Granulomatous Disease in CVID: Retrospective Analysis of Clinical Characteristics and Treatment Efficacy in a Cohort of 59 Patients
Identifieur interne : 000233 ( Istex/Corpus ); précédent : 000232; suivant : 000234Granulomatous Disease in CVID: Retrospective Analysis of Clinical Characteristics and Treatment Efficacy in a Cohort of 59 Patients
Auteurs : Jean-Nicolas Boursiquot ; Laurence Gérard ; Marion Malphettes ; Claire Fieschi ; Lionel Galicier ; David Boutboul ; Raphael Borie ; Jean-François Viallard ; Pauline Soulas-Sprauel ; Alice Berezne ; Arnaud Jaccard ; Eric Hachulla ; Julien Haroche ; Nicolas Schleinitz ; Laurent Têtu ; Eric OksenhendlerSource :
- Journal of Clinical Immunology [ 0271-9142 ] ; 2013-01-01.
English descriptors
Abstract
Abstract: Background: Granulomatous disease (GD) will develop in a subset of patients with common variable immunodeficiency (CVID). Little is known about the efficacy of therapeutic agents used for treating this disorder. Objective: To evaluate the efficacy of immunosuppressive drugs with the help of a set of clinical, biological and radiological criteria. Method: Clinical and laboratory features of CVID patients were collected from the French DEFI cohort, a prospective study on adults with hypogammaglobulinemia. The medical charts of 55 patients (93 %) of the GD cohort were reviewed. Results: Among 436 subjects with CVID, 59 patients (13.5 %) were diagnosed with GD. Of the 55 patients in whom medical charts were available, 32 patients received treatment for the granulomatous disease. Corticosteroids were the most frequently used drug. Complete response to treatment was infrequent. It was achieved with corticosteroids, cyclophosphamide, hydroxychloroquine, rituximab and methotrexate. Azathioprine, cyclosporine, mycophenolate mofetil, sirolimus, infliximab and thalidomide led to partial or absence of response. Complete and partial responses were observed in lymph nodes, lungs, liver, skin, bone marrow and central nervous system. Absent of response for gastrointestinal tract granulomas was noted in all cases of treatment attempt. Conclusion: CVID patients with GD exhibit a particular biological phenotype. Treatment should be considered in any symptomatic patient or if there is evidence of organ dysfunction. Corticosteroids are the drug of choice in most instances but response to treatment is often unsatisfactory.
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DOI: 10.1007/s10875-012-9778-9
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Publique- Hôpitaux de Paris, 1, rue Claude-Vellefaux, 75010, Paris, France</mods:affiliation></affiliation></author><author><name sortKey="Malphettes, Marion" sort="Malphettes, Marion" uniqKey="Malphettes M" first="Marion" last="Malphettes">Marion Malphettes</name><affiliation><mods:affiliation>Department of Clinical Immunology, Hôpital Saint-Louis, Assistance Publique- Hôpitaux de Paris, 1, rue Claude-Vellefaux, 75010, Paris, France</mods:affiliation></affiliation></author><author><name sortKey="Fieschi, Claire" sort="Fieschi, Claire" uniqKey="Fieschi C" first="Claire" last="Fieschi">Claire Fieschi</name><affiliation><mods:affiliation>Department of Clinical Immunology, Hôpital Saint-Louis, Assistance Publique- Hôpitaux de Paris, 1, rue Claude-Vellefaux, 75010, Paris, France</mods:affiliation></affiliation></author><author><name sortKey="Galicier, Lionel" sort="Galicier, Lionel" uniqKey="Galicier L" first="Lionel" last="Galicier">Lionel Galicier</name><affiliation><mods:affiliation>Department of Clinical Immunology, Hôpital Saint-Louis, Assistance Publique- Hôpitaux de Paris, 1, rue Claude-Vellefaux, 75010, Paris, France</mods:affiliation></affiliation></author><author><name sortKey="Boutboul, David" sort="Boutboul, David" uniqKey="Boutboul D" first="David" last="Boutboul">David Boutboul</name><affiliation><mods:affiliation>Department of Clinical Immunology, Hôpital Saint-Louis, Assistance Publique- Hôpitaux de Paris, 1, rue Claude-Vellefaux, 75010, Paris, France</mods:affiliation></affiliation></author><author><name sortKey="Borie, Raphael" sort="Borie, Raphael" uniqKey="Borie R" first="Raphael" last="Borie">Raphael Borie</name><affiliation><mods:affiliation>Department of Respirology, Hôpital Tenon, Paris, France</mods:affiliation></affiliation></author><author><name sortKey="Viallard, Jean Francois" sort="Viallard, Jean Francois" uniqKey="Viallard J" first="Jean-François" last="Viallard">Jean-François Viallard</name><affiliation><mods:affiliation>Department of Internal Medicine, Hôpital Haut Lévêque, Pessac, France</mods:affiliation></affiliation></author><author><name sortKey="Soulas Sprauel, Pauline" sort="Soulas Sprauel, Pauline" uniqKey="Soulas Sprauel P" first="Pauline" last="Soulas-Sprauel">Pauline Soulas-Sprauel</name><affiliation><mods:affiliation>Department of Internal Medicine and INSERM UMR S737, Strasbourg, France</mods:affiliation></affiliation></author><author><name sortKey="Berezne, Alice" sort="Berezne, Alice" uniqKey="Berezne A" first="Alice" last="Berezne">Alice Berezne</name><affiliation><mods:affiliation>Department of Internal Medicine, Hôpital Cochin, Assistance Publique-Hôpitaux de Paris, Paris, France</mods:affiliation></affiliation></author><author><name sortKey="Jaccard, Arnaud" sort="Jaccard, Arnaud" uniqKey="Jaccard A" first="Arnaud" last="Jaccard">Arnaud Jaccard</name><affiliation><mods:affiliation>Department of Hematology, Hôpital Dupuytren, Limoges, France</mods:affiliation></affiliation></author><author><name sortKey="Hachulla, Eric" sort="Hachulla, Eric" uniqKey="Hachulla E" first="Eric" last="Hachulla">Eric Hachulla</name><affiliation><mods:affiliation>Department of Internal Medicine, Hôpital Claude Huriez, Lille, France</mods:affiliation></affiliation></author><author><name sortKey="Haroche, Julien" sort="Haroche, Julien" uniqKey="Haroche J" first="Julien" last="Haroche">Julien Haroche</name><affiliation><mods:affiliation>Department of Internal Medicine, Hôpital de la Salpêtrière, Assistance Publique-Hôpitaux de Paris, Paris, France</mods:affiliation></affiliation></author><author><name