Pulmonary arterial hypertension in interferonophaties: a case report and a review of the literature
Identifieur interne : 000458 ( Pmc/Checkpoint ); précédent : 000457; suivant : 000459Pulmonary arterial hypertension in interferonophaties: a case report and a review of the literature
Auteurs : A. Trombetta [Italie] ; S. Ghirardo [Italie] ; S. Pastore [Italie] ; A. Tesser [Italie] ; E. Piscianz [Italie] ; A. Tommasini [Italie] ; M. Bobbo [Italie] ; A. Taddio [Italie]Source :
- Pulmonary Circulation [ 2045-8932 ] ; 2019.
Abstract
Pulmonary arterial hypertension consists in an increase of mean pulmonary arterial pressure (PAPm ≥ 25 mmHg), and may lead to right ventricular failure. Pulmonary arterial hypertension can arise in several disorders, encompassing inflammatory conditions and connective tissue diseases. The occurrence of pulmonary arterial hypertension has recently been reported in monogenic interferonopathies and in systemic lupus erythematosus, highlighting the pathogenic role of type I interferons and paving the way to therapies aimed at inhibiting interferon signaling.
We describe a 17-year-old boy with DNase II deficiency, presenting a clinical picture with significant overlap with systemic lupus erythematosus. During treatment with the Janus kinase inhibitor ruxolitinib, he developed pulmonary arterial hypertension, raising the question whether it could represent a sign of insufficient disease control or a drug-related adverse event. The disease even worsened after drug withdrawal, but rapidly improved after starting the drug again at higher dosage.
Pulmonary arterial hypertension can complicate type I interferonopathies. We propose that ruxolitinib was beneficial in this case, but the wider role of Janus kinase inhibitors for the treatment of pulmonary arterial hypertension is not clear. For this reason, a strict cardiologic evaluation must be part of the standard care of subjects with interferonopathies, especially when Janus kinase inhibitors are prescribed.
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DOI: 10.1177/2045894019869837
PubMed: 31448075
PubMed Central: 6689922
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Ghirardo</name><affiliation wicri:level="1"><nlm:aff id="aff1-2045894019869837">Department of Pediatrics, University of Trieste, Trieste, Italy</nlm:aff><country xml:lang="fr">Italie</country><wicri:regionArea>Department of Pediatrics, University of Trieste, Trieste</wicri:regionArea><wicri:noRegion>Trieste</wicri:noRegion></affiliation></author><author><name sortKey="Pastore, S" sort="Pastore, S" uniqKey="Pastore S" first="S." last="Pastore">S. Pastore</name><affiliation wicri:level="1"><nlm:aff id="aff2-2045894019869837">Institute for Maternal and Child Health, “IRCCS Burlo Garofolo”, Trieste, Italy</nlm:aff><country xml:lang="fr">Italie</country><wicri:regionArea>Institute for Maternal and Child Health, “IRCCS Burlo Garofolo”, Trieste</wicri:regionArea><wicri:noRegion>Trieste</wicri:noRegion></affiliation></author><author><name sortKey="Tesser, A" sort="Tesser, A" uniqKey="Tesser A" first="A." last="Tesser">A. 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Taddio</name><affiliation wicri:level="1"><nlm:aff id="aff1-2045894019869837">Department of Pediatrics, University of Trieste, Trieste, Italy</nlm:aff><country xml:lang="fr">Italie</country><wicri:regionArea>Department of Pediatrics, University of Trieste, Trieste</wicri:regionArea><wicri:noRegion>Trieste</wicri:noRegion></affiliation><affiliation wicri:level="1"><nlm:aff id="aff2-2045894019869837">Institute for Maternal and Child Health, “IRCCS Burlo Garofolo”, Trieste, Italy</nlm:aff><country xml:lang="fr">Italie</country><wicri:regionArea>Institute for Maternal and Child Health, “IRCCS Burlo Garofolo”, Trieste</wicri:regionArea><wicri:noRegion>Trieste</wicri:noRegion></affiliation></author></titleStmt><publicationStmt><idno