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Scleroderma renal crisis, still a life-threatening complication
Luis Teixeira1, Alfred Mahr, Alice Berezné
1Department of Internal Medicine and Reference Center for Necrotizing Vasculitis and Systemic Sclerosis, Cochin Hospital, Assistance Publique-Hópitaux de Paris (AP-HP), and UPRES EA 4058, Paris-Descartes University, Faculty of Medicine, Paris, France.
Annals of the New York Academy of Sciences
|September 26, 2007
Summary
Scleroderma renal crisis (SRC) is a serious complication of systemic sclerosis (SSc), primarily affecting diffuse SSc patients early in disease. Prompt diagnosis and ACE inhibitor treatment significantly improve outcomes, though dialysis may be needed.
Area of Science:
- Nephrology
- Rheumatology
- Internal Medicine
Background:
- Scleroderma renal crisis (SRC) is a significant complication in systemic sclerosis (SSc).
- SRC predominantly affects patients with diffuse SSc within the first four years of disease onset.
- Corticosteroid use is an identified risk factor for SRC development.
Purpose of the Study:
- To review the key aspects of Scleroderma Renal Crisis (SRC) in Systemic Sclerosis (SSc).
- To highlight the improved prognosis with modern treatments.
- To emphasize the importance of early diagnosis and management.
Main Methods:
- Review of existing literature on Scleroderma Renal Crisis.
- Analysis of epidemiological data regarding SRC occurrence and risk factors.
- Evaluation of treatment outcomes and prognostic factors.
Main Results:
- SRC historically had a high mortality rate but prognosis has improved significantly.
- Angiotensin-converting enzyme (ACE) inhibitors are crucial for managing SRC.
- A substantial number of patients require dialysis, but one-third may discontinue it.
- Renal transplantation is an option for long-term dialysis patients.
Conclusions:
- Early diagnosis and prompt initiation of ACE inhibitors are critical for improving SRC outcomes in SSc patients.
- While ACE inhibitors are primary treatment, additional antihypertensive therapies are often necessary.
- Dialysis and renal transplantation remain important management options for advanced SRC.
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