This raises blood pressure and further damages the renal vasculature, causing a vicious cycle of worsening hypertension and renal dysfunction (e.g., elevated creatinine, edema). Hypertensive emergency with end-organ dysfunction (e.g., encephalopathy, retinal hemorrhage) is common. Thrombocytopenia and microangiopathic hemolytic anemia can be seen. Urinalysis is usually normal but may show mild proteinuria, as in this patient; casts are unexpected.The mainstay of therapy for SRC includes ACE inhibitors, which reduce RAAS activity and improve renal function and blood pressure. Short-acting ACE inhibitors (typically captopril) are used because they can be rapidly uptitrated. An elevated serum creatinine level is not a contraindication for ACE inhibitors in this population, and slight elevations in creatinine are common during drug initiation. Scleroderma renal crisis, the occurrence of acute kidney injury, and malignant hypertension (very high blood pressure with evidence of organ damage) in people with scleroderma are effectively treated with drugs from the class of the ACE inhibitors. The benefit of ACE inhibitors extends even to those who have to commence dialysis to treat their kidney disease, and may give sufficient benefit to allow the discontinuation of renal replacement therapy. Lung disease
Active alveolitis is often treated with pulses of cyclophosphamide, often together with a small dose of steroids. The benefit of this intervention is modest.Pulmonary hypertension may be treated with epoprostenol, treprostinil, bosentan, and possibly aerolized iloprost.
Society and culture
Support groups
The Juvenile Scleroderma Network is an organization dedicated to providing emotional support and educational information to parents and their children living with juvenile scleroderma, supporting pediatric research to identify the cause of and the cure for juvenile scleroderma, and enhancing public awareness.In the US, the Scleroderma Foundation is dedicated to raise awareness of the disease and assist those who are affected.The Scleroderma Research Foundation sponsors research into the condition. Comedian and television presenter Bob Saget, a board member of the SRF, directed the 1996 ABC TV movie For Hope, starring Dana Delany, which depicts a young woman fatally affected by scleroderma; the film was based on the experiences of Sagets sister Gay.Scleroderma and Raynauds UK is a British charity formed by the merger of two smaller organisations in 2016 to provide support for people with scleroderma and fund research into the condition. Prognosis
A 2018 study placed 10-year survival rates at 88%, without differentiation based on subtype. Diffuse systemic sclerosis, internal organ complications, and older age at diagnosis are associated with worse prognoses. Research
Given the difficulty in treating scleroderma, treatments with a smaller evidence base are often tried to control the disease.