated proteins are believed to have etiopathologic role especially in dermatomyositis (DM) and juvenile dermatomyositis (JDM) [<xref rid="REF2" ref-type="bibr">2</xref>]. Roifman et al. first time reported a drastic improvement in a JDM patient with intravenous immunoglobulin (IVIg) who had failed steroids, methotrexate, and cyclophosphamide therapy [<xref rid="REF3" ref-type="bibr">3</xref>]. The exact mechanism of action of IVIg is still not clear and considered multifactorial. There are several theories on how the IVIg works in myositis patients, such as it acts as immunomodulatory drug/an immune booster, reduces the production of autoantibodies, works through complement fixation, neutralizes the assailant autoantibodies, or autoantigens, causes cytokines suppression or blockage [<xref rid="REF4" ref-type="bibr">4</xref>]. IVIg is known to inhibit IL-2, IL-10, TNF-𝛽, and IFN-𝛾, derived from T-cells. The IVIg may effect through its suppressing actions on the dendritic cells and their maturation [<xref rid="REF4" ref-type="bibr">4</xref>]. It blocks the Fc-receptors on autoantibodies, therefore, prevents the phagocytosis of antibody-coated cells [<xref rid="REF5" ref-type="bibr">5</xref>].</p><p>The anti-inflammatory effect of IVIg on autoantibody-induced inflammation may be due to its ability to induce expression of the inhibitory Fc and Fc- y- RIIB receptors. Apart from all the previously mentioned actions, IVIg also has an immediate and long-lasting attenuating effect on complement amplification by stimulating inactivation of C3 convertase precursors [<xref rid="REF5" ref-type="bibr">5</xref>].</p><p>The utility of IVIg therapy is especially found unparalleled in situations where immunosuppressants are contraindicated, such as pregnancy and fulminant infections. IVIg has been selectively used in some specific clinical situations and has shown comparative therapeutic superiority over other therapeutic agents such as myositis with lung and esophageal involvement, as detailed later in this article. The safety profile and low adverse effects of IVIg, as compared to other immunosuppressants and biologics, made it popular drug in the treatment of IIM, despite the rising costs, supply shortages and still not being FDA-approved therapy for myositis. Most immunosuppressants except the pulse steroids need a latent period before the clinical effect can be seen. The IVIg and cyclophosphamide are the drugs that need a variable but relatively short, i.e., in weeks rather than in months, latent period for clinical results. This article explores the utility and current value of IVIg in patients with myositis.</p></sec><sec sec-type="review"><title>Review</title><p>Efficacy and use of IVIg in IIM</p><p>Other than steroids, high dose IVIg is the only drug that is researched and found to be effective for the treatment of IIM in a double-blind and placebo-controlled trial [<xref rid="REF6" ref-type="bibr">6</xref>]. Several studies report the successful use of IVIg in different subgroups of IIM in diverse clinical settings as listed in the following section.</p><p>Two randomized controlled trials (RCT) and several prospective uncontrolled studies have reported the successful use of high dose IVIg in DM and polymyositis (PM) patients who had failed the therapy with steroids and at least one disease-modifying anti-rheumatic drug (DMARD) [<xref rid="REF7" ref-type="bibr">7</xref>, <xref rid="REF8" ref-type="bibr">8</xref>]. The response and efficacy of high dose IVIg in inclusion body myositis (IBM) patients are not well established [<xref rid="REF9" ref-type="bibr">9</xref>]. In the controlled cross-over design double-blind, placebo-controlled study, the sporadic inclusion body myositis (s-IBM) patients showed only marginal clinical improvement with high dose IVIg [<xref rid="REF10" ref-type="bibr">10</xref>]. One case report of successfully treating IBM patients with the low dose IVIg begs further exploration in low dose therapy [<xref rid="REF11" ref-type="bibr">11</xref>]. Binns et al. report a good response to IVIg with rituximab and cyclophosphamide in a three anti-signal recognition particle-associated JDM (anti-SRP JDM) patients [<xref rid="REF12" ref-type="bibr">12</xref>]. The positive response to rituximab in anti-SRP JDM patients is already recognized. Therefore, the contribution of IVIg in the combined success when used with rituximab is difficult to assess [<xref rid="REF13" ref-type="bibr">13</xref>]. It is worth mentioning that anti-SRP JDM, along with anti-HMG-CoA-reductase (anti-HMGCR) JDM, is a distinct and severe form of necrotizing muscle predominant and resistant to treat JDM [<xref rid="REF14" ref-type="bibr">14</xref>]. The IVIg is particularly favored and has shown significant success in skin predominant and or resistant to treat skin diseases in JDM patients [<xref rid="REF15" ref-type="bibr">15</xref>]. Several researchers have reported significant response to high dose IVIg in their refractory/steroid-resistant/steroid-dependent classic JDM patients [<xref rid="REF16" ref-type="bibr">16</xref>]. Imataka and Arisaka reported successful treatment of a steroid-refractory Banker-type JDM in two-year-old patients with IVIg (400 mg/kg/five days a week/six week for six cycles) [<xref rid="REF17" ref-type="bibr">17</xref>]. In the largest cohort that received IVIg, Lam et al. reported that IVIg showed better results in steroid-resistant patients than in steroid-dependent patients, but steroid-dependent patients showed lower disease activity than the control JDM patients who did not receive IVIg. They also reported the best response in SR JDM patients [<xref rid="REF18" ref-type="bibr">18</xref>]. Al-Mayouf et al. reported a steroid-sparing effect in their 18-steroid dependent JDM patients who received treatment with IVIg [<xref rid="REF16" ref-type="bibr">16</xref>]. In modern medicine, the use of IVIg is well recognized and prevalent as a second/third line conventional therapy for IIM.</p><p>The experience is wide-ranging when IVIg is used as first-line therapy. Some researchers believe that IVIg should be used as first-line therapy in DM/PM-ILD (interstitial lung disease) patients, while others used effectively in refractory patients [<xref rid="REF19" ref-type="bibr">19</xref>-<xref rid="REF21" ref-type="bibr">21</xref>]. The higher medication cost, as well as risk for exposure to blood-borne diseases, is the prohibiting factors for the use of IVIg as the first-line therapy. The cumulative cost vs. comparative quality analysis (repeat therapies, hospital stay, work/school days lost, quality of life, the dollar value of the therapy) of various therapeutic options for myositis is not performed. The following are some of the experiences where IVIg was used as first-line therapy. IVIg as first-line therapy has been reported to be unsuccessful in an open-label study of 11 patients (5/11 PM and 6/11 DM) [<xref rid="REF8" ref-type="bibr">8</xref>]. Diot et al. and few other researchers recommend IVIg as first-line therapy in myositis patients but along with other immunosuppressants, more so if the patient has interstitial lung disease (ILD) and or dysphagia also [<xref rid="REF19" ref-type="bibr">19</xref>-<xref rid="REF21" ref-type="bibr">21</xref>]. Some researchers recommend IVIg as first-line therapy in patients with esophageal involvement [<xref rid="REF20" ref-type="bibr">20</xref>].