Systemic sclerosis (scleroderma)
Systemic sclerosis is a multisystem autoimmune disease combining small-vessel vasculopathy, immune activation and fibrosis; it can threaten the digits, lungs, pulmonary circulation, heart, kidneys and gut, so early specialist risk stratification and structured surveillance are essential.
Definition
Systemic sclerosis is a chronic multisystem autoimmune connective-tissue disease characterised by small-vessel vasculopathy, immune activation and fibrosis of skin and internal organs. Limited and diffuse cutaneous subsets describe the extent of skin involvement, but important cardiopulmonary, renal, gastrointestinal, digital and other complications may occur in either subset or in overlap disease.
First principles
Vasculopathy, autoimmunity and fibrosis act together
Endothelial injury and small-vessel dysfunction produce Raynaud phenomenon, digital ischaemia and susceptibility to pulmonary vascular and renal complications. Immune activation generates disease-associated autoantibodies and inflammation. Fibroblast activation deposits collagen in skin and organs, causing skin tightening, interstitial lung disease and gastrointestinal dysmotility. The clinical problem is the combination, not any one antibody or skin finding.
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