type="wicri:source">PMC</idno><idno type="pmid">31448075</idno><idno type="pmc">6689922</idno><idno type="url">http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6689922</idno><idno type="RBID">PMC:6689922</idno><idno type="doi">10.1177/2045894019869837</idno><date when="2019">2019</date><idno type="wicri:Area/Pmc/Corpus">000321</idno><idno type="wicri:explorRef" wicri:stream="Pmc" wicri:step="Corpus" wicri:corpus="PMC">000321</idno><idno type="wicri:Area/Pmc/Curation">000321</idno><idno type="wicri:explorRef" wicri:stream="Pmc" wicri:step="Curation">000321</idno><idno type="wicri:Area/Pmc/Checkpoint">000458</idno><idno type="wicri:explorRef" wicri:stream="Pmc" wicri:step="Checkpoint">000458</idno></publicationStmt><sourceDesc><biblStruct><analytic><title xml:lang="en" level="a" type="main">Pulmonary arterial hypertension in interferonophaties: a case report and a review of the literature</title><author><name sortKey="Trombetta, A" sort="Trombetta, A" uniqKey="Trombetta A" first="A." last="Trombetta">A. Trombetta</name><affiliation wicri:level="1"><nlm:aff id="aff1-2045894019869837">Department of Pediatrics, University of Trieste, Trieste, Italy</nlm:aff><country xml:lang="fr">Italie</country><wicri:regionArea>Department of Pediatrics, University of Trieste, Trieste</wicri:regionArea><wicri:noRegion>Trieste</wicri:noRegion></affiliation></author><author><name sortKey="Ghirardo, S" sort="Ghirardo, S" uniqKey="Ghirardo S" first="S." last="Ghirardo">S. Ghirardo</name><affiliation wicri:level="1"><nlm:aff id="aff1-2045894019869837">Department of Pediatrics, University of Trieste, Trieste, Italy</nlm:aff><country xml:lang="fr">Italie</country><wicri:regionArea>Department of Pediatrics, University of Trieste, Trieste</wicri:regionArea><wicri:noRegion>Trieste</wicri:noRegion></affiliation></author><author><name sortKey="Pastore, S" sort="Pastore, S" uniqKey="Pastore S" first="S." last="Pastore">S. Pastore</name><affiliation wicri:level="1"><nlm:aff id="aff2-2045894019869837">Institute for Maternal and Child Health, “IRCCS Burlo Garofolo”, Trieste, Italy</nlm:aff><country xml:lang="fr">Italie</country><wicri:regionArea>Institute for Maternal and Child Health, “IRCCS Burlo Garofolo”, Trieste</wicri:regionArea><wicri:noRegion>Trieste</wicri:noRegion></affiliation></author><author><name sortKey="Tesser, A" sort="Tesser, A" uniqKey="Tesser A" first="A." last="Tesser">A. Tesser</name><affiliation wicri:level="1"><nlm:aff id="aff2-2045894019869837">Institute for Maternal and Child Health, “IRCCS Burlo Garofolo”, Trieste, Italy</nlm:aff><country xml:lang="fr">Italie</country><wicri:regionArea>Institute for Maternal and Child Health, “IRCCS Burlo Garofolo”, Trieste</wicri:regionArea><wicri:noRegion>Trieste</wicri:noRegion></affiliation></author><author><name sortKey="Piscianz, E" sort="Piscianz, E" uniqKey="Piscianz E" first="E." last="Piscianz">E. Piscianz</name><affiliation wicri:level="1"><nlm:aff id="aff2-2045894019869837">Institute for Maternal and Child Health, “IRCCS Burlo Garofolo”, Trieste, Italy</nlm:aff><country xml:lang="fr">Italie</country><wicri:regionArea>Institute for Maternal and Child Health, “IRCCS Burlo Garofolo”, Trieste</wicri:regionArea><wicri:noRegion>Trieste</wicri:noRegion></affiliation></author><author><name sortKey="Tommasini, A" sort="Tommasini, A" uniqKey="Tommasini A" first="A." last="Tommasini">A. Tommasini</name><affiliation wicri:level="1"><nlm:aff id="aff2-2045894019869837">Institute for Maternal and Child Health, “IRCCS Burlo Garofolo”, Trieste, Italy</nlm:aff><country xml:lang="fr">Italie</country><wicri:regionArea>Institute for Maternal and Child Health, “IRCCS Burlo Garofolo”, Trieste</wicri:regionArea><wicri:noRegion>Trieste</wicri:noRegion></affiliation></author><author><name sortKey="Bobbo, M" sort="Bobbo, M" uniqKey="Bobbo M" first="M." last="Bobbo">M. Bobbo</name><affiliation