</p><p>There are some studies where IVIg is used in ILD-associated PM/DM. ILD is considered as a poor prognostic factor and predictor of high mortality despite aggressive therapy in patients with myositis [<xref rid="REF21" ref-type="bibr">21</xref>]. It is recognized now that the interstitial inflammation may be part of the initial disease process, which may go undetected if not careful. Certain factors such as anti-MDA5 antibody positivity and hyperferritinemia are not only the risk factors for developing ILD but also predict its expansion to rapidly progressive ILD [<xref rid="REF22" ref-type="bibr">22</xref>]. The manifestations, diseases course, pathophysiology, immunologic profiles, histopathology findings and outcomes of ILD have been heterogeneous and different in subgroups of IIM suggesting variations in immunopathogenic mechanisms [<xref rid="REF23" ref-type="bibr">23</xref>]. High/persistent fever at presentation (known as Hamman-Rich-like presentation), hypoalbuminemia, cardiac involvement, amyopathic diseases with interstitial pneumonia, are bad prognostic factors for development and progression of PM/DM-associated ILD [<xref rid="REF24" ref-type="bibr">24</xref>]. The presence of ILD which also correlates with the severity of Gottron's papules/rash, cardiac involvement, the age of onset at 40 years or above, arthralgia, pulmonary fibrosis, and fevers are considered poor diseases prognostic factors in all the subgroups of IIM (JDM/DM/PM) [<xref rid="REF25" ref-type="bibr">25</xref>].</p><p>Some serologic markers such as serum KL-6 level, serum surfactant protein-D (SP-D), serum surfactant protein-A (SP-A), combined SP-D and SP-A, may be potentially useful serum markers for the diagnosis of ILD in PM/DM patients.</p><p>The serum SP-A is a prognostic factor, while serial serum KL-6 levels are also used to monitor response to the therapy in PM/DM-ILD in patients [<xref rid="REF26" ref-type="bibr">26</xref>]. Suzuki et al. described their retrospective experience (1985-2007) with five PM/DM/amyopathic dermatomyositis (AMD) patients (one PM, four ADM with one positive for anti-Jo-antibody, three females, two males) with severe resistant to treat ILD with similar clinical courses, parameters, and demography. These five patients were treated with IVIg after they had failed conventional therapy, including cyclosporine and cyclophosphamide. They had a total of 57 patients admitted with PM/DM with ILD during the said period, but only five who did not respond to high dose steroids and one more immunosuppressant were labeled as ‘resistant’ and were treated with IVIg as ‘salvage therapy.’ Only 2/5 patients (one PM, one ADM) survived [<xref rid="REF27" ref-type="bibr">27</xref>]. Fujisawa et al., in their series of 28 patients (16 PM-ILD, 12 DM-ILD) identified that ILD-DM patients are more likely to be corticosteroid-refractory than ILD-PM and have a worse prognosis [<xref rid="REF28" ref-type="bibr">28</xref>]. Ye et al., in their series of 145 patients (1998-2005), not only confirmed findings of Fujisawa et al. but also noted that the worst outcome was in AMD-ILD followed by DM-ILD and the best outcome amongst the three subgroups was in PM-ILD [<xref rid="REF29" ref-type="bibr">29</xref>]. The anti-melanoma differentiation-associated gene five antibodies (anti-MDA5) positive patients are reported to have a rapidly progressing disease course followed by death [<xref rid="REF22" ref-type="bibr">22</xref>]. The anti-aminoacyl-tRNA synthetase (anti-ARS) positive patients respond to corticosteroids early in the diseases, but then frequently relapse and run a progressive course and finally may become refractory to steroid therapy over time [<xref rid="REF22" ref-type="bibr">22</xref>]. The anti-ARS antibody-positive patients comparatively do better than anti-MDA5 positive patients [<xref rid="REF22" ref-type="bibr">22</xref>]. The difference in various ratios of IL-4, IFN-γ, and serum IL-8 levels is believed to be the cause of pathophysiologic differences in anti-ARS-ILD and anti-MDA5-ILD [<xref rid="REF30" ref-type="bibr">30</xref>].</p><p>The early recognition and appropriate selection of the correct subtype of the patient are vital to select appropriate therapeutic strategies.</p><p>Apart from IVIg, the cyclosporine and tacrolimus had been used selectively successfully in ILD+PM/DM patients [<xref rid="REF31" ref-type="bibr">31</xref>].</p><p>IVIg in dysphagia associated with myositis</p><p>As a part of systemic myositis, the cricopharyngeal, oropharyngeal, sternomastoid muscle, and esophageal muscles may also get involved leading to difficulty in swallowing, achalasia, aspiration, voice change and associated complications [<xref rid="REF32" ref-type="bibr">32</xref>]. The dysphagia occurs in a wide range of IIM patients (10-73%) and can contribute to a poor outcome and high mortality [<xref rid="REF33" ref-type="bibr">33</xref>]. The new development of dysphagia can either suggest the progression of disease relapse.</p><p>Mugii et al., in their 92 adult Japanese DM patients, reported that the positive anti-TIF-1γ antibody, malignancy-comorbidity, higher age, male sex, lower mean MMT scores of sternomastoid muscle, and interstitial pneumonia are strongly associated with the likelihood of developing dysphagia in adult myositis patients. These all risk factors are independently also considered as bad prognostic features [<xref rid="REF34" ref-type="bibr">34</xref>].</p><p>The prevalence of dysphagia in JDM is not precisely reported as extensively as in adults. There are technological barriers in the interpretation of the videofluoroscopic swallowing study (VFSS) in children. Despite the paucity of data, most researchers agree that dysphagia in childhood PM/JDM is a worse prognostic marker and associated with a higher mortality rate [<xref rid="REF35" ref-type="bibr">35</xref>]. As opposed to adults, researchers have yet not identified any correlation between VFSS scores and other disease activity indices (MMT/CMAS, physician VAS, CHAQ) in pediatric myositis patients. Another difference in pediatric myositis was that the clinical and laboratory markers could not predict the abnormal swallowing scores by VFSS, suggesting that dysphagia and rest of the JDM disease activity may not run hand in hand [<xref rid="REF36" ref-type="bibr">36</xref>].</p><p>Taieb et al., in their 70 JDM patient series, concluded that dysphagia was independently a worse prognostic factor, and patients with dysphagia had higher mortality rates than those without [<xref rid="REF37" ref-type="bibr">37</xref>]. In the UK and Ireland National Registry/Repository for JDM, 33/114 (29%) patients had symptomatic dysphagia, and 22/126 (17%) had symptomatic dysphonia [<xref rid="REF38" ref-type="bibr">38</xref>]. Chiu et al. reported dysphagia in 19% of their series of 32 JDM patients [<xref rid="REF39" ref-type="bibr">39</xref>]. In McCann’s series of 14 JDM patients, two asymptomatic patients had abnormal, and three symptomatic patients had normal VFSS. De Inocencio et al. reported reduced side effects and better tolerability and effectiveness of subcutaneous (SC) immune globulins with high-dose recombinant human hyaluronidase. The experience comes from their five juvenile dermatomyositis patients who could not tolerate high dose IVIg due to side effects such as nausea, vomiting, severe headaches, or who had poor venous access. They have reported comparable serum IgG levels after SC IVIg with dose adjustments (fSCIg 1 g/kg on days 1 and 6 of every 28-day cycle) [<xref rid="REF40" ref-type="bibr">40</xref>].