Progressive systemic sclerosis and nephrotic syndrome. An unusual association resulting in postpartum acute renal failure

Abstract

A 27-year-old, full-term pregnant woman with progressive systemic sclerosis (PSS) came to the hospital with marked proteinuria and edema. Two days later, she gave birth to a normal baby. After delivery and during the next 48 hours, renal failure developed. A renal biopsy specimen disclosed findings characteristic of PSS, and immunofluorescence studies displayed nonspecific deposits of fibrinogen and complement. The patient's general condition deteriorated, with development of pericarditis and pulmonary failure; after several peritoneal dialysis treatments, a peritoneal infection developed, and the patient died of Gram-negative sepsis. The association of PSS and nephrotic syndrome is unusual.