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Neuromuscular Involvement in Nephrogenic Systemic Fibrosis

Introduction: Nephrogenic fibrosing dermopathy causes fibrotic skin changes in the setting of renal failure. Although this condition was initially felt to be limited to the skin, recent studies have described widespread involvement, and the term nephrogenic systemic fibrosis (NSF) has been suggested. Objective: To describe the clinical, electrophysiologic, and pathologic findings in three NSF patients with neuromuscular involvement. Methods: Case report. Results: Three patients developed progressive hardening of the skin and underlying fascia and muscles. Strength was difficult to assess because of limited joint mobility. electromyography/nerve conduction studies suggested involvement of both nerve and muscle. Muscle biopsy demonstrated primarily myopathic changes with variable fibrosis. Conclusion: Neuromuscular involvement occurs in NSF and may be difficult to appreciate clinically because of joint and skin fibrosis.