</p><p>Marie at al., in the retrospective chart review of 73 adult refractory myositis patients (39/73 PM, 34/73 DM) with dysphagia, reported their experience with high dose (2 g/kg/dose/month) IVIg therapy. They not only reported good outcomes but also recommend the combination of IVIg and high-dose steroids as the first-line therapy in life-threatening cases of dysphagia [<xref rid="REF19" ref-type="bibr">19</xref>]. Earlier, Marie et al. had reported a 100% success and remission in their three severely dysphagic steroid-resistant DM/PM patients with three doses of IVIg [<xref rid="REF41" ref-type="bibr">41</xref>]. Several reports confirm the sustained response to high dose IVIg in patients with PM/DM dysphagia [<xref rid="REF41" ref-type="bibr">41</xref>]. In these reports, the IVIg was used successfully with or without other DMARDs, methylprednisolone and oral steroids in DM/PM patients who were poor steroid responders, steroid-refractory or dependent and had rapidly progressive life-threatening dysphagia.</p><p>Unlike other IIM, dysphagia in IBM is more common and resistant to steroid therapy, which makes it difficult to treat [<xref rid="REF42" ref-type="bibr">42</xref>]. Experience with IVIg is varied amongst different researchers. Jones et al. performed a detailed Cochrane review and concluded that there is no significant value of using IVIg in IBM patients with dysphagia [<xref rid="REF43" ref-type="bibr">43</xref>]. In a double-blind, placebo-controlled crossover study in 19 IBM patients with dysphagia (age 42-74 years) with average disease duration of 5.6 years (range 3-10 years), the patients received monthly infusions of 2 g/kg IVIg/placebo for three months. Though the study failed to show a muscle strength improvement in the treatment arm, a mild improvement in swallowing duration (in seconds) measured with ultrasound was reported in the treatment arm (p < 0.05) [<xref rid="REF3" ref-type="bibr">3</xref>]. Cherin et al. showed sustained improvement in dysphagia with subcutaneous Ig (SCIg) in their six patients with IBM, though relapses were frequent. The longest duration of sustained improvement in their series was 12 months [<xref rid="REF44" ref-type="bibr">44</xref>].</p><p>Summary of IVIg therapy in IIM</p><p>Except for IBM, IVIg therapy is considered a safe and effective therapy for most subgroups of IIM patients. It is known to produce relatively early and sustained improvement, especially in DM/JDM patients. IVIg is especially useful with patients with ILD, lung involvement, skin-predominant disease, in the setting of infection, and in patients with swallowing difficulties. The usual dose is 2 g/kg/month, but it has been used variably by different researchers. Promising results are reported even after low dose as well as high dose subcutaneous IVIg when given intermittently or through a programmable pump [<xref rid="REF44" ref-type="bibr">44</xref>]. Significant improvements in muscle strengths, muscle enzyme levels, quality of life and steroid-sparing effect have been reported in most DM/JDM patients who were considered refractory. The guidelines of the American Academy of Neurology (2012) suggest that there is no conclusive evidence to support the use of IVIg in polymyositis patients [<xref rid="REF45" ref-type="bibr">45</xref>]. It is an expensive drug that prohibits its use as a first- or second-line therapy or even as a long-term therapy. Currently, the IVIg commonly is utilized as salvage therapy in refractory patients, but its early use in the specific clinical scenarios such as myositis with ILD, myositis with dysphagia, myositis with sepsis, and skin predominant refractory disease should be encouraged.</p><p>Some researchers recommend IVIg therapy as first-line therapy in viral or drug-induced myopathy which closely resembles inflammatory myopathy, such as due to mycoplasma and several viruses-induced myopathies [<xref rid="REF8" ref-type="bibr">8</xref>].</p><p>IVIg in skin predominant IIM</p><p>The skin component of DM or JDM could be more recalcitrant to treatment than that of muscle disease and can have a severe disability, morbidity, and quality of life (QoL) issues [<xref rid="REF46" ref-type="bibr">46</xref>]. The skin disease could be in pre-muscle disease, post- muscle disease, predominant skin disease, or skin only dermatomyositis/JDM (amyotrophic myositis). Aggressive sun protection (more than 70% SPF) is universally advised along with other therapies. The topical approaches such as antipruritic agents, tacrolimus ointment, steroid ointments and oral antimalarial (hydroxychloroquine 5 mg/kg/day, maximum 400 mg, quinacrine, and chloroquine) are added to the systemic therapy as add-on or sole therapies based on the severity of diseases as measured by the Cutaneous Dermatomyositis Disease Area and Severity Index (CDASI: 0 to 100). The second line treatment includes DMARDs such as methotrexate (MTX) at the dose of 15 mg/m<sup>2</sup> or 1 mg/kg once/week, maximum 40 mg, mycophenolate mofetil (MMF) at the dose of 600 mg/m<sup>2</sup>, or azathioprine (AZA) at the dose of 1 to 2 mg/kg/day. The cyclosporine is used as a bridge short-term treatment. The intravenous immunoglobulin (IVIg) therapy (2 g/kg/day spread over 2-5 days) is reserved for the refractory skin diseases myositis patients. Therapies such as dapsone and thalidomide are considered in exceptional circumstances weighing the risk vs. benefits. The rituximab has been used if the patient has failed all the above-listed medications. Anecdotal cases of treatment of recalcitrant cutaneous diseases in myositis by total body irradiation, stem cell transplantation, and plasmapheresis have been reported [<xref rid="REF47" ref-type="bibr">47</xref>].</p><p>IVIG reduces the concentration of MAC deposits in the skin as well as myofibril capillaries, downregulates T cells, reduces the adhesion molecules expression (ICAM-I, VCAM, TGF-βand MHC-I molecules) and down-regulates cytokine production, in myositis patients [<xref rid="REF48" ref-type="bibr">48</xref>]. The IVIG treatment is effective both for the skin as well as muscle disease in DM, JDM, and some polymyositis patients.</p><p>Value of IVIg in myositis calcinosis cutis and calcinosis</p><p>Abnormal deposition of calcium salts in skin, subcutaneous tissue (calcinosis cutis), and muscles is called calcinosis, which can be dystrophic, idiopathic, iatrogenic, transplant-related, tumoral calcinosis or metastatic. The calcinosis in the presence of normal serum calcium and phosphate levels is called dystrophic calcinosis.</p><p>The jury is still out for the answers if calcinosis is a part/signature of the myositis diseases itself or occurs as a result of the nonspecific chronic micro-injury and inflammation. There is no structured trial, but several reports support the value of IVIg in IIM patients with calcinosis.