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Acute renal failure without fibrotic skin changes in progressive systemic sclerosis
P W Sanders1, G A Herrera, G V Ball
1Department of Medicine, University of Alabama, Birmingham.
Nephron
|January 1, 1988
Summary
Scleroderma renal crisis can occur in patients with progressive systemic sclerosis even without skin changes. This highlights the importance of considering scleroderma in unexplained kidney failure and hypertension.
Area of Science:
- Nephrology
- Rheumatology
- Internal Medicine
Background:
- Progressive systemic sclerosis (PSS) typically presents with characteristic dermal fibrosis.
- Scleroderma renal crisis (SRC) involves accelerated hypertension, hyperreninemia, and acute renal failure in PSS patients.
Observation:
- Two cases of PSS with SRC were identified.
- These patients lacked the typical fibrotic skin changes of scleroderma.
Findings:
- Progressive systemic sclerosis can manifest with scleroderma renal crisis even in the absence of cutaneous fibrosis.
- This challenges the traditional diagnostic reliance on skin manifestations.
Implications:
- Consider PSS in the differential diagnosis of unexplained acute renal failure and accelerated hypertension, regardless of skin findings.
- Broadens the diagnostic criteria for PSS, especially in renal presentations.
- Emphasizes the need for early recognition of SRC in at-risk patients.