What is the prognosis of systemic sclerosis (SSc)?

Updated: Nov 11, 2020
  • Author: Robert A Schwartz, MD, MPH; Chief Editor: Dirk M Elston, MD  more...
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Answer

The prognosis depends on the type of systemic sclerosis (SSc). In lSSc, a patient's condition can be stable for years. However, in dSSc, the disease can rapidly lead to death, if it is not treated promptly. Pulmonary hypertension may be an important cause of mortality in these patients. Survival complicated by pulmonary hypertension remains poor despite currently available treatment options. [8] Pulmonary hypertension was documented 25% of elderly patients, yet only in 12% of the youngest ones in a large study that stressed the differences in early- versus late-onset systemic sclerosis. [9] Cigarette smoking has been found to reduce overall survival. [10]  In a multicenter French cohort study, age older than 60 years at diagnosis, diffuse cutaneous SSc, scleroderma renal crisis, dyspnea, 6-minute walking distance, forced vital capacity less than 70%, diffusing capacity of the lungs for carbon monoxide less than 70%, pulmonary hypertension, telangiectasia, valvular disease, malignancy, anemia, and C-reactive protein greater than 8mg/L were linked with poorer survival. [11]


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