Disease Course, Treatments, and Outcomes of Children With Systemic Juvenile Idiopathic Arthritis–Associated Lung Disease
Authors' conclusion
Patients with SJIA-LD had highly varied courses, with lower mortality than previously reported but frequent hypoxia and requirement for respiratory support.
Study context
- Population
- Children with sJIA-associated lung disease (SJIA-LD)
- Participants
- 41 children
- Outcome
- Long-term outcomes of SJIA-LD: survival, chronic oxygen or ventilator support, abnormal overnight oximetry
- Follow-up
- Last (median 2.9 years)
Main finding
As reported in the abstract
Alive at last follow-up
93%
Progressed to requiring chronic oxygen or other ventilator support
37%
Abnormal overnight oximetry studies, which changed over time
65%
Observational data: associations, not effects.
Further findings
Disease history
- At least one episode of macrophage activation syndrome
- 85%
- Adverse reactions to a biologic
- 41%
HLA-DRB1*15 haplotype
- Carried HLA-DRB1*15, significantly more than patients without LD
- 84%
Serum biomarkers
- Markedly elevated serum IL-18, variable CXCL9, and significantly elevated matrix metalloproteinase 7
Treatment and clinical course
- Variable use of anti-IL-1/6 biologics and addition of other immunomodulatory and lung-directed therapies
- Broad range of current clinical status independent of time from diagnosis or continued biologic treatment
- Imaging features least likely to improve with time
Original publication
Disease Course, Treatments, and Outcomes of Children With Systemic Juvenile Idiopathic Arthritis–Associated Lung Disease. Arthritis Care Res (Hoboken). Published 27 November 2023. doi:10.
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