Management review of scleroderma renal crisis: An update with practical pointers

• Published in: Modern rheumatology Vol. 33; no. 1; pp. 12 - 20
• Main Authors: Foocharoen, Chingching, Tonsawan, Pantipa, Pongkulkiat, Patnarin, Anutrakulchai, Sirirat, Mahakkanukrauh, Ajanee, Suwannaroj, Siraphop
• Format: Journal Article
• Language: English
• Published: England 03.01.2023
• Subjects: Acute Kidney Injury - etiology; Acute Kidney Injury - pathology; Acute Kidney Injury - therapy; Angiotensin-Converting Enzyme Inhibitors - therapeutic use; Humans; Kidney - pathology; Scleroderma, Localized; Scleroderma, Systemic - complications; Scleroderma, Systemic - drug therapy; Scleroderma, Systemic - pathology; thrombotic microangiopathy; vasculopathy; renal crisis; Systemic sclerosis; scleroderma
• ISSN: 1439-7595; 1439-7609
• DOI: 10.1093/mr/roac028

Scleroderma renal crisis (SRC) represents severe, fatal internal organ involvement brought on by systemic sclerosis. A high rate of renal replacement therapy and mortality persists despite various treatments. Depending on the stage of SRC, a vasodilator called 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 and systemic sclerosis-thrombotic microangiopathy. SRC prophylaxis using angiotensin-converting enzyme inhibitor 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.