Systemic sclerosis (scleroderma)

A non-contagious, chronic, multi-system disease.

Initial symptoms are non-specific and include fatigue, vague musculoskeletal complaints, diffuse swelling of hands, and Raynaud's phenomenon.

Aetiology and pathogenesis are unknown.

Disease course is variable, but the condition rarely subsides spontaneously.

There are two main subtypes of systemic sclerosis (SSc): limited cutaneous SSc and diffuse cutaneous SSc. The limited cutaneous form tends to have less severe internal organ involvement and a better prognosis, but these subjects still need to be followed closely for possible complications.

Clinical course is determined by extent of vascular and fibrosing complications. Vascular involvement includes Raynaud's phenomenon, ischaemic digital ulcers, hypertensive crisis, and pulmonary arterial hypertension. Fibrosis can involve lungs, heart, and gastrointestinal tract.

Treatment is targeted on disease processes that are potentially reversible (e.g., active inflammation or vasoconstriction) and aims to minimise functional impairment of the patient.

Systemic sclerosis (SSc), also known as scleroderma, is a multi-system, autoimmune disease, characterised by functional and structural abnormalities of small blood vessels, fibrosis of skin and internal organs, and production of auto-antibodies.