A case of slowly progressive scleroderma kidney

Abstract

A rapidly progressive renal deterioration accompanied by acute-onset/uncontrolled hypertension characterizes scleroderma renal crisis (SRC), a life-threatening complication that occurs in patients with systemic sclerosis (SSc). To date, however, SSc with advanced renal failure has only rarely been reported in the absence of SRC. We report here an atypical case of diffuse cutaneous SSc where renal insufficiency progressed slowly to end-stage renal failure over a 14-year follow-up period after the diagnosis of SSc. In the renal biopsy, which was obtained at a relatively early stage of renal impairment, we found histological findings consistent with those of scleroderma kidneys. Unlike typical SRC, however, the larger renal arteries seemed to be unaffected. These histological findings were probably responsible for the “slowly progressive” renal impairment over the years without causing typical SRC.