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Renal complications and scleroderma renal crisis
C P Denton1, G Lapadula, L Mouthon
1Centre for Rheumatology, Royal Free Hospital, Rheumatology Unit, London, UK. c.denton@medsch.ucl.ac.uk
Rheumatology (Oxford, England)
|June 3, 2009
Summary
Scleroderma renal crisis (SRC) affects 5-10% of SSc patients, causing hypertension and kidney failure. Early ACE inhibitor treatment improves outcomes, but dialysis and mortality remain significant concerns.
Area of Science:
- Nephrology
- Rheumatology
- Internal Medicine
Background:
- Scleroderma renal crisis (SRC) is a serious complication affecting 5-10% of patients with systemic sclerosis (SSc).
- Risk factors include diffuse cutaneous or rapidly progressive SSc and high-dose corticosteroid use.
- SRC presents with abrupt hypertension, acute renal failure, and systemic symptoms.
Observation:
- Laboratory findings may include hypercreatinaemia, microangiopathic hemolytic anemia (MAHA), thrombocytopenia, and hyperreninemia.
- Specific autoantibodies, such as anti-RNA polymerase I and II, and a speckled ANA pattern are associated with SRC.
- Absence of anti-centromere antibodies is also noted in patients with SRC.
Findings:
- Early and aggressive treatment with angiotensin-converting enzyme (ACE) inhibitors has improved the prognosis of SRC.
- Despite treatment, 40% of patients may require dialysis, with a 5-year mortality rate of 30-40%.
- Recovery typically occurs within 1-3 years, though prognosis is worse for males.
Implications:
- Modification of endothelin overactivity presents a potential future therapeutic strategy for SRC.
- Understanding the pathogenesis and risk factors is crucial for early detection and management of SRC.
- Continued research into novel treatments is necessary to further improve outcomes for SSc patients experiencing SRC.
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