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<a href="http://doi.org/10.1136/annrheumdis-2019-216040" target="_blank" rel="noreferrer noopener">http://doi.org/10.1136/annrheumdis-2019-216040</a>
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Pages
1722-1731
Issue
12
Volume
78
ISSN
1468-2060
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Title
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Emergent high fatality lung disease in systemic juvenile arthritis
Publisher
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Annals Of The Rheumatic Diseases
Date
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2019
2019-12
Subject
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treatment; inflammation; adult onset still's disease; DMARDs (biologic); juvenile idiopathic arthritis
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Saper Vivian E; Chen Guangbo; Deutsch Gail H; Guillerman R Paul; Birgmeier Johannes; Jagadeesh Karthik; Canna Scott; Schulert Grant; Deterding Robin; Xu Jianpeng; Leung Ann N; Bouzoubaa Layla; Abulaban Khalid; Baszis Kevin; Behrens Edward M; Birmingham James; Casey Alicia; Cidon Michal; Cron Randy Q; De Aliva; De Benedetti Fabrizio; Ferguson Ian; Fishman Martha P; Goodman Steven I; Graham T Brent; Grom Alexei A; Haines Kathleen; Hazen Melissa; Henderson Lauren A; Ho Assunta; Ibarra Maria; Inman Christi J; Jerath Rita; Khawaja Khulood; Kingsbury Daniel J; Klein-Gitelman Marisa; Lai Khanh; Lapidus Sivia; Lin Clara; Lin Jenny; Liptzin Deborah R; Milojevic Diana; Mombourquette Joy; Onel Karen; Ozen Seza; Perez Maria; Phillippi Kathryn; Prahalad Sampath; Radhakrishna Suhas; Reinhardt Adam; Riskalla Mona; Rosenwasser Natalie; Roth Johannes; Schneider Rayfel; Schonenberg-Meinema Dieneke; Shenoi Susan; Smith Judith A; Sönmez Hafize Emine; Stoll Matthew L; Towe Christopher; Vargas Sara O; Vehe Richard K; Young Lisa R; Yang Jacqueline; Desai Tushar; Balise Raymond; Lu Ying; Tian Lu; Bejerano Gill; Davis Mark M; Khatri Purvesh; Mellins Elizabeth D; Childhood Arthritis and Rheumatology Research Alliance Registry Investigators
Description
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OBJECTIVE: To investigate the characteristics and risk factors of a novel parenchymal lung disease (LD), increasingly detected in systemic juvenile idiopathic arthritis (sJIA). METHODS: In a multicentre retrospective study, 61 cases were investigated using physician-reported clinical information and centralised analyses of radiological, pathological and genetic data. RESULTS: LD was associated with distinctive features, including acute erythematous clubbing and a high frequency of anaphylactic reactions to the interleukin (IL)-6 inhibitor, tocilizumab. Serum ferritin elevation and/or significant lymphopaenia preceded LD detection. The most prevalent chest CT pattern was septal thickening, involving the periphery of multiple lobes ± ground-glass opacities. The predominant pathology (23 of 36) was pulmonary alveolar proteinosis and/or endogenous lipoid pneumonia (PAP/ELP), with atypical features including regional involvement and concomitant vascular changes. Apparent severe delayed drug hypersensitivity occurred in some cases. The 5-year survival was 42%. Whole exome sequencing (20 of 61) did not identify a novel monogenic defect or likely causal PAP-related or macrophage activation syndrome (MAS)-related mutations. Trisomy 21 and young sJIA onset increased LD risk. Exposure to IL-1 and IL-6 inhibitors (46 of 61) was associated with multiple LD features. By several indicators, severity of sJIA was comparable in drug-exposed subjects and published sJIA cohorts. MAS at sJIA onset was increased in the drug-exposed, but was not associated with LD features. CONCLUSIONS: A rare, life-threatening lung disease in sJIA is defined by a constellation of unusual clinical characteristics. The pathology, a PAP/ELP variant, suggests macrophage dysfunction. Inhibitor exposure may promote LD, independent of sJIA severity, in a small subset of treated patients. Treatment/prevention strategies are needed.
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<a href="http://doi.org/10.1136/annrheumdis-2019-216040" target="_blank" rel="noreferrer noopener">10.1136/annrheumdis-2019-216040</a>
PMID: 31562126
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Journal Article
2019
Abulaban Khalid
adult onset still's disease
Akron Children's Hospital
Annals of the rheumatic diseases
Balise Raymond
Baszis Kevin
Behrens Edward M
Bejerano Gill
Birgmeier Johannes
Birmingham James
Bouzoubaa Layla
Canna Scott
Casey Alicia
Chen Guangbo
Childhood Arthritis and Rheumatology Research Alliance Registry Investigators
Cidon Michal
Cron Randy Q
Davis Mark M
De Aliva
De Benedetti Fabrizio
Department of Pediatrics
Desai Tushar
Deterding Robin
Deutsch Gail H
DMARDs (biologic)
Ferguson Ian
Fishman Martha P
Goodman Steven I
Graham T Brent
Grom Alexei A
Guillerman R Paul
Haines Kathleen
Hazen Melissa
Henderson Lauren A
Ho Assunta
Ibarra Maria
Inflammation
Inman Christi J
Jagadeesh Karthik
Jerath Rita
Journal Article
juvenile idiopathic arthritis
Khatri Purvesh
Khawaja Khulood
Kingsbury Daniel J
Klein-Gitelman Marisa
Lai Khanh
Lapidus Sivia
Leung Ann N
Lin Clara
Lin Jenny
Liptzin Deborah R
Lu Ying
Mellins Elizabeth D
Milojevic Diana
Mombourquette Joy
NEOMED College of Medicine
November 2019 Update
Onel Karen
Ozen Seza
Perez Maria
Phillippi Kathryn
Prahalad Sampath
Radhakrishna Suhas
Reinhardt Adam
Riskalla Mona
Rosenwasser Natalie
Roth Johannes
Saper Vivian E
Schneider Rayfel
Schonenberg-Meinema Dieneke
Schulert Grant
Shenoi Susan
Smith Judith A
Sönmez Hafize Emine
Stoll Matthew L
Tian Lu
Towe Christopher
Treatment
Vargas Sara O
Vehe Richard K
Xu Jianpeng
Yang Jacqueline
Young Lisa R