wicri:level="1"><nlm:aff id="aff2-2045894019869837">Institute for Maternal and Child Health, “IRCCS Burlo Garofolo”, Trieste, Italy</nlm:aff><country xml:lang="fr">Italie</country><wicri:regionArea>Institute for Maternal and Child Health, “IRCCS Burlo Garofolo”, Trieste</wicri:regionArea><wicri:noRegion>Trieste</wicri:noRegion></affiliation></author><author><name sortKey="Taddio, A" sort="Taddio, A" uniqKey="Taddio A" first="A." last="Taddio">A. Taddio</name><affiliation wicri:level="1"><nlm:aff id="aff1-2045894019869837">Department of Pediatrics, University of Trieste, Trieste, Italy</nlm:aff><country xml:lang="fr">Italie</country><wicri:regionArea>Department of Pediatrics, University of Trieste, Trieste</wicri:regionArea><wicri:noRegion>Trieste</wicri:noRegion></affiliation><affiliation wicri:level="1"><nlm:aff id="aff2-2045894019869837">Institute for Maternal and Child Health, “IRCCS Burlo Garofolo”, Trieste, Italy</nlm:aff><country xml:lang="fr">Italie</country><wicri:regionArea>Institute for Maternal and Child Health, “IRCCS Burlo Garofolo”, Trieste</wicri:regionArea><wicri:noRegion>Trieste</wicri:noRegion></affiliation></author></analytic><series><title level="j">Pulmonary Circulation</title><idno type="ISSN">2045-8932</idno><idno type="eISSN">2045-8940</idno><imprint><date when="2019">2019</date></imprint></series></biblStruct></sourceDesc></fileDesc><profileDesc><textClass></textClass></profileDesc></teiHeader><front><div type="abstract" xml:lang="en"><sec><title>Background</title><p>Pulmonary arterial hypertension consists in an increase of mean pulmonary arterial pressure (PAPm ≥ 25 mmHg), and may lead to right ventricular failure. Pulmonary arterial hypertension can arise in several disorders, encompassing inflammatory conditions and connective tissue diseases. The occurrence of pulmonary arterial hypertension has recently been reported in monogenic interferonopathies and in systemic lupus erythematosus, highlighting the pathogenic role of type I interferons and paving the way to therapies aimed at inhibiting interferon signaling.</p></sec><sec><title>Case</title><p>We describe a 17-year-old boy with DNase II deficiency, presenting a clinical picture with significant overlap with systemic lupus erythematosus. During treatment with the Janus kinase inhibitor ruxolitinib, he developed pulmonary arterial hypertension, raising the question whether it could represent a sign of insufficient disease control or a drug-related adverse event. The disease even worsened after drug withdrawal, but rapidly improved after starting the drug again at higher dosage.</p></sec><sec><title>Summary and conclusion</title><p>Pulmonary arterial hypertension can complicate type I interferonopathies. We propose that ruxolitinib was beneficial in this case, but the wider role of Janus kinase inhibitors for the treatment of pulmonary arterial hypertension is not clear. For this reason, a strict cardiologic evaluation must be part of the standard care of subjects with interferonopathies, especially when Janus kinase inhibitors are prescribed.</p></sec></div></front><back><div1 type="bibliography"><listBibl><biblStruct><analytic><author><name sortKey="Volpi, S" uniqKey="Volpi S">S Volpi</name></author><author><name sortKey="Picco, P" uniqKey="Picco P">P Picco</name></author><author><name sortKey="Caorsi, R" uniqKey="Caorsi R">R Caorsi</name></author></analytic></biblStruct><biblStruct><analytic><author><name sortKey="Dhala, A" uniqKey="Dhala A">A Dhala</name></author></analytic></biblStruct><biblStruct><analytic><author><name sortKey="Galie, N" uniqKey="Galie N">N Galiè</name></author><author><name sortKey="Humbert, M" uniqKey="Humbert M">M Humbert</name></author><author><name sortKey="Vachiery, Jl" uniqKey="Vachiery J">JL Vachiery</name></author></analytic></biblStruct><biblStruct><analytic><author><name sortKey="Rodero, Mp" uniqKey="Rodero M">MP