</p><p>Shahani reports successfully treating a 30-year-old patient with DM with painful calcinosis who failed steroid, and several DMARDs and responded to 2 gm/kg/month-IVIg over two consecutive days [<xref rid="REF49" ref-type="bibr">49</xref>]. A survey on pediatric rheumatologist suggested that just over 1/4th rheumatologists perform initial screening imaging for calcinosis in newly diagnosed IIM patients. Sixty-seven percent of survey responders preferred to step up the immunosuppression as the first step for calcinosis, 13% did not do something specific, but continue with the ongoing therapy for IIM, 13% add other ‘non-immunosuppressive’ therapies. According to a survey of rheumatologist by Childhood Arthritis and Rheumatology Research Alliance (CARRA), the changes in immune therapy that were made after the diagnosis of calcinosis were: 57% rheumatologists added IVIg, 48% added pulse steroids, 40% added methotrexate if the patient was already not on it, 25% survey responders added TNF-alpha inhibitors, 19% added rituximab, 11% added abatacept, and 12% used other immunosuppressants (anakinra, cyclophosphamide, lenalidomide, sirolimus). Of all the survey responders, 32% believed pulse steroids as a most successful option, 30% believed that IVIg was the most successful treatment option, and 29% believed IVIg was the second-best (after steroids) treatment option. The non-immunosuppressant treatment options by frequency of use included bisphosphonates (73%), calcium channel blockers (43%), topical formulation (14%), sodium thiosulfate (10%) and other agents such as warfarin, minocycline (4%) [<xref rid="REF50" ref-type="bibr">50</xref>].</p></sec><sec sec-type="conclusions"><title>Conclusions</title><p>The understanding of the etiology and pathogenesis of idiopathic immune myositis is fast evolving, and so is the classification of myositis subtypes. The diversity in genetics, major histocompatibility complex expressions, immunohistochemical, and specific and associated autoantibodies begs for subtype-specific treatment options for specific subtypes of IIM. The diversity in subtypes of IIM also explains the individual variability in response to specific therapies. The recent advances in biological therapies have made the notion of subtype-specific therapy option a possibility. Despite all these new advances in the knowledge and understanding of IIM and the novel biologics therapies, IVIg remains relevant, valid, unreplaceable treatment option in the management of certain subtype of IIM patients and certain specific clinical scenarios. It is an effective therapy in infection-associated myositis, IIM with dysphagia/dysphonia/IIM-associated ILD, refractory IIM and IIM with dystrophic calcinosis. Its utility in refractory IIM skin diseases has been long established. Apart from the efficacy, the tolerability and safety profile of IVIg remains unmatched to date.</p></sec></body><back><fn-group content-type="competing-interests"><fn fn-type="COI-statement"><p>The authors have declared that no competing interests exist.</p></fn></fn-group><ref-list><title>References</title><ref id="REF1"><label>1</label><element-citation publication-type="journal"><article-title>Pathophysiology of inflammatory and autoimmune myopathies</article-title><source>Presse Med</source><person-group><name><surname>Dalakas</surname><given-names>MC</given-names></name></person-group><fpage>237</fpage><lpage>247</lpage><volume>40</volume><year>2011</year></element-citation></ref><ref id="REF2"><label>2</label><element-citation publication-type="journal"><article-title>Innate immune-response mechanisms in dermatomyositis: an update on pathogenesis, diagnosis and treatment</article-title><source>Drugs</source><person-group><name><surname>Hornung</surname><given-names>T</given-names></name><name><surname>Wenzel</surname><given-names>J</given-names></name></person-group><fpage>981</fpage><lpage>998</lpage><volume>74</volume><year>2014</year><pub-id pub-id-type="pmid">24939511</pub-id></element-citation></ref><ref id="REF3"><label>3</label><element-citation publication-type="journal"><article-title>Reversal of chronic polymyositis following intravenous immune serum globulin therapy</article-title><source>JAMA</source><person-group><name><surname>Roifman</surname><given-names>CM</given-names></name><name><surname>Schaffer</surname><given-names>FM</given-names></name><name><surname>Wachsmuth</surname><given-names>SE</given-names></name><name><surname>Murphy</surname><given-names>G</given-names></name><name><surname>Gelfand</surname><given-names>EW</given-names></name></person-group><fpage>513</fpage><lpage>515</lpage><volume>258</volume><year>1987</year><pub-id pub-id-type="pmid">3599350</pub-id></element-citation></ref><ref id="REF4"><label>4</label><element-citation publication-type="journal"><article-title>Pooled human gammaglobulin modulates surface molecule expression and induces apoptosis in human B cells</article-title><source>Am J Transplant</source><person-group><name><surname>Toyoda</surname><given-names>M</given-names></name><name><surname>Pao</surname><given-names>A</given-names></name><name><surname>Petrosian</surname><given-names>A</given-names></name><name><surname>Jordan</surname><given-names>SC</given-names></name></person-group><fpage>156</fpage><lpage>166</lpage><volume>3</volume><year>2003</year><pub-id pub-id-type="pmid">12603211</pub-id></element-citation></ref><ref id="REF5"><label>5</label><element-citation publication-type="journal"><article-title>Anti-inflammatory activity of IVIG mediated through the inhibitory Fc receptor</article-title><source>Science</source><person-group><name><surname>Samuelsson</surname><given-names>A</given-names></name><name><surname>Towers</surname><given-names>TL</given-names></name><name><surname>Ravetch</surname><given-names>JV</given-names></name></person-group><fpage>484</fpage><lpage>486</lpage><volume>291</volume><year>2001</year><pub-id pub-id-type="pmid">11161202</pub-id></element-citation></ref><ref id="REF6"><label>6</label><element-citation publication-type="journal"><article-title>A controlled trial of high-dose intravenous immune globulin infusions as treatment for dermatomyositis</article-title><source>N Engl J Med</source><person-group><name><surname>Dalakas</surname><given-names>MC</given-names></name><name><surname>Illa</surname><given-names>I</given-names></name><name><surname>Dambrosia</surname><given-names>JM</given-names></name><etal></etal></person-group><fpage>1993</fpage><lpage>2000</lpage><volume>329</volume><year>1993</year><pub-id pub-id-type="pmid">8247075</pub-id></element-citation></ref><ref id="REF7"><label>7</label><element-citation publication-type="journal"><article-title>Efficacy of high-dose intravenous immunoglobulin therapy in Japanese patients with steroid-resistant polymyositis and dermatomyositis</article-title><source>Mod Rheumatol</source><person-group><name><surname>Saito</surname><given-names>E</given-names></name><name><surname>Koike</surname><given-names>T</given-names></name><name><surname>Hashimoto</surname><given-names>H</given-names></name><etal></etal></person-group><fpage>34</fpage><lpage>44</lpage><volume>18</volume><year>2008</year><pub-id pub-id-type="pmid">18217197</pub-id></element-citation></ref><ref id="REF8"><label>8</label><element-citation publication-type="journal"><article-title>Intravenous gamma globulin as first line therapy in polymyositis and dermatomyositis: an open study in 11 adult patients</article-title><source>J Rheumatol</source><person-group><name><surname>Cherin</surname><given-names>P</given-names></name><name><surname>Piette</surname><given-names>JC</given-names></name><name><surname>Wechsler</surname><given-names>B</given-names></name><etal></etal></person-group><fpage>1092</fpage><lpage>1097</lpage><volume>21</volume><year>1994</year><uri