Management Review of Scleroderma Renal Crisis: An Update with Practical pointers.

Scleroderma renal crisis (SRC) represents severe, fatal internal organ involvement brought on by systemic sclerosis (SSc). A high rate of renal replacement therapy and mortality persists despite various treatments. Depending on the stage of SRC, a vasodilator called ACEI (angiotensin converting enzyme inhibitor) is the treatment of choice. The efficacy of various other vasodilators (i.e., endothelin-1 receptor antagonist) and complement cascade blocker for SRC have been investigated, however, no randomized control trial has been conducted. A new approach has been proposed for the management of SRC; categorized by specific clinical features of narrowly defined SRC (ndSRC) and SSc-thrombotic microangiopathy (SSc-TMA). SRC prophylaxis using ACEI might be harmful, leading to a poor renal outcome, so the pathogenesis of SRC needs to be clarified in order to identify other possible preventions or therapies.