Rodero</name></author><author><name sortKey="Tesser, A" uniqKey="Tesser A">A Tesser</name></author><author><name sortKey="Bartok, E" uniqKey="Bartok E">E Bartok</name></author></analytic></biblStruct><biblStruct><analytic><author><name sortKey="An, J" uniqKey="An J">J An</name></author><author><name sortKey="Woodward, Jj" uniqKey="Woodward J">JJ Woodward</name></author><author><name sortKey="Sasaki, T" uniqKey="Sasaki T">T Sasaki</name></author></analytic></biblStruct><biblStruct><analytic><author><name sortKey="Fremond, M L" uniqKey="Fremond M">M-L Frémond</name></author><author><name sortKey="Rodero, Mp" uniqKey="Rodero M">MP Rodero</name></author><author><name sortKey="Jeremiah, N" uniqKey="Jeremiah N">N Jeremiah</name></author></analytic></biblStruct><biblStruct><analytic><author><name sortKey="Tabarroki, A" uniqKey="Tabarroki A">A Tabarroki</name></author><author><name sortKey="Lindner, Dj" uniqKey="Lindner D">DJ Lindner</name></author><author><name sortKey="Visconte, V" uniqKey="Visconte V">V Visconte</name></author></analytic></biblStruct><biblStruct><analytic><author><name sortKey="Kim, H" uniqKey="Kim H">H Kim</name></author><author><name sortKey="Sanchez, Ga" uniqKey="Sanchez G">GA Sanchez</name></author><author><name sortKey="Goldbach Mansky, R" uniqKey="Goldbach Mansky R">R Goldbach-Mansky</name></author></analytic></biblStruct><biblStruct><analytic><author><name sortKey="Sanchez, Gam" uniqKey="Sanchez G">GAM Sanchez</name></author><author><name sortKey="Reinhardt, A" uniqKey="Reinhardt A">A Reinhardt</name></author><author><name sortKey="Ramsey, S" uniqKey="Ramsey S">S Ramsey</name></author></analytic></biblStruct><biblStruct><analytic><author><name sortKey="Adang, La" uniqKey="Adang L">LA Adang</name></author><author><name sortKey="Frank, Db" uniqKey="Frank D">DB Frank</name></author><author><name sortKey="Gilani, A" uniqKey="Gilani A">A Gilani</name></author></analytic></biblStruct><biblStruct><analytic><author><name sortKey="Low, At" uniqKey="Low A">AT Low</name></author><author><name sortKey="Howard, L" uniqKey="Howard L">L Howard</name></author><author><name sortKey="Harrison, C" uniqKey="Harrison C">C Harrison</name></author></analytic></biblStruct><biblStruct><analytic><author><name sortKey="Masri, Fa" uniqKey="Masri F">FA Masri</name></author><author><name sortKey="Xu, W" uniqKey="Xu W">W Xu</name></author><author><name sortKey="Comhair, Sa" uniqKey="Comhair S">SA Comhair</name></author></analytic></biblStruct><biblStruct><analytic><author><name sortKey="George, Pm" uniqKey="George P">PM George</name></author><author><name sortKey="Oliver, E" uniqKey="Oliver E">E Oliver</name></author><author><name sortKey="Dorfmuller, P" uniqKey="Dorfmuller P">P Dorfmuller</name></author></analytic></biblStruct><biblStruct><analytic><author><name sortKey="Savale, L" uniqKey="Savale L">L Savale</name></author><author><name sortKey="Chaumais, Mc" uniqKey="Chaumais M">MC Chaumais</name></author><author><name sortKey="Sitbon, O" uniqKey="Sitbon O">O Sitbon</name></author></analytic></biblStruct></listBibl></div1></back></TEI><pmc article-type="case-report"><pmc-dir>properties open_access</pmc-dir>
<front><journal-meta><journal-id journal-id-type="nlm-ta">Pulm Circ</journal-id><journal-id journal-id-type="iso-abbrev">Pulm Circ</journal-id><journal-id journal-id-type="publisher-id">PUL</journal-id><journal-id journal-id-type="hwp">sppul</journal-id><journal-title-group><journal-title>Pulmonary Circulation</journal-title></journal-title-group><issn pub-type="ppub">2045-8932</issn><issn pub-type="epub">2045-8940</issn><publisher><publisher-name>SAGE Publications</publisher-name><publisher-loc>Sage UK: London, England</publisher-loc></publisher></journal-meta><article-meta><article-id pub-id-type="pmid">31448075</article-id><article-id pub-id-type="pmc">6689922</article-id><article-id pub-id-type="doi">10.1177/2045894019869837</article-id><article-id pub-id-type="publisher-id">10.1177_2045894019869837</article-id><article-categories><subj-group subj-group-type="heading"><subject>Case Report</subject></subj-group></article-categories><title-group><article-title>Pulmonary arterial hypertension in interferonophaties: a case report and a review of the literature</article-title></title-group><contrib-group><contrib contrib-type="author"><name><surname>Trombetta</surname><given-names>A.