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

OBJECTIVES: This study aimed to characterize the clinical phenotypes of adult-onset Still's disease-related lung disease (AOSD-LD) in a Chinese cohort, and to evaluate the associations of lung disease and distinct radiographic patterns with macrophage activation syndrome (MAS) and disease relapse. METHODS: In this cohort of 209 patients with AOSD, individuals were retrospectively stratified into AOSD-LD and non-lung disease groups based on radiographic findings. Clinical features associated with AOSD-LD were characterized. Multivariable Cox regression and subgroup analyses were performed to evaluate the impact of AOSD-LD on MAS and disease relapse. AOSD-LD was further categorized into acute exudative, interstitial, and suspected pulmonary hypertension (PH) patterns to assess pattern-specific outcomes. RESULTS: Lung involvement was identified in 49 (23.4%) patients, yet 69.4% were asymptomatic. Pleuritis emerged as the strongest independent risk factors of AOSD-LD (P<0.0001). AOSD-LD was independently associated with both MAS (HR = 2.62, 95% CI: 1.38-4.96, P=0.0041) and disease relapse (HR = 2.66, 95% CI: 1.50-4.71, P=0.0012) in multivariable COX analysis. Radiographically, the acute exudative pattern was most prevalent, followed by interstitial and suspected PH patterns. Prognostic stratification revealed the acute exudative pattern was strongly associated with early MAS development (P=0.0315), whereas the interstitial pattern was associated with a chronic, refractory course (P=0.0059). CONCLUSION: AOSD-LD defines a severe disease phenotype and serves as a critical independent predictor for life-threatening MAS and chronic relapse. Differentiating radiographic patterns offers valuable prognostic insights, underscoring the necessity for chest CT screening and phenotype-guided management strategies in AOSD.