sortKey="Schleinitz, Nicolas" sort="Schleinitz, Nicolas" uniqKey="Schleinitz N" first="Nicolas" last="Schleinitz">Nicolas Schleinitz</name><affiliation><mods:affiliation>Department of Internal Medicine, Hôpital de la Conception, Marseille, France</mods:affiliation></affiliation></author><author><name sortKey="Tetu, Laurent" sort="Tetu, Laurent" uniqKey="Tetu L" first="Laurent" last="Têtu">Laurent Têtu</name><affiliation><mods:affiliation>Service de Pneumologie, Hôpital Rangueil-Larrey, Toulouse, France</mods:affiliation></affiliation></author><author><name sortKey="Oksenhendler, Eric" sort="Oksenhendler, Eric" uniqKey="Oksenhendler E" first="Eric" last="Oksenhendler">Eric Oksenhendler</name><affiliation><mods:affiliation>Department of Clinical Immunology, Hôpital Saint-Louis, Assistance Publique- Hôpitaux de Paris, 1, rue Claude-Vellefaux, 75010, Paris, France</mods:affiliation></affiliation></author></analytic><monogr></monogr><series><title level="j">Journal of Clinical Immunology</title><title level="j" type="abbrev">J Clin Immunol</title><idno type="ISSN">0271-9142</idno><idno type="eISSN">1573-2592</idno><imprint><publisher>Springer US; http://www.springer-ny.com</publisher><pubPlace>Boston</pubPlace><date type="published" when="2013-01-01">2013-01-01</date><biblScope unit="volume">33</biblScope><biblScope unit="issue">1</biblScope><biblScope unit="page" from="84">84</biblScope><biblScope unit="page" to="95">95</biblScope></imprint><idno type="ISSN">0271-9142</idno></series></biblStruct></sourceDesc><seriesStmt><idno type="ISSN">0271-9142</idno></seriesStmt></fileDesc><profileDesc><textClass><keywords scheme="KwdEn" xml:lang="en"><term>CVID</term><term>antibody deficiency</term><term>granulomatous disease</term><term>primary immune deficiency</term><term>treatment</term></keywords></textClass><langUsage><language ident="en">en</language></langUsage></profileDesc></teiHeader><front><div type="abstract" xml:lang="en">Abstract: Background: Granulomatous disease (GD) will develop in a subset of patients with common variable immunodeficiency (CVID). Little is known about the efficacy of therapeutic agents used for treating this disorder. Objective: To evaluate the efficacy of immunosuppressive drugs with the help of a set of clinical, biological and radiological criteria. Method: Clinical and laboratory features of CVID patients were collected from the French DEFI cohort, a prospective study on adults with hypogammaglobulinemia. The medical charts of 55 patients (93 %) of the GD cohort were reviewed. Results: Among 436 subjects with CVID, 59 patients (13.5 %) were diagnosed with GD. Of the 55 patients in whom medical charts were available, 32 patients received treatment for the granulomatous disease. Corticosteroids were the most frequently used drug. Complete response to treatment was infrequent. It was achieved with corticosteroids, cyclophosphamide, hydroxychloroquine, rituximab and methotrexate. Azathioprine, cyclosporine, mycophenolate mofetil, sirolimus, infliximab and thalidomide led to partial or absence of response. Complete and partial responses were observed in lymph nodes, lungs, liver, skin, bone marrow and central nervous system. Absent of response for gastrointestinal tract granulomas was noted in all cases of treatment attempt. Conclusion: CVID patients with GD exhibit a particular biological phenotype. Treatment should be considered in any symptomatic patient or if there is evidence of organ dysfunction. Corticosteroids are the drug of choice in most instances but response to treatment is often unsatisfactory.</div></front></TEI><istex><corpusName>springer-journals</corpusName><author><json:item><name>Jean-Nicolas Boursiquot</name><affiliations><json:string>Department of Clinical Immunology and Allergy, Centre Hospitalier Universitaire de Québec, Laval University, Quebec City, Quebec, Canada</json:string><json:string>E-mail: jean-nicolas.boursiquot@fmed.ulaval.ca</json:string></affiliations></json:item><json:item><name>Laurence Gérard</name><affiliations><json:string>Department of Clinical Immunology, Hôpital Saint-Louis, Assistance Publique- Hôpitaux de Paris, 1, rue Claude-Vellefaux, 75010, Paris, France</json:string></affiliations></json:item><json:item><name>Marion Malphettes</name><affiliations><json:string>Department of Clinical Immunology, Hôpital Saint-Louis, Assistance Publique- Hôpitaux de Paris, 1, rue Claude-Vellefaux, 75010, Paris, France</json:string></affiliations></json:item><json:item><name>Claire Fieschi</name><affiliations><json:string>Department of Clinical Immunology, Hôpital Saint-Louis, Assistance Publique- Hôpitaux de Paris, 1, rue Claude-Vellefaux, 75010, Paris, France</json:string></affiliations></json:item><json:item><name>Lionel Galicier</name><affiliations><json:string>Department of Clinical Immunology, Hôpital Saint-Louis, Assistance Publique- Hôpitaux de Paris, 1, rue Claude-Vellefaux, 75010, Paris, France</json:string></affiliations></json:item><json:item><name>David Boutboul</name><affiliations><json:string>Department of Clinical Immunology, Hôpital Saint-Louis, Assistance Publique- Hôpitaux de Paris, 1, rue Claude-Vellefaux, 75010, Paris, France</json:string></affiliations></json:item><json:item><name>Raphael Borie</name><affiliations><json:string>Department of Respirology, Hôpital Tenon, Paris, France</json:string></affiliations></json:item><json:item><name>Jean-François Viallard</name><affiliations><json:string>Department of Internal Medicine, Hôpital Haut Lévêque, Pessac, France</json:string></affiliations></json:item><json:item><name>Pauline Soulas-Sprauel</name><affiliations><json:string>Department of Internal Medicine and INSERM UMR S737, Strasbourg, France</json:string></affiliations></json:item><json:item><name>Alice Berezne</name><affiliations><json:string>Department of Internal Medicine, Hôpital Cochin, Assistance Publique-Hôpitaux de Paris, Paris, France</json:string></affiliations></json:item><json:item><name>Arnaud Jaccard</name><affiliations><json:string>Department of Hematology, Hôpital Dupuytren, Limoges, France</json:string></affiliations></json:item><json:item><name>Eric