Autoinflammatory & rare systemic diseases

Lung disease in adult-onset Still’s disease: a Chinese cohort study

Rheumatology (Oxford)Published Retrospective cohort study

Main finding · MASResult — go to the resultHazard ratio 2.62 95% CI 1.38–4.96

Authors' conclusion

AOSD-LD defines a severe disease phenotype and serves as a critical independent predictor for life-threatening MAS and chronic relapse.

Study context

Population
Chinese cohort of patients with AOSD
Participants
209 participants
Exposure
AOSD-related lung disease vs no lung disease
  • AOSD-LDn = 49
Outcome
MAS and disease relapse
Methodological detailsmultivariable Cox regression, subgroup analyses
  • Multivariable Cox regression
  • Subgroup analyses

Main finding

MAS

Hazard ratio

2.62

95% CI 1.38–4.96

P = 0.0041

AOSD-LDvs Non-lung disease
HR 2.62 (95% CI 1.38–4.96); P = 0.0041

As reported in the abstract

Observational data: associations, not effects.

Further findings

OutcomeHR (95% CI)P value
Disease relapse (multivariable Cox)2.66 (1.50–4.71)P = 0.0012

Lung involvement in the AOSD cohort (prevalence)

Lung involvement identified
49 (23.4%)
Asymptomatic
69.4%

Risk factor for AOSD-LD

Pleuritis was the strongest independent risk factor of AOSD-LD
P < 0.0001

Radiographic patterns of AOSD-LD and outcomes

Acute exudative pattern strongly associated with early macrophage activation syndrome (MAS) development
P = 0.0315
Interstitial pattern associated with a chronic, refractory course
P = 0.0059
Acute exudative pattern most prevalent, followed by interstitial and suspected pulmonary hypertension (PH) patterns

Original publication

Lung disease in adult-onset Still’s disease: a Chinese cohort study. Rheumatology (Oxford). Published 17 July 2026. doi:10.1093/rheumatology/keag375

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