What question did this study set out to answer?

To characterize systemic juvenile idiopathic arthritis patients who develop severe pulmonary complications and compare them to a broader registry cohort.

November 8, 2012Arthritis Care & Research

Pulmonary Hypertension and Other Potentially Fatal Pulmonary Complications in Systemic Juvenile Idiopathic Arthritis

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Authors

YKYukiko KimuraKawasaki Medical School JWJennifer E. WeissHackensack University Medical Center KHKathryn L. HaroldsonHackensack University Medical Center

Discussion

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Implication

Comparative cohort study reveals high mortality from severe pulmonary complications in systemic juvenile idiopathic arthritis, highlighting links to intense systemic activity and biologic exposure.

Key Points

To characterize systemic juvenile idiopathic arthritis patients who develop severe pulmonary complications and compare them to a broader registry cohort.
Identified 25 systemic juvenile idiopathic arthritis (JIA) patients with pulmonary arterial hypertension (PAH), interstitial lung disease (ILD), or alveolar proteinosis (AP) through an electronic listserv.
Collected demographic, clinical, and drug exposure data and compared these patients to a control cohort of 389 systemic JIA patients from the Childhood Arthritis and Rheumatology Research Alliance (CARRA) registry.
Patients with pulmonary complications (n = 25) were significantly more likely than CARRA registry controls (n = 389) to be female, show systemic features, and have prior exposure to IL-1 inhibitors, tocilizumab, corticosteroids, intravenous immunoglobulin, cyclosporine, and cyclophosphamide (P < 0.05).
Macrophage activation syndrome occurred in 80% (n = 20) of pulmonary patients during their disease course, and 68% (n = 17) were actively using or had recently stopped a biologic agent at symptom onset.
Pulmonary diagnoses included PAH (n = 16), ILD (n = 7), and AP (n = 5), with an overall mortality rate of 68% (n = 17) occurring at a mean of 10.2 months after pulmonary diagnosis.

Cite This Study

Kimura et al. (2012) studied this question.

synapsesocial.com/papers/6a1981e1ff42a97fac5836bd https://doi.org/10.1002/acr.21889

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Implication

Comparative cohort study reveals high mortality from severe pulmonary complications in systemic juvenile idiopathic arthritis, highlighting links to intense systemic activity and biologic exposure.

Pulmonary Hypertension and Other Potentially Fatal Pulmonary Complications in Systemic Juvenile Idiopathic Arthritis

Discussion

Member takes

Implication

Key Points

Cite This Study

Pulmonary Hypertension and Other Potentially Fatal Pulmonary Complications in Systemic Juvenile Idiopathic Arthritis

Discussion

Member takes

Implication

Key Points

Cite This Study

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