Treatment Options for Scleroderma
Prognosis for Scleroderma
There is great variability among patients with scleroderma. Systemic scleroderma may progress very slowly in some patients and in others, especially diffuse scleroderma, may progress very rapidly. Patients with limited systemic scleroderma and CREST may remain with limited disease over many years. The 10-year survival rate for patients with CREST syndrome has been estimated as 75%. In general, the prognosis for limited scleroderma is good for the overwhelming majority of people. About 10-15% of patients with limited scleroderma develop debilitating lung disease over time.
Survival rates have improved over the past 20 years for patients with diffuse scleroderma partly because of earlier diagnosis and drug therapies to delay involvement of various organs. Most patients with diffuse scleroderma develop organ involvement at some point in varying degrees. Many progress towards organ failure over time which is the basis for a poorer prognosis. If organ complications appear early during the disease process, this is also a poor indicator of prognosis. Prognosis is also reduced if onset of diffuse scleroderma occurs after the age of 60, if there is anemia, a high sedimentation rate, or early cardiac involvement. The estimate of long-term survival (at least 10 years) for patients with diffuse scleroderma is approximately 55%.
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