Systemic
sclerosis (SSc; also called scleroderma) refers to an autoimmune connective
tissue disease with autoantibodies, vasculopathy, and fibrosis.
Most patients have sclerodactyly (thickened skin of the fingers) and may or may not have more extensive skin fibrosis. There are large differences in the prevalence of internal organ involvement and other features, although nearly all have Raynaud phenomenon (RP), many have esophageal dysmotility, and 8% to 50% have pulmonary arterial hypertension (PAH), cardiac involvement, interstitial lung disease, inflammatory arthritis, and digital ulcers.