xlink:href="https://europepmc.org/abstract/med/7932419">https://europepmc.org/abstract/med/7932419</uri><pub-id pub-id-type="pmid">7932419</pub-id></element-citation></ref><ref id="REF9"><label>9</label><element-citation publication-type="journal"><article-title>A controlled study of intravenous immunoglobulin combined with prednisone in the treatment of IBM</article-title><source>Neurology</source><person-group><name><surname>Dalakas</surname><given-names>MC</given-names></name><name><surname>Koffman</surname><given-names>B</given-names></name><name><surname>Fujii</surname><given-names>M</given-names></name><name><surname>Spector</surname><given-names>S</given-names></name><name><surname>Sivakumar</surname><given-names>K</given-names></name><name><surname>Cupler</surname><given-names>E</given-names></name></person-group><fpage>323</fpage><lpage>327</lpage><volume>56</volume><year>2001</year><pub-id pub-id-type="pmid">11171896</pub-id></element-citation></ref><ref id="REF10"><label>10</label><element-citation publication-type="journal"><article-title>High-dose immunoglobulin therapy in sporadic inclusion body myositis: a double-blind, placebo-controlled study</article-title><source>J Neurol</source><person-group><name><surname>Walter</surname><given-names>MC</given-names></name><name><surname>Lochmuller</surname><given-names>H</given-names></name><name><surname>Toepfer</surname><given-names>M</given-names></name><etal></etal></person-group><fpage>22</fpage><lpage>28</lpage><volume>247</volume><year>2000</year><uri xlink:href="https://link.springer.com/article/10.1007/s004150050005">https://link.springer.com/article/10.1007/s004150050005</uri><pub-id pub-id-type="pmid">10701893</pub-id></element-citation></ref><ref id="REF11"><label>11</label><element-citation publication-type="journal"><article-title>Treatment of inclusion body myositis: is low-dose intravenous immunoglobulin the solution?</article-title><source>Rheumatol Int</source><person-group><name><surname>Recher</surname><given-names>M</given-names></name><name><surname>Sahrbacher</surname><given-names>U</given-names></name><name><surname>Bremer</surname><given-names>J</given-names></name><name><surname>Arndt</surname><given-names>B</given-names></name><name><surname>Steiner</surname><given-names>U</given-names></name><name><surname>Fontana</surname><given-names>A</given-names></name></person-group><fpage>469</fpage><lpage>472</lpage><volume>32</volume><year>2012</year><pub-id pub-id-type="pmid">20044785</pub-id></element-citation></ref><ref id="REF12"><label>12</label><element-citation publication-type="journal"><article-title>Effective induction therapy for anti-SRP associated myositis in childhood: a small case series and review of the literature</article-title><source>Pediatr Rheumatol</source><person-group><name><surname>Binns</surname><given-names>EL</given-names></name><name><surname>Moraitis</surname><given-names>E</given-names></name><name><surname>Maillard</surname><given-names>S</given-names></name><etal></etal></person-group><fpage>77</fpage><volume>15</volume><year>2017</year></element-citation></ref><ref id="REF13"><label>13</label><element-citation publication-type="journal"><article-title>Rituximab therapy for myopathy associated with anti-signal recognition particle antibodies: a case series</article-title><source>Arthritis Care Res</source><person-group><name><surname>Valiyil</surname><given-names>R</given-names></name><name><surname>Casciola-Rosen</surname><given-names>L</given-names></name><name><surname>Hong</surname><given-names>G</given-names></name><name><surname>Mammen</surname><given-names>A</given-names></name><name><surname>Christopher-Stine</surname><given-names>L</given-names></name></person-group><fpage>1328</fpage><lpage>1334</lpage><volume>62</volume><year>2010</year></element-citation></ref><ref id="REF14"><label>14</label><element-citation publication-type="journal"><article-title>Longitudinal course of disease in a large cohort of myositis patients with autoantibodies recognizing the signal recognition particle</article-title><source>Arthritis Care Res</source><person-group><name><surname>Pinal-Fernandez</surname><given-names>I</given-names></name><name><surname>Parks</surname><given-names>C</given-names></name><name><surname>Werner</surname><given-names>JL</given-names></name><etal></etal></person-group><fpage>263</fpage><lpage>270</lpage><volume>69</volume><year>2017</year></element-citation></ref><ref id="REF15"><label>15</label><element-citation publication-type="journal"><article-title>Childhood arthritis and rheumatology research alliance consensus clinical treatment plans for juvenile dermatomyositis with persistent skin rash</article-title><source>J Rheumatol</source><person-group><name><surname>Huber</surname><given-names>AM</given-names></name><name><surname>Kim</surname><given-names>S</given-names></name><name><surname>Reed</surname><given-names>AM</given-names></name><etal></etal></person-group><fpage>110</fpage><lpage>116</lpage><volume>44</volume><year>2017</year><pub-id pub-id-type="pmid">27803135</pub-id></element-citation></ref><ref id="REF16"><label>16</label><element-citation publication-type="journal"><article-title>Intravenous immunoglobulin therapy for juvenile dermatomyositis: efficacy and safety</article-title><source>J Rheumatol</source><person-group><name><surname>Al-Mayouf</surname><given-names>SM</given-names></name><name><surname>Laxer</surname><given-names>RM</given-names></name><name><surname>Schneider</surname><given-names>R</given-names></name><name><surname>Silverman</surname><given-names>ED</given-names></name><name><surname>Feldman</surname><given-names>BM</given-names></name></person-group><fpage>2498</fpage><lpage>2503</lpage><volume>27</volume><year>2000</year><uri xlink:href="https://europepmc.org/abstract/med/11036850">https://europepmc.org/abstract/med/11036850</uri><pub-id pub-id-type="pmid">11036850</pub-id></element-citation></ref><ref id="REF17"><label>17</label><element-citation publication-type="journal"><article-title>Long-term, high-dose intravenous immunoglobulin therapy in a patient with banker-type juvenile dermatomyositis</article-title><source>Cell Biochem Biophys</source><person-group><name><surname>Imataka</surname><given-names>G</given-names></name><name><surname>Arisaka</surname><given-names>O</given-names></name></person-group><fpage>747</fpage><lpage>748</lpage><volume>69</volume><year>2014</year><pub-id pub-id-type="pmid">24500836</pub-id></element-citation></ref><ref id="REF18"><label>18</label><element-citation publication-type="journal"><article-title>Efficacy of intravenous Ig therapy in juvenile dermatomyositis</article-title><source>Ann Rheum Dis</source><person-group><name><surname>Lam</surname><given-names>CG</given-names></name><name><surname>Manlhiot</surname><given-names>C</given-names></name><name><surname>Pullenayegum</surname><given-names>EM</given-names></name><name><surname>Feldman</surname><given-names>BM</given-names></name></person-group><fpage>2089</fpage><lpage>2094</lpage><volume>70</volume><year>2011</year><pub-id pub-id-type="pmid">21978999</pub-id></element-citation></ref><ref id="REF19"><label>19</label><element-citation publication-type="journal"><article-title>IV immunoglobulin might be considered as a first-line treatment of severe interstitial lung disease associated with