</given-names></name><xref ref-type="aff" rid="aff1-2045894019869837">1</xref></contrib><contrib contrib-type="author"><name><surname>Ghirardo</surname><given-names>S.</given-names></name><xref ref-type="aff" rid="aff1-2045894019869837">1</xref></contrib><contrib contrib-type="author"><name><surname>Pastore</surname><given-names>S.</given-names></name><xref ref-type="aff" rid="aff2-2045894019869837">2</xref></contrib><contrib contrib-type="author"><name><surname>Tesser</surname><given-names>A.</given-names></name><xref ref-type="aff" rid="aff2-2045894019869837">2</xref></contrib><contrib contrib-type="author"><name><surname>Piscianz</surname><given-names>E.</given-names></name><xref ref-type="aff" rid="aff2-2045894019869837">2</xref></contrib><contrib contrib-type="author"><name><surname>Tommasini</surname><given-names>A.</given-names></name><xref ref-type="aff" rid="aff2-2045894019869837">2</xref><xref ref-type="corresp" rid="corresp1-2045894019869837"></xref></contrib><contrib contrib-type="author"><name><surname>Bobbo</surname><given-names>M.</given-names></name><xref ref-type="aff" rid="aff2-2045894019869837">2</xref></contrib><contrib contrib-type="author"><name><surname>Taddio</surname><given-names>A.</given-names></name><xref ref-type="aff" rid="aff1-2045894019869837">1</xref><xref ref-type="aff" rid="aff2-2045894019869837">2</xref></contrib></contrib-group><aff id="aff1-2045894019869837"><label>1</label>Department of Pediatrics, University of Trieste, Trieste, Italy</aff><aff id="aff2-2045894019869837"><label>2</label>Institute for Maternal and Child Health, “IRCCS Burlo Garofolo”, Trieste, Italy</aff><author-notes><corresp id="corresp1-2045894019869837">A. Tommasini, Via dell’Istria 65/1, 34137 Trieste, Italy. Email: <email>alberto.tommasini@burlo.trieste.it</email></corresp></author-notes><pub-date pub-type="epub"><day>09</day><month>8</month><year>2019</year></pub-date><pub-date pub-type="collection"><season>Jul-Sep</season><year>2019</year></pub-date><volume>9</volume><issue>3</issue><elocation-id>2045894019869837</elocation-id><history><date date-type="received"><day>25</day><month>4</month><year>2019</year></date><date date-type="accepted"><day>11</day><month>7</month><year>2019</year></date></history><permissions><copyright-statement>© The Author(s) 2019</copyright-statement><copyright-year>2019</copyright-year><copyright-holder content-type="society">SAGE Publications Ltd, or Pulmonary Vascular Research Institute, unless otherwise noted. Manuscript content on this site is licensed under Creative Commons Licenses</copyright-holder><license license-type="creative-commons" xlink:href="http://creativecommons.org/licenses/by-nc/4.0/"><license-p>Creative Commons Non Commercial CC BY-NC: This article is distributed under the terms of the Creative Commons Attribution-NonCommercial 4.0 License (<ext-link ext-link-type="uri" xlink:href="http://www.creativecommons.org/licenses/by-nc/4.0/">http://www.creativecommons.org/licenses/by-nc/4.0/</ext-link>) which permits non-commercial use, reproduction and distribution of the work without further permission provided the original work is attributed as specified on the SAGE and Open Access pages (<ext-link ext-link-type="uri" xlink:href="https://us.sagepub.com/en-us/nam/open-access-at-sage">https://us.sagepub.com/en-us/nam/open-access-at-sage</ext-link>).