A, Prevalence of main SSc-related clinical manifestations in the 3 SSc subsets (χ2 test or Fisher test as appropriate; P value for comparisons of ssSSc with lcSSc and ssSSc with dcSSc, detailed results and comparisons for other manifestations are provided in eTable 2 in Supplement 1). B, Comparison of overall survival in patients with ssSSc, lcSSc, and dcSSc (censored at after 15 years of follow-up). Shaded areas represent 95% CIs. ACA indicates anticentromere antibodies; dcSSc, diffuse cutaneous systemic sclerosis; DUs, digital ulcers; ILD, interstitial lung disease; lcSSc, limited cutaneous systemic sclerosis; PF, pulmonary fibrosis; PH, pulmonary hypertension; SRC, history of scleroderma renal crisis; SSc, systemic sclerosis; ssSSc, systemic sclerosis sine scleroderma.
aP < .001.
bP = .03.