title: Involvement of skeletal muscle in dialysis-associated systemic fibrosis (nephrogenic fibrosing dermopathy).
abstract: Nephrogenic fibrosing dermopathy (NFD), a newly recognized scleroderma-like disease, was originally described as a purely cutaneous disorder. More widespread involvement, including fibrosis of pulmonary and cardiac tissues, has been documented only recently, and it has been suggested that a more appropriate designation is dialysis-associated systemic fibrosis. We report five cases of this novel disorder, spanning a spectrum of primarily skin to primarily muscle involvement. Clinical, radiological, electrophysiological, and pathological studies revealed moderate to severe fibrosis of striated muscles. All patients had end-stage renal failure on chronic dialysis, subacute to chronic hardening of the skin and muscles, restriction of limb movements with joint contractures, but normal to only mildly weak muscle strength. Limitation of movements was caused predominantly by skin tightness and induration, and by joint contractures rather than muscle weakness. Computerized tomography showed fibrosis of the fascia and muscles in the most severely affected patients, and electromyography showed mild to severe myopathic changes. Histopathology of affected muscles revealed a spectrum of mild to severe fibrosis, degenerating fibers, and chronic inflammatory cells. These results further support the contention that NFD is not a purely cutaneous disease, but is part of a larger systemic fibrotic process that may involve muscles.
authors.affiliation: Department of Neurology, University of Pennsylvania Medical Center, Philadelphia, Pennsylvania, USA.
authors.forename: Joshua M
authors.initials: JM
authors.lastname: Levine
authors.forename: Robert A
authors.initials: RA
authors.lastname: Taylor
authors.forename: Lauren B
authors.initials: LB
authors.lastname: Elman
authors.forename: Shawn J
authors.initials: SJ
authors.lastname: Bird
authors.forename: Ehud
authors.initials: E
authors.lastname: Lavi
authors.forename: Ethan D
authors.initials: ED
authors.lastname: Stolzenberg
authors.forename: Michael L
authors.initials: ML
authors.lastname: McGarvey
authors.forename: Arthur K
authors.initials: AK
authors.lastname: Asbury
authors.forename: Sergio A
authors.initials: SA
authors.lastname: Jimenez
mesh_terms: D000328:Adult; D005260:Female; D005355:Fibrosis; D006801:Humans; D007676:Kidney Failure, Chronic / Q000150:complications / Q000473:pathology* / Q000503:physiopathology / Q000628:therapy; D008297:Male; D008875:Middle Aged; D018482:Muscle, Skeletal / Q000473:pathology*; D009135:Muscular Diseases / Q000150:complications / Q000473:pathology / Q000503:physiopathology; D006435:Renal Dialysis / Q000009:adverse effects*; D012595:Scleroderma, Systemic / Q000150:complications / Q000473:pathology* / Q000503:physiopathology