Juxtaglomerular hyperplasia and hyperreninemia in progressive systemic sclerosis complicated by acute renal failure

The etiology of renal failure in progressive systemic sclerosis remains unexplained. In this patient with progressive systemic sclerosis rapidly progressive azotemia developed resulting in death. Kidney tissue obtained by percutaneous renal biopsy and later at autopsy revealed striking hyperplasia of the juxtaglomerular apparatus. The plasma renin activity measured by bioassay was extremely high. The clinical and morphologic findings in this patient suggest a possible pathophysiologic role for the renin-angiotensin system in the acute renal decompensation that occasionally occurs in progressive systemic sclerosis.