Scleroderma renal crisis: Case reports and update on critical issues.

Area of Science:

• Nephrology
• Rheumatology
• Internal Medicine

Background:

• Scleroderma renal crisis (SRC) is a serious complication of systemic sclerosis (SSc), associated with significant morbidity and mortality.
• Identifying predictors and understanding the role of medications like ACE inhibitors and corticosteroids in SSc patients at risk for SRC is crucial.
• Recent research suggests chronic kidney disease, systemic hypertension, and proteinuria may predict SRC, differing from prior reports.

Purpose of the Study:

• To review current understanding of SRC in systemic sclerosis (SSc).
• To discuss potential predictors and the role of drug exposure in SRC.
• To highlight the potential distinction between SRC and SSc-thrombotic microangiopathy (SSc-TMA) and present case studies.

Main Methods:

• Review of recent literature on SRC predictors and SSc-TMA.
• Analysis of risk factors, clinical presentation, and treatment of three SSc patients with SRC.
• Discussion of current therapeutic strategies for SRC.

Main Results:

• Chronic kidney disease, systemic arterial hypertension, and proteinuria are identified as potential predictors of SRC.
• SSc-thrombotic microangiopathy (SSc-TMA) is proposed as a distinct entity in some SRC cases, possibly involving complement pathway dysregulation.
• ACE inhibitors and rapid blood pressure control remain primary treatments for SRC.

Conclusions:

• SRC remains a life-threatening condition in SSc patients.
• Newer insights suggest specific risk factors and a potential distinction for SSc-TMA.
• Optimal management involves ACE inhibitors, blood pressure control, and consideration of plasma exchange in refractory SSc-TMA cases.