title: Ischemic myopathy revealing systemic calciphylaxis.
abstract: Patients with renal failure who are being treated with dialysis frequently develop neuromuscular manifestations. Renal failure-associated calciphylaxis, also termed calcific uremic arteriolopathy (CUA), is a life-threatening condition usually observed in patients with end-stage renal disease on chronic dialysis or after renal transplantation.
  
  
  
  We describe a hemodialyzed patient who presented with rapidly progressive unexplained systemic vasculopathy, muscle atrophy, and proximal weakness, that unexpectedly proved to be caused by calciphylaxis.
  
  
  
  Quadriceps muscle biopsy disclosed diffuse vascular calcific deposits on medium- and small-sized vessels, characteristic of CUA. Other changes included ischemic myopathy, focal intracellular calcium accumulation within myofibers, and calcium deposits in endomysial capillaries associated with marked complement activation and C5b9 formation.
  
  
  
  There are only a few descriptions of muscle involvement in the context of CUA, a condition with a prognosis that depends on early diagnosis and treatment. This report underscores the usefulness of muscle biopsy in the diagnosis of systemic calciphylaxis. Muscle Nerve 56: 529-533, 2017.
authors.affiliation: Reference Center for Neuromuscular Diseases, Hôpital Henri Mondor, Assistance Publique des Hôpitaux de Paris (APHP); INSERM U955-Team 10, Université Paris Est-Créteil, 94 Avenue du Marechal de Lattre de Tassigny, 94010, Creteil, France.|Transplantation and Nephrology Department, Hôpital Henri Mondor, APHP, Institut Francilien de recherche en Néphrologie et Transplantation, INSERM U955, Université Paris Est-Créteil, Créteil, France.
authors.forename: Jessie
authors.initials: J
authors.lastname: Aouizerate
authors.affiliation: Dermatology Department, Hôpital Henri Mondor, APHP, Créteil, France.
authors.forename: Laurence
authors.initials: L
authors.lastname: Valleyrie-Allanore
authors.affiliation: Internal Medicine Department, Hôpital Henri Mondor, APHP, Créteil, France.
authors.forename: Nicolas
authors.initials: N
authors.lastname: Limal
authors.affiliation: Clinical Neurophysiology Department, Hôpital Henri Mondor, APHP, EA4391, Université Paris Est-Créteil, Créteil, France.
authors.forename: S Samar
authors.initials: SS
authors.lastname: Ayache
authors.affiliation: Reference Center for Neuromuscular Diseases, Hôpital Henri Mondor, Assistance Publique des Hôpitaux de Paris (APHP); INSERM U955-Team 10, Université Paris Est-Créteil, 94 Avenue du Marechal de Lattre de Tassigny, 94010, Creteil, France.
authors.forename: Romain K
authors.initials: RK
authors.lastname: Gherardi
authors.affiliation: Transplantation and Nephrology Department, Hôpital Henri Mondor, APHP, Institut Francilien de recherche en Néphrologie et Transplantation, INSERM U955, Université Paris Est-Créteil, Créteil, France.
authors.forename: Vincent
authors.initials: V
authors.lastname: Audard
authors.affiliation: Reference Center for Neuromuscular Diseases, Hôpital Henri Mondor, Assistance Publique des Hôpitaux de Paris (APHP); INSERM U955-Team 10, Université Paris Est-Créteil, 94 Avenue du Marechal de Lattre de Tassigny, 94010, Creteil, France.
authors.forename: François
authors.initials: F
authors.lastname: Jerôme Authier
mesh_terms: D002115:Calciphylaxis / Q000150:complications / Q000175:diagnosis*; D006801:Humans; D007511:Ischemia / Q000150:complications / Q000175:diagnosis*; D016030:Kidney Transplantation / Q000639:trends; D008297:Male; D008875:Middle Aged; D009135:Muscular Diseases / Q000150:complications / Q000175:diagnosis*; D052097:Quadriceps Muscle / Q000473:pathology; D006435:Renal Dialysis / Q000639:trends; D056647:Systemic Vasculitis / Q000150:complications / Q000175:diagnosis*
keywords: calciphylaxis; chronic renal failure; complement; ischemic myopathy; muscle biopsy; von Kossa staining