Systemic sclerosis & Raynaud's

Baseline disease activity index and forced vital capacity predict clinically meaningful complications and mortality in systemic sclerosis

Rheumatology (Oxford)Published Prospective cohort study

Main finding · Primary composite outcomeResult — go to the resultHazard ratio (multivariable) 2.21 95% CI 1.06–4.59

Authors' conclusion

Baseline mDAI and forced vital capacity independently predict disease-related clinically meaningful complications and mortality in SSc.

Study context

Population
Patients diagnosed with SSc enrolled in a prospective SSc registry45 (19.7%) had primary outcome
Participants
227 patients
Exposure
Active disease vs inactive disease at baseline
  • Active diseasen = 42
Outcome
Composite of disease-related clinically meaningful complications (revised CRISS step 1 events:
  • ILD progression
  • Precapillary pulmonary hypertension
  • Scleroderma renal crisis
  • HF
  • Severe digital ischaemia
  • Severe gastrointestinal dysfunction) or all-cause mortality
Follow-up
Median 38 months
Methodological detailsprospective SSc registry, multivariable Cox proportional hazards models, sensitivity analyses with alternative end point definitions
  • Prospective SSc registry
  • Multivariable Cox proportional hazards models
  • Sensitivity analyses with alternative end point definitions

Main finding

Primary composite outcome

Hazard ratio (multivariable)

2.21

95% CI 1.06–4.59

Active diseasevs Inactive disease
HR (multivariable) 2.21 (95% CI 1.06–4.59)

As reported in the abstract

Patients with the primary outcome (overall)
45 (19.7%)

Observational data: associations, not effects.

Further findings

Lower FVC as predictor of primary composite outcome

HR per % FVC (multivariable), 95% CI 0.96-0.99; lower FVC independently predicted adverse outcomes
0.98

Sensitivity analyses with alternative end point definitions

Results consistent across sensitivity analyses

Original publication

Baseline disease activity index and forced vital capacity predict clinically meaningful complications and mortality in systemic sclerosis. Rheumatology (Oxford). Published 4 September 2026. doi:10.1093/rheumatology/keag481

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