• Lior Bear, Nancy Agmon Levine, Ronen Ghinea, Tammy Hod, Ido Nachmany, and Eytan Mor.
• Department of Surgery, Sheba Medical Center, Tel Hashomer, Israel, Faculty of Medicine, Tel Aviv University, Tel Aviv, Israel.
• Isr Med Assoc J. 2024 Oct 1; 26 (9): 593595593-595.

AbstractKidney involvement in systemic sclerosis (SSc) is common with altered kidney function present in approximately half of the patients [1]. Scleroderma renal crisis (SRC), the most severe kidney manifestation, occurs in about 20% of patients with this autoimmune disorder [1]. SRC mainly affects patients with the diffuse cutaneous systemic sclerosis (dcSSc) subtype of the disease, and particularly in those who are seropositive to anti RNA polymerase III antibodies [2]. In recent years, the prevalence of SRC has decreased following the initiation of medication therapy with angiotensin-converting-enzyme inhibitors (ACE-i). Previously, SRC mortality rates were as high as 78%. Contemporary studies in the post-ACE-i era suggest lower rates, with mortality rate ranging from 30% to 36% [3]. Nevertheless, progression to end-stage renal disease (ESRD) is evident and may require renal replacement therapies (RRTs). While renal transplant rates in SSc have increased, they constitute a small proportion of SSc-SRC patients (3-8%) and SSc-ESRD patients (4-17%).

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