polymyositis</article-title><source>Chest</source><person-group><name><surname>Diot</surname><given-names>E</given-names></name><name><surname>Carmier</surname><given-names>D</given-names></name><name><surname>Marquette</surname><given-names>D</given-names></name><name><surname>Marchand-Adam</surname><given-names>S</given-names></name><name><surname>Diot</surname><given-names>P</given-names></name><name><surname>Lesire</surname><given-names>V</given-names></name></person-group><fpage>562</fpage><lpage>563</lpage><volume>140</volume><year>2011</year><pub-id pub-id-type="pmid">21813543</pub-id></element-citation></ref><ref id="REF20"><label>20</label><element-citation publication-type="journal"><article-title>Intravenous immunoglobulins for steroid-refractory esophageal involvement related to polymyositis and dermatomyositis: a series of 73 patients</article-title><source>Arthritis Care Res</source><person-group><name><surname>Marie</surname><given-names>I</given-names></name><name><surname>Menard</surname><given-names>JF</given-names></name><name><surname>Hatron</surname><given-names>PY</given-names></name><etal></etal></person-group><fpage>1748</fpage><lpage>1755</lpage><volume>62</volume><year>2010</year></element-citation></ref><ref id="REF21"><label>21</label><element-citation publication-type="journal"><article-title>Short-term and long-term outcomes of interstitial lung disease in polymyositis and dermatomyositis: a series of 107 patients</article-title><source>Arthritis Rheum</source><person-group><name><surname>Marie</surname><given-names>I</given-names></name><name><surname>Hatron</surname><given-names>PY</given-names></name><name><surname>Dominique</surname><given-names>S</given-names></name><name><surname>Cherin</surname><given-names>P</given-names></name><name><surname>Mouthon</surname><given-names>L</given-names></name><name><surname>Menard</surname><given-names>JF</given-names></name></person-group><fpage>3439</fpage><lpage>3447</lpage><volume>63</volume><year>2011</year><pub-id pub-id-type="pmid">21702020</pub-id></element-citation></ref><ref id="REF22"><label>22</label><element-citation publication-type="journal"><article-title>Recent treatment of interstitial lung disease with idiopathic inflammatory myopathies</article-title><source>Clin Med Insights Circ Respir Pulm Med</source><person-group><name><surname>Kawasumi</surname><given-names>H</given-names></name><name><surname>Gono</surname><given-names>T</given-names></name><name><surname>Kawaguchi</surname><given-names>Y</given-names></name><name><surname>Yamanaka</surname><given-names>H</given-names></name></person-group><fpage>9</fpage><lpage>17</lpage><volume>9</volume><year>2015</year><pub-id pub-id-type="pmid">26279636</pub-id></element-citation></ref><ref id="REF23"><label>23</label><element-citation publication-type="journal"><article-title>Interstitial lung disease in polymyositis and dermatomyositis</article-title><source>Curr Opin Rheumatol</source><person-group><name><surname>Hirakata</surname><given-names>M</given-names></name><name><surname>Nagai</surname><given-names>S</given-names></name></person-group><fpage>501</fpage><lpage>508</lpage><volume>12</volume><year>2000</year><uri xlink:href="https://www.ncbi.nlm.nih.gov/pubmed/11092199">https://www.ncbi.nlm.nih.gov/pubmed/11092199</uri><pub-id pub-id-type="pmid">11092199</pub-id></element-citation></ref><ref id="REF24"><label>24</label><element-citation publication-type="journal"><article-title>Predictive factors and unfavourable prognostic factors of interstitial lung disease in patients with polymyositis or dermatomyositis: a retrospective study</article-title><source>Chin Med J (Engl)</source><person-group><name><surname>Ji</surname><given-names>SY</given-names></name><name><surname>Zeng</surname><given-names>FQ</given-names></name><name><surname>Guo</surname><given-names>Q</given-names></name><etal></etal></person-group><fpage>517</fpage><lpage>522</lpage><volume>123</volume><year>2010</year><pub-id pub-id-type="pmid">20367973</pub-id></element-citation></ref><ref id="REF25"><label>25</label><element-citation publication-type="journal"><article-title>The predictive factors and unfavourable prognostic factors of interstitial lung disease in patients with polymyositis/dermatomyositis. (Article in Chinese)</article-title><source>Zhonghua Jie He He Hu Xi Za Zhi</source><person-group><name><surname>Wang</surname><given-names>PZ</given-names></name><name><surname>Guan</surname><given-names>JL</given-names></name><name><surname>Han</surname><given-names>XH</given-names></name></person-group><fpage>417</fpage><lpage>420</lpage><volume>31</volume><year>2008</year><uri xlink:href="https://europepmc.org/abstract/med/19031800">https://europepmc.org/abstract/med/19031800</uri><pub-id pub-id-type="pmid">19031800</pub-id></element-citation></ref><ref id="REF26"><label>26</label><element-citation publication-type="journal"><article-title>KL-6: a serological biomarker for interstitial lung disease in patients with polymyositis and dermatomyositis</article-title><source>J Intern Med</source><person-group><name><surname>Fathi</surname><given-names>M</given-names></name><name><surname>Barbasso Helmers</surname><given-names>S</given-names></name><name><surname>Lundberg</surname><given-names>IE</given-names></name></person-group><fpage>589</fpage><lpage>597</lpage><volume>271</volume><year>2012</year><pub-id pub-id-type="pmid">21950266</pub-id></element-citation></ref><ref id="REF27"><label>27</label><element-citation publication-type="journal"><article-title>Intravenous immunoglobulin therapy for refractory interstitial lung disease associated with polymyositis/dermatomyositis</article-title><source>Lung</source><person-group><name><surname>Suzuki</surname><given-names>Y</given-names></name><name><surname>Hayakawa</surname><given-names>H</given-names></name><name><surname>Miwa</surname><given-names>S</given-names></name><etal></etal></person-group><fpage>201</fpage><lpage>206</lpage><volume>187</volume><year>2009</year><pub-id pub-id-type="pmid">19387736</pub-id></element-citation></ref><ref id="REF28"><label>28</label><element-citation publication-type="journal"><article-title>Differences in clinical features and prognosis of interstitial lung diseases between polymyositis and dermatomyositis</article-title><source>J Rheumatol</source><person-group><name><surname>Fujisawa</surname><given-names>T</given-names></name><name><surname>Suda</surname><given-names>T</given-names></name><name><surname>Nakamura</surname><given-names>Y</given-names></name><etal></etal></person-group><fpage>58</fpage><lpage>64</lpage><volume>32</volume><year>2005</year><uri xlink:href="http://www.jrheum.org/content/32/1/58.short">http://www.jrheum.org/content/32/1/58.short</uri><pub-id pub-id-type="pmid">15630726</pub-id></element-citation></ref><ref id="REF29"><label>29</label><element-citation publication-type="journal"><article-title>Adult clinically amyopathic dermatomyositis with rapid progressive interstitial lung disease: a retrospective cohort study</article-title><source>Clin Rheumatol</source><person-group><name><surname>Ye</surname><given-names>S</given-names></name><name><surname>Chen</surname><given-names>XX</given-names></name><name><surname>Lu</surname><given-names>XY</given-names></name><etal></etal></person-group><fpage>1647</fpage><lpage>1654</lpage><volume>26</volume><year>2007</year><pub-id pub-id-type="pmid">17308858</pub-id></element-citation></ref><ref