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Neuromuscular involvement in nephrogenic systemic fibrosis
Salah Keyrouz1, Stacy A Rudnicki
1From the Department of Neurology, University of Arkansas for Medical Sciences, Little Rock, AR 72205, USA. sarudnicki@uams.edu
Journal of Clinical Neuromuscular Disease
|December 20, 2007
Summary
Nephrogenic Systemic Fibrosis (NSF) can affect nerves and muscles, not just skin. This neuromuscular involvement in NSF may be masked by skin and joint fibrosis, making clinical assessment challenging.
Area of Science:
- Nephrology
- Dermatology
- Neurology
Background:
- Nephrogenic fibrosing dermopathy, now termed Nephrogenic Systemic Fibrosis (NSF), is associated with renal failure and fibrotic skin changes.
- Emerging evidence suggests NSF extends beyond the skin, indicating widespread systemic involvement.
Observation:
- This case report details three patients with NSF exhibiting significant neuromuscular complications.
- Clinical presentation included progressive hardening of skin, fascia, and muscles, limiting joint mobility and complicating strength assessment.
Findings:
- Electromyography and nerve conduction studies indicated concurrent nerve and muscle involvement in NSF patients.
- Muscle biopsies revealed predominantly myopathic changes alongside variable degrees of fibrosis.
Implications:
- Neuromuscular involvement is a recognized feature of Nephrogenic Systemic Fibrosis.
- The clinical diagnosis of NSF-associated neuromuscular issues can be obscured by fibrotic changes in the skin and joints.
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