Myopathy associated with anti-signal recognition peptide antibodies: clinical heterogeneity contrasts with stereotyped histopathology

Muscle Nerve. 2007 Mar;35(3):389-95. doi: 10.1002/mus.20693.

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.

Publication types

  • Case Reports
  • Research Support, Non-U.S. Gov't

MeSH terms

  • Adrenal Cortex Hormones / therapeutic use
  • Adult
  • Autoantibodies / blood*
  • Biomarkers / analysis
  • Biomarkers / metabolism
  • Biopsy
  • Complement Membrane Attack Complex / immunology
  • Complement Membrane Attack Complex / metabolism
  • Diagnosis, Differential
  • Disease Progression
  • Female
  • Humans
  • Immunosuppressive Agents / therapeutic use
  • Male
  • Middle Aged
  • Muscle Fibers, Skeletal / immunology
  • Muscle Fibers, Skeletal / metabolism
  • Muscle Fibers, Skeletal / pathology
  • Muscle, Skeletal / immunology*
  • Muscle, Skeletal / pathology*
  • Muscle, Skeletal / physiopathology
  • Muscular Diseases / diagnosis*
  • Muscular Diseases / immunology*
  • Muscular Diseases / physiopathology
  • Muscular Dystrophies, Limb-Girdle / diagnosis
  • Muscular Dystrophies, Limb-Girdle / immunology
  • Muscular Dystrophies, Limb-Girdle / physiopathology
  • Phenotype
  • Polymyositis / diagnosis
  • Polymyositis / immunology
  • Polymyositis / physiopathology
  • Predictive Value of Tests
  • Rhabdomyolysis / diagnosis
  • Rhabdomyolysis / immunology
  • Rhabdomyolysis / physiopathology
  • Signal Recognition Particle / immunology*

Substances

  • Adrenal Cortex Hormones
  • Autoantibodies
  • Biomarkers
  • Complement Membrane Attack Complex
  • Immunosuppressive Agents
  • Signal Recognition Particle