id="REF30"><label>30</label><element-citation publication-type="journal"><article-title>Cytokine profiles in polymyositis and dermatomyositis complicated by rapidly progressive or chronic interstitial lung disease</article-title><source>Rheumatology (Oxford)</source><person-group><name><surname>Gono</surname><given-names>T</given-names></name><name><surname>Kaneko</surname><given-names>H</given-names></name><name><surname>Kawaguchi</surname><given-names>Y</given-names></name><etal></etal></person-group><fpage>2196</fpage><lpage>2203</lpage><volume>53</volume><year>2014</year><pub-id pub-id-type="pmid">24970922</pub-id></element-citation></ref><ref id="REF31"><label>31</label><element-citation publication-type="journal"><article-title>Polymyositis/dermatomyositis and interstitial lung disease: a new therapeutic approach with T-cell-specific immunosuppressants</article-title><source>Autoimmunity</source><person-group><name><surname>Takada</surname><given-names>K</given-names></name><name><surname>Nagasaka</surname><given-names>K</given-names></name><name><surname>Miyasaka</surname><given-names>N</given-names></name></person-group><fpage>383</fpage><lpage>392</lpage><volume>38</volume><year>2005</year><pub-id pub-id-type="pmid">16227154</pub-id></element-citation></ref><ref id="REF32"><label>32</label><element-citation publication-type="journal"><article-title>Evaluation and treatment of speech and swallowing disorders associated with myopathies</article-title><source>Curr Opin Rheumatol</source><person-group><name><surname>Sonies</surname><given-names>BC</given-names></name></person-group><fpage>486</fpage><lpage>495</lpage><volume>9</volume><year>1997</year><uri xlink:href="https://europepmc.org/abstract/med/9375277">https://europepmc.org/abstract/med/9375277</uri><pub-id pub-id-type="pmid">9375277</pub-id></element-citation></ref><ref id="REF33"><label>33</label><element-citation publication-type="journal"><article-title>Long-term prognosis of 69 patients with dermatomyositis or polymyositis</article-title><source>Clin Exp Rheumatol</source><person-group><name><surname>Maugars</surname><given-names>YM</given-names></name><name><surname>Berthelot</surname><given-names>JM</given-names></name><name><surname>Abbas</surname><given-names>AA</given-names></name><name><surname>Mussini</surname><given-names>JM</given-names></name><name><surname>Nguyen</surname><given-names>JM</given-names></name><name><surname>Prost</surname><given-names>AM</given-names></name></person-group><fpage>263</fpage><lpage>274</lpage><volume>14</volume><year>1996</year><uri xlink:href="https://europepmc.org/abstract/med/8809440">https://europepmc.org/abstract/med/8809440</uri><pub-id pub-id-type="pmid">8809440</pub-id></element-citation></ref><ref id="REF34"><label>34</label><element-citation publication-type="journal"><article-title>Oropharyngeal dysphagia in dermatomyositis: associations with clinical and laboratory features including autoantibodies</article-title><source>PLoS One</source><person-group><name><surname>Mugii</surname><given-names>N</given-names></name><name><surname>Hasegawa</surname><given-names>M</given-names></name><name><surname>Matsushita</surname><given-names>T</given-names></name><etal></etal></person-group><fpage>154746</fpage><volume>11</volume><year>2016</year></element-citation></ref><ref id="REF35"><label>35</label><element-citation publication-type="journal"><article-title>Childhood dermatomyositis: clinical course and long-term follow-up</article-title><source>Clin Pediatr (Phila)</source><person-group><name><surname>Miller</surname><given-names>LC</given-names></name><name><surname>Michael</surname><given-names>AF</given-names></name><name><surname>Kim</surname><given-names>Y</given-names></name></person-group><fpage>561</fpage><lpage>566</lpage><volume>26</volume><year>1987</year><pub-id pub-id-type="pmid">3665326</pub-id></element-citation></ref><ref id="REF36"><label>36</label><element-citation publication-type="journal"><article-title>Oropharyngeal dysphagia in juvenile dermatomyositis (JDM): an evaluation of videofluoroscopy swallow study (VFSS) changes in relation to clinical symptoms and objective muscle scores</article-title><source>Rheumatology</source><person-group><name><surname>McCann</surname><given-names>LJ</given-names></name><name><surname>Garay</surname><given-names>SM</given-names></name><name><surname>Ryan</surname><given-names>MM</given-names></name><name><surname>Harris</surname><given-names>R</given-names></name><name><surname>Riley</surname><given-names>P</given-names></name><name><surname>Pilkington</surname><given-names>CA</given-names></name></person-group><fpage>1363</fpage><lpage>1366</lpage><volume>46</volume><year>2007</year><pub-id pub-id-type="pmid">17569746</pub-id></element-citation></ref><ref id="REF37"><label>37</label><element-citation publication-type="journal"><article-title>Prognosis in juvenile dermatopolymyositis: a cooperative retrospective study of 70 cases</article-title><source>Pediatr Dermatol</source><person-group><name><surname>Taieb</surname><given-names>A</given-names></name><name><surname>Guichard</surname><given-names>C</given-names></name><name><surname>Salamon</surname><given-names>R</given-names></name><name><surname>Maleville</surname><given-names>J</given-names></name></person-group><fpage>275</fpage><lpage>281</lpage><volume>2</volume><year>1985</year><uri xlink:href="https://www.ncbi.nlm.nih.gov/pubmed/4011505">https://www.ncbi.nlm.nih.gov/pubmed/4011505</uri><pub-id pub-id-type="pmid">4011505</pub-id></element-citation></ref><ref id="REF38"><label>38</label><element-citation publication-type="journal"><article-title>The juvenile dermatomyositis national registry and repository (UK and Ireland)--clinical characteristics of children recruited within the first 5 yr</article-title><source>Rheumatology (Oxford)</source><person-group><name><surname>McCann</surname><given-names>LJ</given-names></name><name><surname>Juggins</surname><given-names>AD</given-names></name><name><surname>Maillard</surname><given-names>SM</given-names></name><name><surname>Wedderburn</surname><given-names>LR</given-names></name><name><surname>Davidson</surname><given-names>JE</given-names></name><name><surname>Murray</surname><given-names>KJ</given-names></name><name><surname>Pilkington</surname><given-names>CA</given-names></name></person-group><fpage>1255</fpage><lpage>1260</lpage><volume>45</volume><year>2006</year><pub-id pub-id-type="pmid">16567354</pub-id></element-citation></ref><ref id="REF39"><label>39</label><element-citation publication-type="journal"><article-title>Ten-year experience of juvenile dermatomyositis: a retrospective study</article-title><source>J Microbiol Immunol Infect</source><person-group><name><surname>Chiu</surname><given-names>SK</given-names></name><name><surname>Yang</surname><given-names>YH</given-names></name><name><surname>Wang</surname><given-names>LC</given-names></name><name><surname>Chiang</surname><given-names>BL</given-names></name></person-group><fpage>68</fpage><lpage>73</lpage><volume>40</volume><year>2007</year><uri xlink:href="https://www.ncbi.nlm.nih.gov/pubmed/?term=Ten-year+experience+of+juvenile+dermatomyositis%3A+a+retrospective+study.+Journal+of+microbiology%2C+immunology%2C+and+infection">https://www.ncbi.nlm.nih.gov/pubmed/?term=Ten-year+experience+of+juvenile+dermatomyositis%3A+a+retrospective+study.