Pediatric rheumatology

Clinical characteristics and prognosis of interstitial lung disease in systemic juvenile idiopathic arthritis: a two-center retrospective observational cohort study

Pediatr Rheumatol Online JPublished Retrospective cohort study

Authors' conclusion

Patients with SJIA-ILD exhibited heightened inflammation, increased hip joint and cervical spine involvement, and were more susceptible to developing lymphopenia and MAS, suggesting a relatively poor prognosis.

Study context

Population
Children newly diagnosed with sJIA, two centres in China, October 2010 to December 2021
Participants
176 children
Exposure
Systemic juvenile idiopathic arthritis (SJIA) with interstitial lung disease (ILD) vs SJIA without ILD
  • ILDn = 35
  • Non-ILDn = 141reference
Outcome
Clinical inactive disease without GC within 6 to 12 months of treatment
Follow-up
6 to 12 months
Methodological detailstwo-center
  • Two-center

Main finding

As reported in the abstract

P = 0.006

Observational data: associations, not effects.

Further findings

OutcomeILDP value
Cervical spine involvement28.6%P = 0.031
Hip involvement40.0%P = 0.029
Macrophage activation syndrome (MAS)40%P = 0.047
Lymphopenia · more prone in ILD
P = 0.009
Combination therapy required · more in ILD
P = 0.006
Disease relapse rates
P > 0.05
Deaths from respiratory failure (ILD, non-remission)
2 · events

Complete remission and non-remission in ILD · median follow-up 6.0 years (range 3.9-9.5)

Complete remission
54.3%
Non-remission
17.1%

Disease relapse rates

No significant difference between the two groups

Original publication

Clinical characteristics and prognosis of interstitial lung disease in systemic juvenile idiopathic arthritis: a two-center retrospective observational cohort study. Pediatr Rheumatol Online J. Published 24 October 2024. doi:10.1186/s12969-024-01028-5

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