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The scleroderma kidney: progress in risk factors, therapy, and prevention
Guillaume Bussone1, Alice Bérezné, Vincent Pestre
1Pôle de Médecine Interne, Hôpital Cochin, 27 Rue du Faubourg Saint-Jacques, 75679 Paris Cedex 14, France.
Current Rheumatology Reports
|November 10, 2010
Summary
Scleroderma renal crisis (SRC) is a severe complication of systemic sclerosis (SSc), leading to kidney failure and hypertension. Early ACE inhibitor treatment and avoiding corticosteroids in diffuse SSc patients can improve outcomes.
Area of Science:
- Nephrology
- Rheumatology
- Internal Medicine
Background:
- Scleroderma renal crisis (SRC) is a life-threatening complication of systemic sclerosis (SSc).
- SRC presents with severe hypertension and acute kidney injury, carrying significant mortality.
- It affects 2-5% of SSc patients, especially those with diffuse cutaneous SSc early in the disease course.
Purpose of the Study:
- To summarize the characteristics, treatment, and prevention strategies for Scleroderma Renal Crisis.
- To highlight the association between certain therapies and SRC risk.
- To inform clinical management and preventative measures for SSc patients at risk of SRC.
Main Methods:
- Review of retrospective studies and clinical observations regarding SRC.
- Analysis of treatment outcomes with angiotensin-converting enzyme inhibitors and dialysis.
- Identification of risk factors, including corticosteroid use and antibody presence.
Main Results:
- High-dose corticosteroid therapy is linked to an increased risk of SRC.
- Anti-RNA-polymerase III antibodies are found in one-third of SRC patients.
- Early blood pressure control with ACE inhibitors is crucial; dialysis and transplantation are options for advanced cases.
Conclusions:
- Prompt management of hypertension with ACE inhibitors is key for SRC.
- Corticosteroids and nephrotoxic drugs should be avoided in diffuse cutaneous SSc patients to prevent SRC.
- Further consensus on SRC prevention strategies is needed, but risk factor avoidance is paramount.
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