title: Nephrogenic systemic fibrosis presenting as myopathy: a case report with histopathologic correlation.
abstract: Nephrogenic systemic fibrosis is primarily a skin disorder associated with renal insufficiency and exposure to gadolinium-containing (GAD+) contrast. We present the case of a 64-year-old man who was exposed to gadolinium while in acute renal failure, and months later developed limb stiffness, proximal weakness, and woody muscle texture. Muscle biopsy demonstrated chronic non-inflammatory fibrosing myopathy. CD34+ fibroblasts have previously been reported to be specific for nephrogenic systemic fibrosis dermopathy, and we found these in fibrotic areas of muscle and fascia. Nephrogenic systemic fibrosis is an emerging disorder, and our case highlights that it may present as a progressive myopathy with minimal skin findings.
authors.affiliation: Department of Neurology, Oregon Health & Science University, Portland, OR, USA.
authors.forename: Eric
authors.initials: E
authors.lastname: Edgar
authors.forename: Randy
authors.initials: R
authors.lastname: Woltjer
authors.forename: Ruth
authors.initials: R
authors.lastname: Whitham
authors.forename: S Humayun
authors.initials: SH
authors.lastname: Gultekin
authors.forename: Suzanne
authors.initials: S
authors.lastname: Watnick
authors.forename: Edward J
authors.initials: EJ
authors.lastname: Cupler
mesh_terms: D018952:Antigens, CD34 / Q000235:genetics; D001706:Biopsy; D003287:Contrast Media / Q000009:adverse effects; D005347:Fibroblasts / Q000473:pathology; D005355:Fibrosis; D005682:Gadolinium / Q000009:adverse effects; D006801:Humans; D007668:Kidney / Q000473:pathology*; D008279:Magnetic Resonance Imaging; D008297:Male; D008875:Middle Aged; D018908:Muscle Weakness / Q000209:etiology / Q000473:pathology; D018482:Muscle, Skeletal / Q000473:pathology; D054989:Nephrogenic Fibrosing Dermopathy / Q000235:genetics / Q000473:pathology*; D009431:Neural Conduction / Q000502:physiology; D011115:Polyneuropathies / Q000473:pathology
chemical_list: D018952:Antigens, CD34; D003287:Contrast Media; D005682:Gadolinium