Rheumatology (Oxford, England) · 2026
Baseline disease activity index and forced vital capacity predict clinically meaningful complications and mortality in systemic sclerosis.
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Yen Tsai-Hung, Isomura Yohei, Suzuki Mikito, Huang Wen-Nan, Kuwana Masataka
Аннотация
OBJECTIVE: The objective of this study was to evaluate whether the modified disease activity index (mDAI) predicts subsequent disease progression and organ damage in SSc.
METHODS: We analysed 227 patients diagnosed with SSc who were enrolled in a prospective SSc registry. Baseline disease activity was assessed using the modified EUSTAR disease activity index (mDAI), with active disease being defined as an mDAI of ≥2.5. The primary outcome was a composite of disease-related clinically meaningful complications-based on revised CRISS step 1 events (interstitial lung disease progression, precapillary pulmonary hypertension, scleroderma renal crisis, heart failure, severe digital ischaemia, or severe gastrointestinal dysfunction)-or all-cause mortality. Associations were examined using Cox proportional hazards models.
RESULTS: At baseline, 42 (18.5%) had active disease. During a median follow-up period of 38 months, 45 (19.7%) patients had experienced the primary outcome. Active disease was associated with a higher risk of events (log-rank P < 0.001). In multivariable analysis, baseline active disease [hazard ratio (HR) 2.21, 95% CI 1.06-4.59] and a lower forced vital capacity (HR 0.98 per %, 95% CI 0.96-0.99) independently predicted adverse outcomes. Results were consistent across sensitivity analyses using alternative end point definitions.
CONCLUSION: Baseline mDAI and forced vital capacity independently predict disease-related clinically meaningful complications and mortality in SSc. The mDAI provides a simple, feasible tool for risk stratification beyond progression of interstitial lung disease.
METHODS: We analysed 227 patients diagnosed with SSc who were enrolled in a prospective SSc registry. Baseline disease activity was assessed using the modified EUSTAR disease activity index (mDAI), with active disease being defined as an mDAI of ≥2.5. The primary outcome was a composite of disease-related clinically meaningful complications-based on revised CRISS step 1 events (interstitial lung disease progression, precapillary pulmonary hypertension, scleroderma renal crisis, heart failure, severe digital ischaemia, or severe gastrointestinal dysfunction)-or all-cause mortality. Associations were examined using Cox proportional hazards models.
RESULTS: At baseline, 42 (18.5%) had active disease. During a median follow-up period of 38 months, 45 (19.7%) patients had experienced the primary outcome. Active disease was associated with a higher risk of events (log-rank P < 0.001). In multivariable analysis, baseline active disease [hazard ratio (HR) 2.21, 95% CI 1.06-4.59] and a lower forced vital capacity (HR 0.98 per %, 95% CI 0.96-0.99) independently predicted adverse outcomes. Results were consistent across sensitivity analyses using alternative end point definitions.
CONCLUSION: Baseline mDAI and forced vital capacity independently predict disease-related clinically meaningful complications and mortality in SSc. The mDAI provides a simple, feasible tool for risk stratification beyond progression of interstitial lung disease.