Hachulla</name><affiliations><json:string>Department of Internal Medicine, Hôpital Claude Huriez, Lille, France</json:string></affiliations></json:item><json:item><name>Julien Haroche</name><affiliations><json:string>Department of Internal Medicine, Hôpital de la Salpêtrière, Assistance Publique-Hôpitaux de Paris, Paris, France</json:string></affiliations></json:item><json:item><name>Nicolas Schleinitz</name><affiliations><json:string>Department of Internal Medicine, Hôpital de la Conception, Marseille, France</json:string></affiliations></json:item><json:item><name>Laurent Têtu</name><affiliations><json:string>Service de Pneumologie, Hôpital Rangueil-Larrey, Toulouse, France</json:string></affiliations></json:item><json:item><name>Eric Oksenhendler</name><affiliations><json:string>Department of Clinical Immunology, Hôpital Saint-Louis, Assistance Publique- Hôpitaux de Paris, 1, rue Claude-Vellefaux, 75010, Paris, France</json:string></affiliations></json:item></author><subject><json:item><lang><json:string>eng</json:string></lang><value>CVID</value></json:item><json:item><lang><json:string>eng</json:string></lang><value>granulomatous disease</value></json:item><json:item><lang><json:string>eng</json:string></lang><value>antibody deficiency</value></json:item><json:item><lang><json:string>eng</json:string></lang><value>primary immune deficiency</value></json:item><json:item><lang><json:string>eng</json:string></lang><value>treatment</value></json:item><json:item><lang><json:string>eng</json:string></lang><value>CVID : Common variable immunodeficiency</value></json:item><json:item><lang><json:string>eng</json:string></lang><value>GD : Granulomatous disease</value></json:item><json:item><lang><json:string>eng</json:string></lang><value>TR : Therapeutic regimen</value></json:item></subject><articleId><json:string>9778</json:string><json:string>s10875-012-9778-9</json:string></articleId><arkIstex>ark:/67375/VQC-ZQSX5PDG-G</arkIstex><language><json:string>eng</json:string></language><originalGenre><json:string>OriginalPaper</json:string></originalGenre><abstract>Abstract: Background: Granulomatous disease (GD) will develop in a subset of patients with common variable immunodeficiency (CVID). Little is known about the efficacy of therapeutic agents used for treating this disorder. Objective: To evaluate the efficacy of immunosuppressive drugs with the help of a set of clinical, biological and radiological criteria. Method: Clinical and laboratory features of CVID patients were collected from the French DEFI cohort, a prospective study on adults with hypogammaglobulinemia. The medical charts of 55 patients (93 %) of the GD cohort were reviewed. Results: Among 436 subjects with CVID, 59 patients (13.5 %) were diagnosed with GD. Of the 55 patients in whom medical charts were available, 32 patients received treatment for the granulomatous disease. Corticosteroids were the most frequently used drug. Complete response to treatment was infrequent. It was achieved with corticosteroids, cyclophosphamide, hydroxychloroquine, rituximab and methotrexate. Azathioprine, cyclosporine, mycophenolate mofetil, sirolimus, infliximab and thalidomide led to partial or absence of response. Complete and partial responses were observed in lymph nodes, lungs, liver, skin, bone marrow and central nervous system. Absent of response for gastrointestinal tract granulomas was noted in all cases of treatment attempt. Conclusion: CVID patients with GD exhibit a particular biological phenotype. Treatment should be considered in any symptomatic patient or if there is evidence of organ dysfunction. Corticosteroids are the drug of choice in most instances but response to treatment is often unsatisfactory.</abstract><qualityIndicators><score>9.76</score><pdfWordCount>6740</pdfWordCount><pdfCharCount>45883</pdfCharCount><pdfVersion>1.4</pdfVersion><pdfPageCount>12</pdfPageCount><pdfPageSize>595.276 x 790.866 pts</pdfPageSize><refBibsNative>false</refBibsNative><abstractWordCount>230</abstractWordCount><abstractCharCount>1638</abstractCharCount><keywordCount>8</keywordCount></qualityIndicators><title>Granulomatous Disease in CVID: Retrospective Analysis of Clinical Characteristics and Treatment Efficacy in a Cohort of 59 Patients</title><corporate><json:item><name>the DEFI study group</name></json:item></corporate><genre><json:string>research-article</json:string></genre><host><title>Journal of Clinical 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Patients</title></titleStmt><publicationStmt><authority>ISTEX</authority><publisher scheme="https://scientific-publisher.data.istex.fr">Springer US; http://www.springer-ny.com</publisher><pubPlace>Boston</pubPlace><availability><licence><p>Springer Science+Business Media, LLC, 2012</p></licence><p scheme="https://loaded-corpus.data.istex.fr/ark:/67375/XBH-3XSW68JL-F">springer</p></availability><date>2012-05-29</date></publicationStmt><notesStmt><note type="research-article" scheme="https://content-type.data.istex.fr/ark:/67375/XTP-1JC4F85T-7">research-article</note><note type="journal" scheme="https://publication-type.data.istex.fr/ark:/67375/JMC-0GLKJH51-B">journal</note><note>Original Research</note></notesStmt><sourceDesc><biblStruct type="inbook"><analytic><title level="a" type="main" xml:lang="en">Granulomatous Disease in CVID: Retrospective Analysis of Clinical Characteristics and Treatment Efficacy in a Cohort of 59 Patients</title><author role="org"><orgName>the DEFI study group</orgName></author><author xml:id="author-0000" corresp="yes"><persName><forename type="first">Jean-Nicolas</forename><surname>Boursiquot</surname></persName><email>jean-nicolas.boursiquot@fmed.ulaval.ca</email><affiliation>Department of Clinical Immunology and Allergy, Centre Hospitalier Universitaire de Québec, Laval University, Quebec City, Quebec, Canada</affiliation></author><author xml:id="author-0001"><persName><forename type="first">Laurence</forename><surname>Gérard</surname></persName><affiliation>Department of Clinical Immunology, Hôpital Saint-Louis, Assistance