Eosinophilic myositis in calpainopathy: could immunosuppression of the eosinophilic myositis alter the early natural course of the dystrophic disease?

Neuromuscul Disord. 2009 Apr;19(4):261-3. doi: 10.1016/j.nmd.2009.01.010. Epub 2009 Mar 13.

Abstract

An 11-year-old girl with a calpain-3 gene (CAPN-3) mutation and eosinophilic myositis on muscle biopsy had high serum CK levels and eosinophil counts which showed spontaneous fluctuations. After commencement of immunosuppressive therapy reciprocal changes occurred in response to alterations in doses of the medications. Subacutely evolving and spreading muscle weakness developed during tapering of the immunosuppressive medications. These observations suggest that either the occurrence of eosinophilic myositis or the withdrawal of the immunosuppressive treatment may have accelerated the clinical course of the calpainopathy in this case. The positive effect of immunosuppressive therapy might have implications for the management of calpainopathy with an inflammatory component.

Publication types

  • Case Reports

MeSH terms

  • Azathioprine / administration & dosage
  • Azathioprine / adverse effects
  • Calpain / genetics*
  • Child
  • Creatine Kinase / analysis
  • Creatine Kinase / blood
  • Disease Progression
  • Dose-Response Relationship, Drug
  • Drug Therapy, Combination
  • Eosinophilia-Myalgia Syndrome / complications
  • Eosinophilia-Myalgia Syndrome / drug therapy
  • Eosinophilia-Myalgia Syndrome / immunology*
  • Eosinophils / pathology
  • Female
  • Genetic Predisposition to Disease / genetics
  • Humans
  • Immunosuppression / methods*
  • Immunosuppressive Agents / administration & dosage
  • Immunosuppressive Agents / adverse effects
  • Leukocyte Count
  • Methylphenazonium Methosulfate / administration & dosage
  • Methylphenazonium Methosulfate / adverse effects
  • Muscle Proteins / genetics*
  • Muscle Weakness / etiology
  • Muscle, Skeletal / immunology*
  • Muscle, Skeletal / metabolism
  • Muscle, Skeletal / pathology
  • Muscular Dystrophies, Limb-Girdle / drug therapy
  • Muscular Dystrophies, Limb-Girdle / immunology*
  • Muscular Dystrophies, Limb-Girdle / pathology
  • Myositis / drug therapy
  • Myositis / immunology*
  • Myositis / pathology
  • Prednisolone / administration & dosage
  • Prednisolone / adverse effects
  • Treatment Outcome

Substances

  • Immunosuppressive Agents
  • Muscle Proteins
  • Methylphenazonium Methosulfate
  • Prednisolone
  • Creatine Kinase
  • CAPN3 protein, human
  • Calpain
  • Azathioprine