Scleroderma renal crisis and renal involvement in systemic sclerosis

Area of Science:

• Nephrology
• Rheumatology
• Internal Medicine

Background:

• Scleroderma renal crisis (SRC) is a severe complication affecting 2-15% of systemic sclerosis (SSc) patients.
• Typically occurs early in diffuse cutaneous SSc, within 3-5 years of symptom onset.
• Characterized by hypertension, rising creatinine, oliguria, and thrombotic microangiopathy.

Purpose of the Study:

• To review advancements in identifying and managing patients at risk of SRC.
• To provide recommendations for optimizing care in SRC patients, especially those with chronic kidney failure.

Main Methods:

• Review of current literature on Scleroderma renal crisis.
• Analysis of treatment strategies and patient outcomes.
• Discussion of risk factors and proactive management approaches.

Main Results:

• Angiotensin-converting-enzyme inhibitors have significantly improved SRC prognosis since the 1980s.
• Despite treatment, 1-year survival is 70-82%, decreasing to 50-60% at 5 years.
• Renal transplantation is an option for SRC patients with non-recovering renal function.

Conclusions:

• Proactive identification and management are crucial for improving SRC outcomes.
• Optimizing blood pressure control and considering renal replacement therapy are key.
• Further research is needed to enhance long-term survival for SSc patients with SRC.