Background and Objective: Renal failure, pulmonary hypertension, and inters
titial lung disease are major causes of morbidity and mortality in systemic
sclerosis (SSc). However, the concomitant occurrence of pulmonary hemorrha
ge associated with acute renal failure in SSc has been rarely described. Th
e present study is the first analysis of pulmonary-renal syndrome in SSc.
Patient and Methods: We present a 44-year-old woman with SSc who died of a
fulminant course of acute renal failure associated with diffuse alveolar he
morrhage. We termed this uncommon and fatal complication of SSc scleroderma
-pulmonary-renal syndrome (SPRS). A search of the English-written literatur
e yielded reports of 10 additional similar cases. These patients, together
with our present case, form the basis of the present analysis.
Results: The average age of the patients with SPRS was 46 years. The majori
ty of the patients (80%) were women, and most had diffuse SSc. SPRS occurre
d an average of 6.4 years after disease onset and was associated with prior
fibrosing alveolitis and/or D-penicillamine treatment. Interestingly, norm
otensive renal failure seems to characterize the scleroderma patients, beca
use 9 of 11 (82%) had normal blood pressure. SPRS bears a poor prognosis: a
ll of the 11 patients (100%) died within 12 months of admission. However, o
nly 60% of the 5 patients for whom we have treatment data received corticos
Conclusions: Pulmonary-renal syndrome is a rare but fatal complication of S
Sc. Because the treatment data are scarce and the prognosis is poor, aggres
sive treatment with pulse corticosteroids, cyclophosphamide, and possibly p
lasmapheresis is suggested.
Semin Arthritis Rheum 30:403-410. Copyright (C) 2001 by W.B. Saunders Compa