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.


Altman RD; Medsger TA Jr; Bloch DA; Michel BA. Predictors of survival in systemic sclerosis (scleroderma). Arthritis Rheum 1991 Apr;34(4):403-13

Brenot F. Primary pulmonary hypertension. Case series from France.

Chest 1994 Feb;105(2 Suppl):29S-32S

Chest 1994 Feb;105(2 Suppl):33S-36S

D'Alonzo GE et al. Survival in patients with primary pulmonary hypertension. Results from a national prospective registry. Ann Intern Med 1991 Sep 1;115(5):343-9

Hinderliter AL et al. Frequency and prognostic significance of pericardial effusion in primary pulmonary hypertension. PPH Study Group. Am J Cardiol 1999 Aug 15;84(4):481-4, A10

Kunieda T et al. Prognoses of primary pulmonary hypertension and chronic majorvessel thromboembolic pulmonary hypertension determined from cumulative survival curves. Intern Med 1999 Jul;38(7):543-6

McLaughlin VV et al. Compassionate use of continuous prostacyclin in the management of secondary pulmonary hypertension: a case series. Ann Intern Med. 2000 Jan 18;132(2):165

Oakley CW. Primary pulmonary hypertension. Case series from the United Kingdom.

Okada O et al. Prediction of life expectancy in patients with primary pulmonary hypertension. A retrospective nationwide survey from 1980-1990. Intern Med 1999 Jan;38(1):12-6