SScl - Systemic Sclerosis
Systemic Sclerosis - Pulmonary vascular disease and pulmonary hypertension
The incidence of pulmonary hypertension has varied from 10 to 80 percent in different reports, depending in part upon the methods used to detect it. It occurs most frequently as an isolated pulmonary complication of limited cutaneous systemic sclerosis (lcSSc) , but can be secondary to pulmonary fibrosis.
In the case of coincidence is difficult to assess which of both the pulmonary hypertension or the fibrosis will affect the outcome more.
The degree of impairment in gas transfer is prognostically important. Patients whose gas transfer is less than 50 percent of predicted have a much poorer prognosis than those with higher values. Life expectancy is less than one year in patients with gas transfer below 25 percent of normal. In the case of coexistance of PH and lung fibrosis DLCO corrected for ventilated alveolar volume is a better estimate.
Patients with diffuse cutaneous systemic sclerosis and pulmonary involvement, but no cardiac or renal disease, had a median survival of 78 months, with 50 percent being dead at eight years (Altman RD et al., 1991).
Patients with lcSSc who develop isolated pulmonary hypertension (approximately 10 percent of cases) have a mortality rate of 60 percent at five years.
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