Publique- Hôpitaux de Paris, 1, rue Claude-Vellefaux, 75010, Paris, France</affiliation></author><author xml:id="author-0002"><persName><forename type="first">Marion</forename><surname>Malphettes</surname></persName><affiliation>Department of Clinical Immunology, Hôpital Saint-Louis, Assistance Publique- Hôpitaux de Paris, 1, rue Claude-Vellefaux, 75010, Paris, France</affiliation></author><author xml:id="author-0003"><persName><forename type="first">Claire</forename><surname>Fieschi</surname></persName><affiliation>Department of Clinical Immunology, Hôpital Saint-Louis, Assistance Publique- Hôpitaux de Paris, 1, rue Claude-Vellefaux, 75010, Paris, France</affiliation></author><author xml:id="author-0004"><persName><forename type="first">Lionel</forename><surname>Galicier</surname></persName><affiliation>Department of Clinical Immunology, Hôpital Saint-Louis, Assistance Publique- Hôpitaux de Paris, 1, rue Claude-Vellefaux, 75010, Paris, France</affiliation></author><author xml:id="author-0005"><persName><forename type="first">David</forename><surname>Boutboul</surname></persName><affiliation>Department of Clinical Immunology, Hôpital Saint-Louis, Assistance Publique- Hôpitaux de Paris, 1, rue Claude-Vellefaux, 75010, Paris, France</affiliation></author><author xml:id="author-0006"><persName><forename type="first">Raphael</forename><surname>Borie</surname></persName><affiliation>Department of Respirology, Hôpital Tenon, Paris, France</affiliation></author><author xml:id="author-0007"><persName><forename type="first">Jean-François</forename><surname>Viallard</surname></persName><affiliation>Department of Internal Medicine, Hôpital Haut Lévêque, Pessac, France</affiliation></author><author xml:id="author-0008"><persName><forename type="first">Pauline</forename><surname>Soulas-Sprauel</surname></persName><affiliation>Department of Internal Medicine and INSERM UMR S737, Strasbourg, France</affiliation></author><author xml:id="author-0009"><persName><forename type="first">Alice</forename><surname>Berezne</surname></persName><affiliation>Department of Internal Medicine, Hôpital Cochin, Assistance Publique-Hôpitaux de Paris, Paris, France</affiliation></author><author xml:id="author-0010"><persName><forename type="first">Arnaud</forename><surname>Jaccard</surname></persName><affiliation>Department of Hematology, Hôpital Dupuytren, Limoges, France</affiliation></author><author xml:id="author-0011"><persName><forename type="first">Eric</forename><surname>Hachulla</surname></persName><affiliation>Department of Internal Medicine, Hôpital Claude Huriez, Lille, France</affiliation></author><author xml:id="author-0012"><persName><forename type="first">Julien</forename><surname>Haroche</surname></persName><affiliation>Department of Internal Medicine, Hôpital de la Salpêtrière, Assistance Publique-Hôpitaux de Paris, Paris, France</affiliation></author><author xml:id="author-0013"><persName><forename type="first">Nicolas</forename><surname>Schleinitz</surname></persName><affiliation>Department of Internal Medicine, Hôpital de la Conception, Marseille, France</affiliation></author><author xml:id="author-0014"><persName><forename type="first">Laurent</forename><surname>Têtu</surname></persName><affiliation>Service de Pneumologie, Hôpital Rangueil-Larrey, Toulouse, France</affiliation></author><author xml:id="author-0015"><persName><forename type="first">Eric</forename><surname>Oksenhendler</surname></persName><affiliation>Department of Clinical Immunology, Hôpital Saint-Louis, Assistance Publique- Hôpitaux de Paris, 1, rue Claude-Vellefaux, 75010, Paris, France</affiliation></author><idno type="istex">6D0EC3A09AF8C287E72FDBC64FA5B17ADECD676B</idno><idno type="ark">ark:/67375/VQC-ZQSX5PDG-G</idno><idno type="DOI">10.1007/s10875-012-9778-9</idno><idno type="article-id">9778</idno><idno type="article-id">s10875-012-9778-9</idno></analytic><monogr><title level="j">Journal of Clinical Immunology</title><title level="j" type="abbrev">J Clin Immunol</title><idno type="pISSN">0271-9142</idno><idno type="eISSN">1573-2592</idno><idno type="journal-ID">true</idno><idno type="issue-article-count">34</idno><idno type="volume-issue-count">8</idno><imprint><publisher>Springer US; http://www.springer-ny.com</publisher><pubPlace>Boston</pubPlace><date type="published" when="2013-01-01"></date><biblScope unit="volume">33</biblScope><biblScope unit="issue">1</biblScope><biblScope unit="page" from="84">84</biblScope><biblScope unit="page" to="95">95</biblScope></imprint></monogr></biblStruct></sourceDesc></fileDesc><profileDesc><creation><date>2012-05-29</date></creation><langUsage><language ident="en">en</language></langUsage><abstract xml:lang="en"><p>Abstract: Background: Granulomatous disease (GD) will develop in a subset of patients with common variable immunodeficiency (CVID). Little is known about the efficacy of therapeutic agents used for treating this disorder. Objective: To evaluate the efficacy of immunosuppressive drugs with the help of a set of clinical, biological and radiological criteria. Method: Clinical and laboratory features of CVID patients were collected from the French DEFI cohort, a prospective study on adults with hypogammaglobulinemia. The medical charts of 55 patients (93 %) of the GD cohort were reviewed. Results: Among 436 subjects with CVID, 59 patients (13.5 %) were diagnosed with GD. Of the 55 patients in whom medical charts were available, 32 patients received treatment for the granulomatous disease. Corticosteroids were the most frequently used drug. Complete response to treatment was infrequent. It was achieved with corticosteroids, cyclophosphamide, hydroxychloroquine, rituximab and methotrexate. Azathioprine, cyclosporine, mycophenolate mofetil, sirolimus, infliximab and thalidomide led to partial or absence of response. Complete and partial responses were observed in lymph nodes, lungs, liver, skin, bone marrow and central nervous system. Absent of response for gastrointestinal tract granulomas was noted in all cases of treatment attempt. Conclusion: CVID patients with GD exhibit a particular biological phenotype. Treatment should be considered in any symptomatic patient or if there is evidence of organ dysfunction. Corticosteroids are the drug of choice in most instances but response to treatment is often unsatisfactory.