</license-p></license></permissions><abstract><sec><title>Background</title><p>Pulmonary arterial hypertension consists in an increase of mean pulmonary arterial pressure (PAPm ≥ 25 mmHg), and may lead to right ventricular failure. Pulmonary arterial hypertension can arise in several disorders, encompassing inflammatory conditions and connective tissue diseases. The occurrence of pulmonary arterial hypertension has recently been reported in monogenic interferonopathies and in systemic lupus erythematosus, highlighting the pathogenic role of type I interferons and paving the way to therapies aimed at inhibiting interferon signaling.</p></sec><sec><title>Case</title><p>We describe a 17-year-old boy with DNase II deficiency, presenting a clinical picture with significant overlap with systemic lupus erythematosus. During treatment with the Janus kinase inhibitor ruxolitinib, he developed pulmonary arterial hypertension, raising the question whether it could represent a sign of insufficient disease control or a drug-related adverse event. The disease even worsened after drug withdrawal, but rapidly improved after starting the drug again at higher dosage.</p></sec><sec><title>Summary and conclusion</title><p>Pulmonary arterial hypertension can complicate type I interferonopathies. We propose that ruxolitinib was beneficial in this case, but the wider role of Janus kinase inhibitors for the treatment of pulmonary arterial hypertension is not clear. For this reason, a strict cardiologic evaluation must be part of the standard care of subjects with interferonopathies, especially when Janus kinase inhibitors are prescribed.</p></sec></abstract><kwd-group><kwd>DNase II deficiency</kwd><kwd>echocardiography</kwd><kwd>interferonopathies</kwd><kwd>Janus kinase inhibitors</kwd><kwd>pulmonary hypertension</kwd></kwd-group><funding-group><award-group id="award1-2045894019869837"><funding-source id="funding1-2045894019869837"><institution-wrap><institution>Institute for Maternal and Child Health ,,IRCCS Burlo Garofolo" Trieste, Italy</institution><institution-id institution-id-type="FundRef"></institution-id></institution-wrap></funding-source><award-id rid="funding1-2045894019869837">RC24/17 </award-id></award-group><award-group id="award2-2045894019869837"><funding-source id="funding2-2045894019869837"><institution-wrap><institution>Telethon Foundation</institution><institution-id institution-id-type="FundRef"></institution-id></institution-wrap></funding-source><award-id rid="funding2-2045894019869837">GGP15241</award-id></award-group></funding-group><custom-meta-group><custom-meta><meta-name>cover-date</meta-name><meta-value>July-September 2019</meta-value></custom-meta></custom-meta-group></article-meta></front></pmc><affiliations><list><country><li>Italie</li></country></list><tree><country name="Italie"><noRegion><name sortKey="Trombetta, A" sort="Trombetta, A" uniqKey="Trombetta A" first="A." last="Trombetta">A. Trombetta</name></noRegion><name sortKey="Bobbo, M" sort="Bobbo, M" uniqKey="Bobbo M" first="M." last="Bobbo">M. Bobbo</name><name sortKey="Ghirardo, S" sort="Ghirardo, S" uniqKey="Ghirardo S" first="S." last="Ghirardo">S. Ghirardo</name><name sortKey="Pastore, S" sort="Pastore, S" uniqKey="Pastore S" first="S." last="Pastore">S. Pastore</name><name sortKey="Piscianz, E" sort="Piscianz, E" uniqKey="Piscianz E" first="E." last="Piscianz">E. Piscianz</name><name sortKey="Taddio, A" sort="Taddio, A" uniqKey="Taddio A" first="A." last="Taddio">A. Taddio</name><name sortKey="Taddio, A" sort="Taddio, A" uniqKey="Taddio A" first="A." last="Taddio">A. Taddio</name><name sortKey="Tesser, A" sort="Tesser, A" uniqKey="Tesser A" first="A." last="Tesser">A. Tesser</name><name sortKey="Tommasini, A" sort="Tommasini, A" uniqKey="Tommasini A" first="A." last="Tommasini">A. Tommasini</name></country></tree></affiliations></record>Pour manipuler ce document sous Unix (Dilib)
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