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Systemic scleroderma, or systemic sclerosis, is an autoimmune rheumatic disease characterised by excessive production and accumulation of collagen, called fibrosis, in the skin and internal organs and by injuries to small aeries. There are two major subgroups of systemic sclerosis based on the extent of skin involvement: limited and diffuse. The limited form affects areas below, but not above, the elbows and knees with or without involvement of the face. The diffuse form affects also the skin above the elbows and knees and can spread also to the torso. Visceral organs, including the kidneys, hea, lungs, and gastrointestinal tract can also be affected by the fibrotic process. Prognosis is determined by the form of the disease and the extent of visceral involvement. Patients with limited systemic sclerosis have a 10-year survival rate of 75%; less than 10% develop pulmonary aerial hypeension after 10 to 20 years. Patients with diffuse cutaneous systemic sclerosis have a 10-year survival rate of 55%. Death is most often caused by lung, hea, and kidney involvement. There is also a slight increase in the risk of cancer Skin symptoms In the skin, systemic sclerosis causes hardening and scarring. The skin may appear tight, reddish, or scaly. Blood vessels may also be more visible. Where large areas are affected, fat and muscle wastage may weaken limbs and affect appearance. Patients repo severe and recurrent itching of large skin areas. The severity of these symptoms varies greatly among patients: Some having scleroderma of only a limited area of the skin (such as the fingers) and little involvement of the underlying tissue, while others have progressive skin involvement.Digital ulcers--open wounds on especially on fingeips and less commonly the knuckles--are not uncommon Diffuse scleroderma can cause musculoskeletal, pulmonary, gastrointestinal, renal and other complications. Patients with greater cutaneous involvement are more likely to have involvement of the internal tissues and organs. Most patients (over 80%) have vascular symptoms and Raynaud's phenomenon, which leads to attacks of discoloration of the hands and feet in response to cold. Raynaud's normally affects the fingers and toes. Systemic scleroderma and Raynaud's can cause painful ulcers on the fingers or toes which are known as digital ulcers. Calcinosis (deposition of calcium in lumps under the skin) is also common in systemic scleroderma, and is often seen near the elbows, knees or other joints. Musculoskeletal The first joint symptoms that patients with scleroderma have are typically non specific joint pains, which can lead to ahritis, or cause discomfo in tendons or muscles.Joint mobility, especially of the small joints of the hand, may be restricted by calcinosis or skin thickening.Patients may develop muscle weakness, or myopathy, either from the disease or its treatments. Lungs Some impairment in lung function is almost universally seen in patients with diffuse scleroderma on pulmonary function testing;however, it does not necessarily cause symptoms, such as shoness of breath. Some patients can develop pulmonary hypeension, or elevation in the pressures of the pulmonary aeries. This can be progressive, and can lead to right-sided hea failure. The earliest manifestation of this may be a decreased diffusion capacity on pulmonary function testing. Other pulmonary complications in more advanced disease include aspiration pneumonia, pulmonary hemorrhage and pneumothorax Ref Davidson 23rd edition pg 845
Acroosteolysis
Tufting
Calcinosis cutis
Digital ulcers
Immune system