</p></abstract><textClass xml:lang="en"><keywords scheme="keyword"><list><head>Keywords</head><item><term>CVID</term></item><item><term>granulomatous disease</term></item><item><term>antibody deficiency</term></item><item><term>primary immune deficiency</term></item><item><term>treatment</term></item></list></keywords></textClass><textClass><keywords scheme="keyword"><list><head>Abbreviations</head><item><term>CVID</term><term>Common variable immunodeficiency</term></item><item><term>GD</term><term>Granulomatous disease</term></item><item><term>TR</term><term>Therapeutic regimen</term></item></list></keywords></textClass><textClass><keywords scheme="Journal-Subject-Collection"><list><label>SC3</label><item><term>Biomedical and Life Sciences</term></item></list></keywords></textClass><textClass><keywords scheme="Journal-Subject-Group"><list><label>SCB</label><item><term>Biomedicine</term></item><label>SCB14000</label><item><term>Immunology</term></item><label>SCH33096</label><item><term>Infectious Diseases</term></item><label>SCH33002</label><item><term>Internal Medicine</term></item><label>SCB16003</label><item><term>Medical Microbiology</term></item></list></keywords></textClass></profileDesc><revisionDesc><change when="2012-05-29">Created</change><change when="2013-01-01">Published</change></revisionDesc></teiHeader></istex:fulltextTEI><json:item><extension>txt</extension><original>false</original><mimetype>text/plain</mimetype><uri>https://api.istex.fr/ark:/67375/VQC-ZQSX5PDG-G/fulltext.txt</uri></json:item></fulltext><metadata><istex:metadataXml wicri:clean="corpus springer-journals not found" wicri:toSee="no header"><istex:xmlDeclaration>version="1.0" encoding="UTF-8"</istex:xmlDeclaration><istex:docType PUBLIC="-//Springer-Verlag//DTD A++ V2.4//EN" URI="http://devel.springer.de/A++/V2.4/DTD/A++V2.4.dtd" name="istex:docType"></istex:docType><istex:document><Publisher><PublisherInfo><PublisherName>Springer US</PublisherName><PublisherLocation>Boston</PublisherLocation><PublisherImprintName>Springer</PublisherImprintName><PublisherURL>http://www.springer-ny.com</PublisherURL></PublisherInfo><Journal OutputMedium="All"><JournalInfo JournalProductType="ArchiveJournal" NumberingStyle="Unnumbered"><JournalID>10875</JournalID><JournalPrintISSN>0271-9142</JournalPrintISSN><JournalElectronicISSN>1573-2592</JournalElectronicISSN><JournalTitle>Journal of Clinical Immunology</JournalTitle><JournalAbbreviatedTitle>J Clin Immunol</JournalAbbreviatedTitle><JournalSubjectGroup><JournalSubject Code="SCB" Type="Primary">Biomedicine</JournalSubject><JournalSubject Code="SCB14000" Priority="1" Type="Secondary">Immunology</JournalSubject><JournalSubject Code="SCH33096" Priority="2" Type="Secondary">Infectious Diseases</JournalSubject><JournalSubject Code="SCH33002" Priority="3" Type="Secondary">Internal Medicine</JournalSubject><JournalSubject Code="SCB16003" Priority="4" Type="Secondary">Medical Microbiology</JournalSubject><SubjectCollection Code="SC3">Biomedical and Life Sciences</SubjectCollection></JournalSubjectGroup></JournalInfo><Volume OutputMedium="All"><VolumeInfo TocLevels="0" VolumeType="Regular"><VolumeIDStart>33</VolumeIDStart><VolumeIDEnd>33</VolumeIDEnd><VolumeIssueCount>8</VolumeIssueCount></VolumeInfo><Issue IssueType="Regular" OutputMedium="All"><IssueInfo IssueType="Regular" TocLevels="0"><IssueIDStart>1</IssueIDStart><IssueIDEnd>1</IssueIDEnd><IssueArticleCount>34</IssueArticleCount><IssueHistory><OnlineDate><Year>2013</Year><Month>1</Month><Day>25</Day></OnlineDate><PrintDate><Year>2013</Year><Month>1</Month><Day>24</Day></PrintDate><CoverDate><Year>2013</Year><Month>1</Month></CoverDate><PricelistYear>2013</PricelistYear></IssueHistory><IssueCopyright><CopyrightHolderName>Springer Science+Business Media New York</CopyrightHolderName><CopyrightYear>2013</CopyrightYear></IssueCopyright></IssueInfo><Article ID="s10875-012-9778-9" OutputMedium="All"><ArticleInfo ArticleType="OriginalPaper" ContainsESM="No" Language="En" NumberingStyle="Unnumbered" TocLevels="0"><ArticleID>9778</ArticleID><ArticleDOI>10.1007/s10875-012-9778-9</ArticleDOI><ArticleSequenceNumber>11</ArticleSequenceNumber><ArticleTitle Language="En">Granulomatous Disease in CVID: Retrospective Analysis of Clinical Characteristics and Treatment Efficacy in a Cohort of 59 Patients</ArticleTitle><ArticleCategory>Original Research</ArticleCategory><ArticleFirstPage>84</ArticleFirstPage><ArticleLastPage>95</ArticleLastPage><ArticleHistory><RegistrationDate><Year>2012</Year><Month>8</Month><Day>21</Day></RegistrationDate><Received><Year>2012</Year><Month>5</Month><Day>29</Day></Received><Accepted><Year>2012</Year><Month>8</Month><Day>21</Day></Accepted><OnlineDate><Year>2012</Year><Month>9</Month><Day>18</Day></OnlineDate></ArticleHistory><ArticleCopyright><CopyrightHolderName>Springer Science+Business Media, LLC</CopyrightHolderName><CopyrightYear>2012</CopyrightYear></ArticleCopyright><ArticleGrants Type="Regular"><MetadataGrant Grant="OpenAccess"></MetadataGrant><AbstractGrant Grant="OpenAccess"></AbstractGrant><BodyPDFGrant Grant="Restricted"></BodyPDFGrant><BodyHTMLGrant Grant="Restricted"></BodyHTMLGrant><BibliographyGrant Grant="Restricted"></BibliographyGrant><ESMGrant Grant="Restricted"></ESMGrant></ArticleGrants></ArticleInfo><ArticleHeader><AuthorGroup><Author AffiliationIDS="Aff1" CorrespondingAffiliationID="Aff1"><AuthorName DisplayOrder="Western"><GivenName>Jean-Nicolas</GivenName><FamilyName>Boursiquot</FamilyName></AuthorName><Contact><Email>jean-nicolas.boursiquot@fmed.ulaval.ca</Email></Contact></Author><Author AffiliationIDS="Aff2"><AuthorName DisplayOrder="Western"><GivenName>Laurence</GivenName><FamilyName>Gérard</FamilyName></AuthorName></Author><Author AffiliationIDS="Aff2"><AuthorName DisplayOrder="Western"><GivenName>Marion</GivenName><FamilyName>Malphettes</FamilyName></AuthorName></Author><Author