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The changing face of severe scleroderma in five patients
1Department of Medicine, Medical University of South Carolina, Charleston 29425.
Clinical and Experimental Rheumatology
|July 1, 1990
Summary
New antihypertensive drugs, like CEI, have shifted scleroderma renal disease. It now presents as a slow decline affecting kidneys, heart, and lungs, rather than acute, fatal kidney failure.
Area of Science:
- Nephrology
- Rheumatology
- Cardiology
Background:
- Scleroderma renal crisis traditionally presented as acute, severe kidney failure.
- Potent antihypertensive therapies have become standard in managing scleroderma.
Observation:
- Recent clinical experience shows a shift in the presentation of scleroderma renal involvement.
- The introduction of angiotensin I-II converting enzyme inhibitors (CEI) has coincided with this pattern change.
Findings:
- Scleroderma renal involvement now often manifests as an indolent, progressive decline.
- This decline affects multiple organs, including the kidneys, heart, and lungs concurrently.
- The pattern has evolved from acute, oliguric, and often fatal renal failure.
Implications:
- This evolving pattern necessitates a reevaluation of monitoring and treatment strategies for scleroderma.
- Long-term management should address the integrated decline of renal, cardiac, and pulmonary function.
- Understanding this transition is crucial for improving patient outcomes in scleroderma.