RESUMEN
Scleroderma renal crises (SRC) is a serious complication of systemic sclerosis whose prognosis remains serious despite management with angiotensin-converting enzyme inhibitors, antihypertensives and dialysis. Pulmonary renal syndrome (PRS), characterised by diffuse alveolar hemorrhage (DAH) and SRC, is rare and carries a grave prognosis. This case report discusses the clinicopathological features of a 43-year-old male presenting with severe hypertension and rapidly progressive renal failure who subsequently developed DAH and died. The clinical course, exhaustive investigative work-up and autopsy findings led to a diagnosis of diffuse systemic sclerosis with PRS subcategorized into PRS with thrombotic microangiopathy. The index case came without a prior diagnosis of systemic sclerosis, thereby posing a serious diagnostic challenge and management issues.
RESUMEN
Early mortality due to hepatitis C virus (HCV)-related liver failure in renal allograft recipients in the absence of fibrosing cholestatic hepatitis is reported infrequently. We report six renal allograft recipients with HCV infection who died of rapid progression to liver failure. Of these, 2 were detected anti-HCV positive at screening prior to kidney transplantation and 4 were diagnosed after transplantation following derangement of liver function (HCV RNA positive in all 4, anti-HCV positive in 2). Median interval between kidney transplantation and derangement of liver function was 11.8 months (range 2 to 25) and median interval between transplant and death was 27 months (range 11 to 53). Liver biopsy performed during the terminal illness in 3 patients and post-mortem liver histology in 2 patients showed chronic hepatitis with mean grade of 10.2 (range 9 to 12) and stage 2.4 (range 2 to 3). None had features of fibrosing cholestatic hepatitis.