AffiliationIDS="Aff2"><AuthorName DisplayOrder="Western"><GivenName>Claire</GivenName><FamilyName>Fieschi</FamilyName></AuthorName></Author><Author AffiliationIDS="Aff2"><AuthorName DisplayOrder="Western"><GivenName>Lionel</GivenName><FamilyName>Galicier</FamilyName></AuthorName></Author><Author AffiliationIDS="Aff2"><AuthorName DisplayOrder="Western"><GivenName>David</GivenName><FamilyName>Boutboul</FamilyName></AuthorName></Author><Author AffiliationIDS="Aff3"><AuthorName DisplayOrder="Western"><GivenName>Raphael</GivenName><FamilyName>Borie</FamilyName></AuthorName></Author><Author AffiliationIDS="Aff4"><AuthorName DisplayOrder="Western"><GivenName>Jean-François</GivenName><FamilyName>Viallard</FamilyName></AuthorName></Author><Author AffiliationIDS="Aff5"><AuthorName DisplayOrder="Western"><GivenName>Pauline</GivenName><FamilyName>Soulas-Sprauel</FamilyName></AuthorName></Author><Author AffiliationIDS="Aff6"><AuthorName DisplayOrder="Western"><GivenName>Alice</GivenName><FamilyName>Berezne</FamilyName></AuthorName></Author><Author AffiliationIDS="Aff7"><AuthorName DisplayOrder="Western"><GivenName>Arnaud</GivenName><FamilyName>Jaccard</FamilyName></AuthorName></Author><Author AffiliationIDS="Aff8"><AuthorName DisplayOrder="Western"><GivenName>Eric</GivenName><FamilyName>Hachulla</FamilyName></AuthorName></Author><Author AffiliationIDS="Aff9"><AuthorName DisplayOrder="Western"><GivenName>Julien</GivenName><FamilyName>Haroche</FamilyName></AuthorName></Author><Author AffiliationIDS="Aff10"><AuthorName DisplayOrder="Western"><GivenName>Nicolas</GivenName><FamilyName>Schleinitz</FamilyName></AuthorName></Author><Author AffiliationIDS="Aff11"><AuthorName DisplayOrder="Western"><GivenName>Laurent</GivenName><FamilyName>Têtu</FamilyName></AuthorName></Author><Author AffiliationIDS="Aff2"><AuthorName DisplayOrder="Western"><GivenName>Eric</GivenName><FamilyName>Oksenhendler</FamilyName></AuthorName></Author><InstitutionalAuthor><InstitutionalAuthorName>the DEFI study group</InstitutionalAuthorName></InstitutionalAuthor><Affiliation ID="Aff1"><OrgName>Department of Clinical Immunology and Allergy, Centre Hospitalier Universitaire de Québec, Laval University</OrgName><OrgAddress><City>Quebec City</City><State>Quebec</State><Country Code="CA">Canada</Country></OrgAddress></Affiliation><Affiliation ID="Aff2"><OrgName>Department of Clinical Immunology, Hôpital Saint-Louis, Assistance Publique- Hôpitaux de Paris</OrgName><OrgAddress><Street>1, rue Claude-Vellefaux</Street><Postcode>75010</Postcode><City>Paris</City><Country Code="FR">France</Country></OrgAddress></Affiliation><Affiliation ID="Aff3"><OrgDivision>Department of Respirology</OrgDivision><OrgName>Hôpital Tenon</OrgName><OrgAddress><City>Paris</City><Country Code="FR">France</Country></OrgAddress></Affiliation><Affiliation ID="Aff4"><OrgDivision>Department of Internal Medicine</OrgDivision><OrgName>Hôpital Haut Lévêque</OrgName><OrgAddress><City>Pessac</City><Country Code="FR">France</Country></OrgAddress></Affiliation><Affiliation ID="Aff5"><OrgName>Department of Internal Medicine and INSERM UMR S737</OrgName><OrgAddress><City>Strasbourg</City><Country Code="FR">France</Country></OrgAddress></Affiliation><Affiliation ID="Aff6"><OrgName>Department of Internal Medicine, Hôpital Cochin, Assistance Publique-Hôpitaux de Paris</OrgName><OrgAddress><City>Paris</City><Country Code="FR">France</Country></OrgAddress></Affiliation><Affiliation ID="Aff7"><OrgDivision>Department of Hematology</OrgDivision><OrgName>Hôpital Dupuytren</OrgName><OrgAddress><City>Limoges</City><Country Code="FR">France</Country></OrgAddress></Affiliation><Affiliation ID="Aff8"><OrgDivision>Department of Internal Medicine</OrgDivision><OrgName>Hôpital Claude Huriez</OrgName><OrgAddress><City>Lille</City><Country Code="FR">France</Country></OrgAddress></Affiliation><Affiliation ID="Aff9"><OrgName>Department of Internal Medicine, Hôpital de la Salpêtrière, Assistance Publique-Hôpitaux de Paris</OrgName><OrgAddress><City>Paris</City><Country Code="FR">France</Country></OrgAddress></Affiliation><Affiliation ID="Aff10"><OrgDivision>Department of Internal Medicine</OrgDivision><OrgName>Hôpital de la Conception</OrgName><OrgAddress><City>Marseille</City><Country Code="FR">France</Country></OrgAddress></Affiliation><Affiliation ID="Aff11"><OrgDivision>Service de Pneumologie</OrgDivision><OrgName>Hôpital Rangueil-Larrey</OrgName><OrgAddress><City>Toulouse</City><Country Code="FR">France</Country></OrgAddress></Affiliation></AuthorGroup><Abstract ID="Abs1" Language="En" OutputMedium="All"><Heading>Abstract</Heading><AbstractSection ID="ASec1"><Heading>Background</Heading><Para>Granulomatous disease (GD) will develop in a subset of patients with common variable immunodeficiency (CVID). Little is known about the efficacy of therapeutic agents used for treating this disorder.</Para></AbstractSection><AbstractSection ID="ASec2"><Heading>Objective</Heading><Para>To evaluate the efficacy of immunosuppressive drugs with the help of a set of clinical, biological and radiological criteria.</Para></AbstractSection><AbstractSection ID="ASec3"><Heading>Method</Heading><Para>Clinical and laboratory features of CVID patients were collected from the French DEFI cohort, a prospective study on adults with hypogammaglobulinemia. The medical charts of 55 patients (93 %) of the GD cohort were reviewed.</Para></AbstractSection><AbstractSection ID="ASec4"><Heading>Results</Heading><Para>Among 436 subjects with CVID, 59 patients (13.5 %) were diagnosed with GD. Of the 55 patients in whom medical charts were available, 32 patients received treatment for the granulomatous disease. Corticosteroids were the most frequently used drug. Complete response to treatment was infrequent. It was achieved with corticosteroids, cyclophosphamide, hydroxychloroquine, rituximab and methotrexate. Azathioprine, cyclosporine, mycophenolate mofetil, sirolimus, infliximab and thalidomide led to partial or absence of response. Complete and partial responses were observed in lymph nodes, lungs, liver, skin, bone marrow and central nervous system. Absent of response for gastrointestinal tract granulomas was noted in all cases of treatment attempt.