Systemic sclerosis (scleroderma) (SSc) is a rare chronic connective tissue disorder characterized by
widespread microvascular damage and excessive deposition of collagen in the skin and internal organs. The etiology is unknown.
Disease manifestation usually starts at the age of 40-50, and the estimated prevalence is 1/6500 adults. Although more common in women, SSc appears as strikingly more severe in men; there is a higher risk of severe cardiovascular involvement in men.
Clinical features of Systemic sclerosis are:
There is no cure for SSc, no treatments exist which affect the hallmark of the disease, fibrosis.
Management consists of controlling symptoms and preventing complications.
Regular monitoring and reviews are aimed at early detection and treatment of complications, including regular review of symptoms, blood pressure, renal function, lung function tests and chest CT scan, ECG and echocardiography.