Scleroderma renal crisis is still a life-threatening syndrome.
Scleroderma renal crisis (SRC) was known as a rare and catastrophic syndrome responsible for acute renal failure (ARF) in a context of widespread microvascular disease occurring in progressive systemic sclerosis (PSS). Following pathogenetic hypoteses, angiotensin converting enzyme (ACE) inhibitors, plasma infusions (PI), and plasma-exchange (PE) have been employed in SRC with favorable results. Our purpose was to verify whether these therapies have consistently changed the fatal prognosis of SRC, even in our experience. In the last 10 years, SRC was diagnosed in eight patients (all eight with histologic data). The first five cases were treated with steroids, antihypertensive-cocktail, and PI: all five died, two within 48 hours, three after 10, 15, and 300 days, respectively. Three other patients were treated with ACE inhibitors, PI, and PE: all three died after 1, 9, and 12 months of HD. Clinical-histological correlations showed a strong relationship between the extent of glomerular involvement and the degree of renal failure, while arterial lesions seem to be more related to the past history of PSS, independently from the previous existence of hypertension. We conclude that "true" SRC diagnosed by restrictive criteria is still a rare life-threatening syndrome, and, unfortunately, no clear predictive biochemical or clinical signs could be identified; vascular renal involvement correlates to the duration of PSS independently of previous clinical evidence of renal failure or hypertension; a glomerular pattern similar to that reported for hemolytic-uremic syndrome (HUS)/thrombotic thrombocytopenic purpura (TTP) syndrome is directly related to the degree of acute renal involvement; SRC may occur even in the absence of hypertension, mainly if cardiomyopathy is present: in our experience. ACE inhibitors and plasma therapies have changed the short-time prognosis of SRC, but they may be unable to provide recovery from dialysis and do not avoid further evolution of extrarenal PSS exiting in late death.