+Journal+of+microbiology%2C+immunology%2C+and+infection</uri><pub-id pub-id-type="pmid">17332910</pub-id></element-citation></ref><ref id="REF40"><label>40</label><element-citation publication-type="journal"><article-title>Subcutaneous immunoglobulin in refractory juvenile dermatomyositis</article-title><source>Pediatrics</source><person-group><name><surname>de Inocencio</surname><given-names>J</given-names></name><name><surname>Enríquez-Merayo</surname><given-names>E</given-names></name><name><surname>Gonzalez-Granado</surname><given-names>LI</given-names></name><name><surname>Casado</surname><given-names>R</given-names></name></person-group><fpage>0</fpage><volume>137</volume><year>2016</year></element-citation></ref><ref id="REF41"><label>41</label><element-citation publication-type="journal"><article-title>Intravenous immunoglobulins as treatment of life threatening esophageal involvement in polymyositis and dermatomyositis</article-title><source>J Rheumatol</source><person-group><name><surname>Marie</surname><given-names>I</given-names></name><name><surname>Hachulla</surname><given-names>E</given-names></name><name><surname>Levesque</surname><given-names>H</given-names></name><etal></etal></person-group><fpage>2706</fpage><lpage>2709</lpage><volume>26</volume><year>1999</year><uri xlink:href="https://www.ncbi.nlm.nih.gov/pubmed/?term=%3A+Intravenous+immunoglobulins+as+treatment+of+life+threatening+esophageal+involvement+in+polymyositis+and+dermatomyositis.+J+Rheumatol.+1999%2C">https://www.ncbi.nlm.nih.gov/pubmed/?term=%3A+Intravenous+immunoglobulins+as+treatment+of+life+threatening+esophageal+involvement+in+polymyositis+and+dermatomyositis.+J+Rheumatol.+1999%2C</uri><pub-id pub-id-type="pmid">10606390</pub-id></element-citation></ref><ref id="REF42"><label>42</label><element-citation publication-type="journal"><article-title>Dysphagia due to inclusion body myositis: case presentation and review of the literature</article-title><source>Ann Otol Rhinol Laryngol</source><person-group><name><surname>Ko</surname><given-names>EH</given-names></name><name><surname>Rubin</surname><given-names>AD</given-names></name></person-group><fpage>605</fpage><lpage>608</lpage><volume>123</volume><year>2014</year><pub-id pub-id-type="pmid">24634148</pub-id></element-citation></ref><ref id="REF43"><label>43</label><element-citation publication-type="journal"><article-title>Interventions for dysphagia in long-term, progressive muscle disease</article-title><source>Cochrane Database Syst Rev</source><person-group><name><surname>Jones</surname><given-names>K</given-names></name><name><surname>Pitceathly</surname><given-names>RD</given-names></name><name><surname>Rose</surname><given-names>MR</given-names></name><name><surname>McGowan</surname><given-names>S</given-names></name><name><surname>Hill</surname><given-names>M</given-names></name><name><surname>Badrising</surname><given-names>UA</given-names></name><name><surname>Hughes</surname><given-names>T</given-names></name></person-group><fpage>4303</fpage><volume>2</volume><year>2016</year></element-citation></ref><ref id="REF44"><label>44</label><element-citation publication-type="journal"><article-title>Long-term subcutaneous immunoglobulin use in inflammatory myopathies: a retrospective review of 19 cases</article-title><source>Autoimmun Rev</source><person-group><name><surname>Cherin</surname><given-names>P</given-names></name><name><surname>Belizna</surname><given-names>C</given-names></name><name><surname>Cartry</surname><given-names>O</given-names></name><etal></etal></person-group><fpage>281</fpage><lpage>286</lpage><volume>15</volume><year>2016</year><uri xlink:href="https://www.ncbi.nlm.nih.gov/pubmed/26688441">https://www.ncbi.nlm.nih.gov/pubmed/26688441</uri><pub-id pub-id-type="pmid">26688441</pub-id></element-citation></ref><ref id="REF45"><label>45</label><element-citation publication-type="journal"><article-title>Evidence-based guideline: intravenous immunoglobulin in the treatment of neuromuscular disorders: report of the Therapeutics and Technology Assessment Subcommittee of the American Academy of Neurology</article-title><source>Neurology</source><person-group><name><surname>Patwa</surname><given-names>HS</given-names></name><name><surname>Chaudhry</surname><given-names>V</given-names></name><name><surname>Katzberg</surname><given-names>H</given-names></name><name><surname>Rae-Grant</surname><given-names>AD</given-names></name><name><surname>So</surname><given-names>YT</given-names></name></person-group><fpage>1009</fpage><lpage>1015</lpage><volume>78</volume><year>2012</year><pub-id pub-id-type="pmid">22454268</pub-id></element-citation></ref><ref id="REF46"><label>46</label><element-citation publication-type="journal"><article-title>The management of dermatomyositis: current treatment options</article-title><source>Expert Opin Pharmacother</source><person-group><name><surname>Sontheimer</surname><given-names>RD</given-names></name></person-group><fpage>1083</fpage><lpage>1099</lpage><volume>5</volume><year>2004</year><pub-id pub-id-type="pmid">15155110</pub-id></element-citation></ref><ref id="REF47"><label>47</label><element-citation publication-type="journal"><article-title>Management of cutaneous dermatomyositis</article-title><source>Dermatol Ther</source><person-group><name><surname>Lam</surname><given-names>C</given-names></name><name><surname>Vleugels</surname><given-names>RA</given-names></name></person-group><fpage>112</fpage><lpage>134</lpage><volume>25</volume><year>2012</year><pub-id pub-id-type="pmid">22741932</pub-id></element-citation></ref><ref id="REF48"><label>48</label><element-citation publication-type="journal"><article-title>High-dose intravenous immunoglobulin in inflammatory myopathies: experience based on controlled clinical trials</article-title><source>Neurol Sci</source><person-group><name><surname>Dalakas</surname><given-names>MC</given-names></name></person-group><fpage>256</fpage><lpage>259</lpage><volume>24</volume><year>2003</year></element-citation></ref><ref id="REF49"><label>49</label><element-citation publication-type="journal"><article-title>Refractory calcinosis in a patient with dermatomyositis: response to intravenous immune globulin</article-title><source>BMJ Case Rep</source><person-group><name><surname>Shahani</surname><given-names>L</given-names></name></person-group><fpage>0</fpage><volume>2012</volume><year>2012</year></element-citation></ref><ref id="REF50"><label>50</label><element-citation publication-type="journal"><article-title>Assessment, classification and treatment of calcinosis as a complication of juvenile dermatomyositis: a survey of pediatric rheumatologists by the childhood arthritis and rheumatology research alliance (CARRA)</article-title><source>Pediatr Rheumatol</source><person-group><name><surname>Orandi</surname><given-names>AB</given-names></name><name><surname>Baszis</surname><given-names>KW</given-names></name><name><surname>Dharnidharka</surname><given-names>VR</given-names></name><name><surname>Huber</surname><given-names>AM</given-names></name><name><surname>Hoeltzel</surname><given-names>MF</given-names></name><name><surname>CARRA Juvenile Myositis</surname><given-names>subgroup</given-names></name></person-group><fpage>71</fpage><volume>15</volume><year>2017</year></element-citation></ref></ref-list></back></pmc></record>Pour manipuler ce document sous Unix (Dilib)
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