Nintedanib was approved for use in the United States Food and Drug Administration on September 6, 2019, to slow the rate of decline in pulmonary function in patients with systemic sclerosis-associated interstitial lung disease (SSc-ILD). Other
Some evidence indicates that plasmapheresis (therapeutic plasma exchange) can be used to treat the systemic form of scleroderma. In Italy, it is a government-approved treatment option. This is done by replacing blood plasma with a fluid consisting of albumin, and is thought to keep the disease at bay by reducing the circulation of scleroderma autoantibodies. Epidemiology
Systemic scleroderma is a rare disease, with an annual incidence that varies in different populations. Estimates of incidence (new cases per million people) range from 3.7 to 43 in the United Kingdom and Europe, 7.2 in Japan, 10.9 in Taiwan, 12.0 to 22.8 in Australia, 13.9 to 21.0 in the United States, and 21.2 in Buenos Aires. The interval of peak onset starts at age 30 and ends at age 50.Globally, estimates of prevalence vary from 31.0 to 658.6 affected people per million. Systemic sclerosis has a female:male ratio of 3:1 (8:1 in mid- to late childbearing years). Incidence is twice as high among African Americans. Full-blooded Choctaw Native Americans in Oklahoma have the highest prevalence in the world (469 per 100,000).The disease has some hereditary association. It may also be caused by an immune reaction to a virus (molecular mimicry) or by toxins.
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 arteries. 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 also affects the skin above the elbows and knees and can also spread to the torso. Visceral organs, including the kidneys, heart, 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 better prognosis than those with the diffuse form. Death is most often caused by lung, heart, and kidney involvement. The risk of cancer is increased slightly.Survival rates have greatly increased with effective treatment for kidney failure. Therapies include immunosuppressive drugs, and in some cases, glucocorticoids. Signs and symptoms
Calcinosis, Raynauds phenomenon, Esophageal dysfunction, Sclerodactyly, and Telangiectasia (CREST syndrome) are associated with limited scleroderma. Other symptoms include:

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 report severe and recurrent itching of large skin areas.