</Para></AbstractSection><AbstractSection ID="ASec5"><Heading>Conclusion</Heading><Para>CVID patients with GD exhibit a particular biological phenotype. Treatment should be considered in any symptomatic patient or if there is evidence of organ dysfunction. Corticosteroids are the drug of choice in most instances but response to treatment is often unsatisfactory.</Para></AbstractSection></Abstract><KeywordGroup Language="En" OutputMedium="All"><Heading>Keywords</Heading><Keyword>CVID</Keyword><Keyword>granulomatous disease</Keyword><Keyword>antibody deficiency</Keyword><Keyword>primary immune deficiency</Keyword><Keyword>treatment</Keyword></KeywordGroup><AbbreviationGroup><Heading>Abbreviations</Heading><DefinitionList><DefinitionListEntry><Term>CVID</Term><Description><Para>Common variable immunodeficiency</Para></Description></DefinitionListEntry><DefinitionListEntry><Term>GD</Term><Description><Para>Granulomatous disease</Para></Description></DefinitionListEntry><DefinitionListEntry><Term>TR</Term><Description><Para>Therapeutic regimen</Para></Description></DefinitionListEntry></DefinitionList></AbbreviationGroup></ArticleHeader><NoBody></NoBody></Article></Issue></Volume></Journal></Publisher></istex:document></istex:metadataXml><mods version="3.6"><titleInfo lang="en"><title>Granulomatous Disease in CVID: Retrospective Analysis of Clinical Characteristics and Treatment Efficacy in a Cohort of 59 Patients</title></titleInfo><titleInfo type="alternative" contentType="CDATA"><title>Granulomatous Disease in CVID: Retrospective Analysis of Clinical Characteristics and Treatment Efficacy in a Cohort of 59 Patients</title></titleInfo><name type="corporate"><namePart>the DEFI study group</namePart></name><name type="personal" displayLabel="corresp"><namePart type="given">Jean-Nicolas</namePart><namePart type="family">Boursiquot</namePart><affiliation>Department of Clinical Immunology and Allergy, Centre Hospitalier Universitaire de Québec, Laval University, Quebec City, Quebec, Canada</affiliation><affiliation>E-mail: jean-nicolas.boursiquot@fmed.ulaval.ca</affiliation><role><roleTerm type="text">author</roleTerm></role></name><name type="personal"><namePart type="given">Laurence</namePart><namePart type="family">Gérard</namePart><affiliation>Department of Clinical Immunology, Hôpital Saint-Louis, Assistance Publique- Hôpitaux de Paris, 1, rue Claude-Vellefaux, 75010, Paris, France</affiliation><role><roleTerm type="text">author</roleTerm></role></name><name type="personal"><namePart type="given">Marion</namePart><namePart type="family">Malphettes</namePart><affiliation>Department of Clinical Immunology, Hôpital Saint-Louis, Assistance Publique- Hôpitaux de Paris, 1, rue Claude-Vellefaux, 75010, Paris, France</affiliation><role><roleTerm type="text">author</roleTerm></role></name><name type="personal"><namePart type="given">Claire</namePart><namePart type="family">Fieschi</namePart><affiliation>Department of Clinical Immunology, Hôpital Saint-Louis, Assistance Publique- Hôpitaux de Paris, 1, rue Claude-Vellefaux, 75010, Paris, France</affiliation><role><roleTerm type="text">author</roleTerm></role></name><name type="personal"><namePart type="given">Lionel</namePart><namePart type="family">Galicier</namePart><affiliation>Department of Clinical Immunology, Hôpital Saint-Louis, Assistance Publique- Hôpitaux de Paris, 1, rue Claude-Vellefaux, 75010, Paris, France</affiliation><role><roleTerm type="text">author</roleTerm></role></name><name type="personal"><namePart type="given">David</namePart><namePart type="family">Boutboul</namePart><affiliation>Department of Clinical Immunology, Hôpital Saint-Louis, Assistance Publique- Hôpitaux de Paris, 1, rue Claude-Vellefaux, 75010, Paris, France</affiliation><role><roleTerm type="text">author</roleTerm></role></name><name type="personal"><namePart type="given">Raphael</namePart><namePart type="family">Borie</namePart><affiliation>Department of Respirology, Hôpital Tenon, Paris, France</affiliation><role><roleTerm type="text">author</roleTerm></role></name><name type="personal"><namePart type="given">Jean-François</namePart><namePart type="family">Viallard</namePart><affiliation>Department of Internal Medicine, Hôpital Haut Lévêque, Pessac, France</affiliation><role><roleTerm type="text">author</roleTerm></role></name><name type="personal"><namePart type="given">Pauline</namePart><namePart type="family">Soulas-Sprauel</namePart><affiliation>Department of Internal Medicine and INSERM UMR S737, Strasbourg, France</affiliation><role><roleTerm type="text">author</roleTerm></role></name><name type="personal"><namePart type="given">Alice</namePart><namePart type="family">Berezne</namePart><affiliation>Department of Internal Medicine, Hôpital Cochin, Assistance Publique-Hôpitaux de Paris, Paris, France</affiliation><role><roleTerm type="text">author</roleTerm></role></name><name type="personal"><namePart type="given">Arnaud</namePart><namePart type="family">Jaccard</namePart><affiliation>Department of Hematology, Hôpital Dupuytren, Limoges, France</affiliation><role><roleTerm type="text">author</roleTerm></role></name><name type="personal"><namePart type="given">Eric</namePart><namePart type="family">Hachulla</namePart><affiliation>Department of Internal Medicine, Hôpital Claude Huriez, Lille, France</affiliation><role><roleTerm type="text">author</roleTerm></role></name><name type="personal"><namePart type="given">Julien</namePart><namePart type="family">Haroche</namePart><affiliation>Department of Internal Medicine, Hôpital de la Salpêtrière, Assistance Publique-Hôpitaux de Paris, Paris, France</affiliation><role><roleTerm type="text">author</roleTerm></role></name><name type="personal"><namePart type="given">Nicolas</namePart><namePart type="family">Schleinitz</namePart><affiliation>Department of Internal Medicine, Hôpital de la Conception, Marseille, France</affiliation><role><roleTerm type="text">author</roleTerm></role></name><name type="personal"><namePart type="given">Laurent</namePart><namePart