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Pulmonary Hypertension and Other Potentially Fatal Pulmonary Complications in Systemic Juvenile Idiopathic Arthritis
dc.contributor.authorKimura, Yukikoen_US
dc.contributor.authorWeiss, Jennifer E.en_US
dc.contributor.authorHaroldson, Kathryn L.en_US
dc.contributor.authorLee, Tzielanen_US
dc.contributor.authorPunaro, Marilynnen_US
dc.contributor.authorOliveira, Sheilaen_US
dc.contributor.authorRabinovich, Eglaen_US
dc.contributor.authorRiebschleger, Meredithen_US
dc.contributor.authorAntón, Jordien_US
dc.contributor.authorBlier, Peter R.en_US
dc.contributor.authorGerloni, Valeriaen_US
dc.contributor.authorHazen, Melissa M.en_US
dc.contributor.authorKessler, Elizabethen_US
dc.contributor.authorOnel, Karenen_US
dc.contributor.authorPasso, Murray H.en_US
dc.contributor.authorRennebohm, Robert M.en_US
dc.contributor.authorWallace, Carol A.en_US
dc.contributor.authorWoo, Patriciaen_US
dc.contributor.authorWulffraat, Nicoen_US
dc.date.accessioned2013-05-02T19:34:58Z
dc.date.available2014-07-01T15:53:28Zen_US
dc.date.issued2013-05en_US
dc.identifier.citationKimura, Yukiko; Weiss, Jennifer E.; Haroldson, Kathryn L.; Lee, Tzielan; Punaro, Marilynn; Oliveira, Sheila; Rabinovich, Egla; Riebschleger, Meredith; Antón, Jordi ; Blier, Peter R.; Gerloni, Valeria; Hazen, Melissa M.; Kessler, Elizabeth; Onel, Karen; Passo, Murray H.; Rennebohm, Robert M.; Wallace, Carol A.; Woo, Patricia; Wulffraat, Nico (2013). "Pulmonary Hypertension and Other Potentially Fatal Pulmonary Complications in Systemic Juvenile Idiopathic Arthritis ." Arthritis Care & Research 65(5): 745-752. <http://hdl.handle.net/2027.42/97453>en_US
dc.identifier.issn2151-464Xen_US
dc.identifier.issn2151-4658en_US
dc.identifier.urihttps://hdl.handle.net/2027.42/97453
dc.description.abstractObjective Systemic juvenile idiopathic arthritis (JIA) is characterized by fevers, rash, and arthritis, for which interleukin‐1 (IL‐1) and IL‐6 inhibitors appear to be effective treatments. Pulmonary arterial hypertension (PAH), interstitial lung disease (ILD), and alveolar proteinosis (AP) have recently been reported with increased frequency in systemic JIA patients. Our aim was to characterize and compare systemic JIA patients with these complications to a larger cohort of systemic JIA patients. Methods Systemic JIA patients who developed PAH, ILD, and/or AP were identified through an electronic Listserv and their demographic, systemic JIA, and pulmonary disease characteristics as well as their medication exposure information were collected. Patients with these features were compared to a cohort of systemic JIA patients enrolled in the Childhood Arthritis and Rheumatology Research Alliance (CARRA) registry. Results The patients (n = 25) were significantly ( P < 0.05) more likely than the CARRA registry cohort (n = 389) to be female; have more systemic features; and have been exposed to an IL‐1 inhibitor, tocilizumab, corticosteroids, intravenous immunoglobulin, cyclosporine, and cyclophosphamide. Twenty patients (80%) were diagnosed with pulmonary disease after 2004. Twenty patients (80%) had macrophage activation syndrome (MAS) during their disease course and 15 patients (60%) had MAS at pulmonary diagnosis. Sixteen patients had PAH, 5 had AP, and 7 had ILD. Seventeen patients (68%) were taking or recently discontinued (<1 month) a biologic agent at pulmonary symptom onset; 12 patients (48%) were taking anti–IL‐1 therapy (primarily anakinra). Seventeen patients (68%) died at a mean of 10.2 months from the diagnosis of pulmonary complications. Conclusion PAH, AP, and ILD are underrecognized complications of systemic JIA that are frequently fatal. These complications may be the result of severe uncontrolled systemic disease activity and may be influenced by medication exposure.en_US
dc.publisherJohn Wiley & Sons, Inc.en_US
dc.titlePulmonary Hypertension and Other Potentially Fatal Pulmonary Complications in Systemic Juvenile Idiopathic Arthritisen_US
dc.typeArticleen_US
dc.rights.robotsIndexNoFollowen_US
dc.subject.hlbsecondlevelGeriatricsen_US
dc.subject.hlbtoplevelHealth Sciencesen_US
dc.description.peerreviewedPeer Revieweden_US
dc.contributor.affiliationumC.S. Mott Children's Hospital, University of Michigan, Ann Arboren_US
dc.contributor.affiliationotherTexas Scottish Rite Hospital, Dallasen_US
dc.contributor.affiliationotherJoseph M. Sanzari Children's Hospital, Hackensack University Medical Center, Hackensack, New Jerseyen_US
dc.contributor.affiliationotherHospital Sant Joan de Déu, Universitat de Barcelona, Barcelona, Spainen_US
dc.contributor.affiliationotherDuke University Medical Center, Durham, North Carolinaen_US
dc.contributor.affiliationotherFederal University, Rio de Janeiro, Brazilen_US
dc.contributor.affiliationotherLucile Packard Children's Hospital, Stanford University School of Medicine, Stanford, Californiaen_US
dc.contributor.affiliationotherDivision of Pediatric Rheumatology, Joseph M. Sanzari Children's Hospital, Hackensack University Medical Center, 30 Prospect Avenue, Hackensack, NJ 07601en_US
dc.contributor.affiliationotherUniversity Medical Center Utrecht, Utrecht, The Netherlandsen_US
dc.contributor.affiliationotherUniversity College London, London, UKen_US
dc.contributor.affiliationotherSeattle Children's Hospital and Research Institute, Seattle, Washingtonen_US
dc.contributor.affiliationotherAlberta Children's Hospital, University of Calgary, Calgary, Alberta, Canadaen_US
dc.contributor.affiliationotherMedical University of South Carolina, Charlestonen_US
dc.contributor.affiliationotherComer Children's Hospital, University of Chicago, Chicago, Illinoisen_US
dc.contributor.affiliationotherChildren's Hospital of Wisconsin, Medical College of Wisconsin, Milwaukeeen_US
dc.contributor.affiliationotherChildren's Hospital Boston, Harvard Medical School, Boston, Massachusettsen_US
dc.contributor.affiliationotherGaetano Pini Institute of Milan, Milan, Italyen_US
dc.contributor.affiliationotherBaystate Children's Hospital, Tufts University School of Medicine, Springfield, Massachussettsen_US
dc.identifier.pmid23139240en_US
dc.description.bitstreamurlhttp://deepblue.lib.umich.edu/bitstream/2027.42/97453/1/21889_ftp.pdf
dc.identifier.doi10.1002/acr.21889en_US
dc.identifier.sourceArthritis Care & Researchen_US
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dc.owningcollnameInterdisciplinary and Peer-Reviewed


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