Pathogenesis and staging of scleroderma

Scleroderma is reviewed as a complex disease of vascular, connective-tissue, and inflammatory reactions. If the scleroderma syndromes related to occupational, immunologic, inflammatory, metabolic, and genetic factors are recognized, then true progressive systemic scleroderma (PSS) appears to be a disease of vascular fibrosis with secondary inflammatory phases. The primary pathology appears to be a fibromucinous change of the vascular endothelium. The vessels also demonstrate altered reactivity to cold and to serotonin. Biopsy of the skin of the inflammatory or mesenchymal scleroderma may reveal (by positive direct immunofluorescence) deposition of globulins at the basement membrane zone. Evaluation of scleroderma is discussed and a system of clinical staging of PSS is proposed which relates the organ involvement to the prognosis.