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Ischemic myopathy revealing systemic calciphylaxis
Author(s) -
Aouizerate Jessie,
ValleyrieAllanore Laurence,
Limal Nicolas,
Ayache S. Samar,
Gherardi Romain K.,
Audard Vincent,
Jerôme Authier François
Publication year - 2017
Publication title -
muscle and nerve
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.025
H-Index - 145
eISSN - 1097-4598
pISSN - 0148-639X
DOI - 10.1002/mus.25505
Subject(s) - calciphylaxis , medicine , muscle biopsy , myopathy , biopsy , dialysis , pathology , muscle weakness , transplantation , renal biopsy , atrophy , surgery , disease
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. Methods 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. Results 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. Conclusion 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