type="family">Têtu</namePart><affiliation>Service de Pneumologie, Hôpital Rangueil-Larrey, Toulouse, France</affiliation><role><roleTerm type="text">author</roleTerm></role></name><name type="personal"><namePart type="given">Eric</namePart><namePart type="family">Oksenhendler</namePart><affiliation>Department of Clinical Immunology, Hôpital Saint-Louis, Assistance Publique- Hôpitaux de Paris, 1, rue Claude-Vellefaux, 75010, Paris, France</affiliation><role><roleTerm type="text">author</roleTerm></role></name><typeOfResource>text</typeOfResource><genre type="research-article" displayLabel="OriginalPaper" authority="ISTEX" authorityURI="https://content-type.data.istex.fr" valueURI="https://content-type.data.istex.fr/ark:/67375/XTP-1JC4F85T-7">research-article</genre><originInfo><publisher>Springer US; http://www.springer-ny.com</publisher><place><placeTerm type="text">Boston</placeTerm></place><dateCreated encoding="w3cdtf">2012-05-29</dateCreated><dateIssued encoding="w3cdtf">2013-01-01</dateIssued><copyrightDate encoding="w3cdtf">2012</copyrightDate></originInfo><language><languageTerm type="code" authority="rfc3066">en</languageTerm><languageTerm type="code" authority="iso639-2b">eng</languageTerm></language><abstract lang="en">Abstract: Background: Granulomatous disease (GD) will develop in a subset of patients with common variable immunodeficiency (CVID). Little is known about the efficacy of therapeutic agents used for treating this disorder. Objective: To evaluate the efficacy of immunosuppressive drugs with the help of a set of clinical, biological and radiological criteria. Method: Clinical and laboratory features of CVID patients were collected from the French DEFI cohort, a prospective study on adults with hypogammaglobulinemia. The medical charts of 55 patients (93 %) of the GD cohort were reviewed. Results: Among 436 subjects with CVID, 59 patients (13.5 %) were diagnosed with GD. Of the 55 patients in whom medical charts were available, 32 patients received treatment for the granulomatous disease. Corticosteroids were the most frequently used drug. Complete response to treatment was infrequent. It was achieved with corticosteroids, cyclophosphamide, hydroxychloroquine, rituximab and methotrexate. Azathioprine, cyclosporine, mycophenolate mofetil, sirolimus, infliximab and thalidomide led to partial or absence of response. Complete and partial responses were observed in lymph nodes, lungs, liver, skin, bone marrow and central nervous system. Absent of response for gastrointestinal tract granulomas was noted in all cases of treatment attempt. Conclusion: CVID patients with GD exhibit a particular biological phenotype. Treatment should be considered in any symptomatic patient or if there is evidence of organ dysfunction. Corticosteroids are the drug of choice in most instances but response to treatment is often unsatisfactory.</abstract><note>Original Research</note><subject lang="en"><genre>Keywords</genre><topic>CVID</topic><topic>granulomatous disease</topic><topic>antibody deficiency</topic><topic>primary immune deficiency</topic><topic>treatment</topic></subject><subject><genre>Abbreviations</genre><topic>CVID : Common variable immunodeficiency</topic><topic>GD : Granulomatous disease</topic><topic>TR : Therapeutic regimen</topic></subject><relatedItem type="host"><titleInfo><title>Journal of Clinical Immunology</title></titleInfo><titleInfo type="abbreviated"><title>J Clin Immunol</title></titleInfo><genre type="journal" authority="ISTEX" authorityURI="https://publication-type.data.istex.fr" valueURI="https://publication-type.data.istex.fr/ark:/67375/JMC-0GLKJH51-B">journal</genre><originInfo><publisher>Springer</publisher><dateIssued encoding="w3cdtf">2013-01-25</dateIssued><copyrightDate encoding="w3cdtf">2013</copyrightDate></originInfo><subject><genre>Journal-Subject-Collection</genre><topic authority="SpringerSubjectCodes" authorityURI="SC3">Biomedical and Life Sciences</topic></subject><subject><genre>Journal-Subject-Group</genre><topic authority="SpringerSubjectCodes" authorityURI="SCB">Biomedicine</topic><topic authority="SpringerSubjectCodes" authorityURI="SCB14000">Immunology</topic><topic authority="SpringerSubjectCodes" authorityURI="SCH33096">Infectious Diseases</topic><topic authority="SpringerSubjectCodes" authorityURI="SCH33002">Internal Medicine</topic><topic authority="SpringerSubjectCodes" authorityURI="SCB16003">Medical Microbiology</topic></subject><identifier type="ISSN">0271-9142</identifier><identifier type="eISSN">1573-2592</identifier><identifier type="JournalID">10875</identifier><identifier type="IssueArticleCount">34</identifier><identifier type="VolumeIssueCount">8</identifier><part><date>2013</date><detail type="volume"><number>33</number><caption>vol.</caption></detail><detail type="issue"><number>1</number><caption>no.</caption></detail><extent unit="pages"><start>84</start><end>95</end></extent></part><recordInfo><recordOrigin>Springer Science+Business Media New York, 2013</recordOrigin></recordInfo></relatedItem><identifier type="istex">6D0EC3A09AF8C287E72FDBC64FA5B17ADECD676B</identifier><identifier type="ark">ark:/67375/VQC-ZQSX5PDG-G</identifier><identifier type="DOI">10.1007/s10875-012-9778-9</identifier><identifier type="ArticleID">9778</identifier><identifier type="ArticleID">s10875-012-9778-9</identifier><accessCondition type="use and reproduction" contentType="copyright">Springer Science+Business Media, LLC, 2012</accessCondition><recordInfo><recordContentSource authority="ISTEX" authorityURI="https://loaded-corpus.data.istex.fr" valueURI="https://loaded-corpus.data.istex.fr/ark:/67375/XBH-3XSW68JL-F">springer</recordContentSource><recordOrigin>Springer Science+Business Media, LLC, 2012</recordOrigin></recordInfo></mods><json:item><extension>json</extension><original>false</original><mimetype>application/json</mimetype><uri>https://api.istex.fr/ark:/67375/VQC-ZQSX5PDG-G/record.json</uri></json:item></metadata><serie></serie></istex></record>Pour manipuler ce document sous Unix (Dilib)
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