title: Myopathy associated with anti-signal recognition peptide antibodies: clinical heterogeneity contrasts with stereotyped histopathology.
abstract: We report three patients with anti-signal recognition particle antibodies who had different presenting clinical pictures, mimicking acute polymyositis, limb-girdle muscular dystrophy, and acute rhabdomyolysis. Muscle biopsies typically showed necrotizing myopathy with little or no inflammation and deposits of membrane attack complex (C5b-9) in endomysial capillaries. The clinical course was severe in two patients and mild in one. The combination of corticosteroid with either an immunosuppressive agent or intravenous immunoglobulins was required to improve the condition of these patients.
authors.affiliation: Centre de Référence des Maladies Neuromusculaires, Service d'Histologie, CHU Henri Mondor, 51 Avenue du Marécal de Lattre de Tassigny, 94010 Créteil, France.
authors.forename: Dalia
authors.initials: D
authors.lastname: Dimitri
authors.forename: Chantal
authors.initials: C
authors.lastname: Andre
authors.forename: Jacques
authors.initials: J
authors.lastname: Roucoules
authors.forename: Hassan
authors.initials: H
authors.lastname: Hosseini
authors.forename: René-Louis
authors.initials: RL
authors.lastname: Humbel
authors.forename: François-Jérôme
authors.initials: FJ
authors.lastname: Authier
mesh_terms: D000305:Adrenal Cortex Hormones / Q000627:therapeutic use; D000328:Adult; D001323:Autoantibodies / Q000097:blood*; D015415:Biomarkers / Q000032:analysis / Q000378:metabolism; D001706:Biopsy; D015938:Complement Membrane Attack Complex / Q000276:immunology / Q000378:metabolism; D003937:Diagnosis, Differential; D018450:Disease Progression; D005260:Female; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D008297:Male; D008875:Middle Aged; D018485:Muscle Fibers, Skeletal / Q000276:immunology / Q000378:metabolism / Q000473:pathology; D018482:Muscle, Skeletal / Q000276:immunology* / Q000473:pathology* / Q000503:physiopathology; D009135:Muscular Diseases / Q000175:diagnosis* / Q000276:immunology* / Q000503:physiopathology; D049288:Muscular Dystrophies, Limb-Girdle / Q000175:diagnosis / Q000276:immunology / Q000503:physiopathology; D010641:Phenotype; D017285:Polymyositis / Q000175:diagnosis / Q000276:immunology / Q000503:physiopathology; D011237:Predictive Value of Tests; D012206:Rhabdomyolysis / Q000175:diagnosis / Q000276:immunology / Q000503:physiopathology; D018271:Signal Recognition Particle / Q000276:immunology*
chemical_list: D000305:Adrenal Cortex Hormones; D001323:Autoantibodies; D015415:Biomarkers; D015938:Complement Membrane Attack Complex; D007166:Immunosuppressive Agents; D018271:Signal Recognition Particle