Eosinophilic myositis in calpainopathy: could immunosuppression of the eosinophilic myositis alter the early natural course of the dystrophic disease?
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.
Oflazer
Piraye Serdaroglu
PS
Department of Neurology, Neuromuscular Unit, Istanbul University, Istanbul Medical Faculty, Capa, Istanbul 34390, Turkey. pirayes@istanbul.edu.tr
Gundesli
Hülya
H
Zorludemir
Suzan
S
Sabuncu
Tevfik
T
Dincer
Pervin
P
D001379:Azathioprine / Q000008:administration & dosage / Q000009:adverse effects; D002154:Calpain / Q000235:genetics*; D002648:Child; D003402:Creatine Kinase / Q000032:analysis / Q000097:blood; D018450:Disease Progression; D004305:Dose-Response Relationship, Drug; D004359:Drug Therapy, Combination; D016603:Eosinophilia-Myalgia Syndrome / Q000150:complications / Q000188:drug therapy / Q000276:immunology*; D004804:Eosinophils / Q000473:pathology; D005260:Female; D020022:Genetic Predisposition to Disease / Q000235:genetics; D006801:Humans; D007165:Immunosuppression Therapy / Q000379:methods*; D007166:Immunosuppressive Agents / Q000008:administration & dosage / Q000009:adverse effects; D007958:Leukocyte Count; D008773:Methylphenazonium Methosulfate / Q000008:administration & dosage / Q000009:adverse effects; D009124:Muscle Proteins / Q000235:genetics*; D018908:Muscle Weakness / Q000209:etiology; D018482:Muscle, Skeletal / Q000276:immunology* / Q000378:metabolism / Q000473:pathology; D049288:Muscular Dystrophies, Limb-Girdle / Q000188:drug therapy / Q000276:immunology* / Q000473:pathology; D009220:Myositis / Q000188:drug therapy / Q000276:immunology* / Q000473:pathology; D011239:Prednisolone / Q000008:administration & dosage / Q000009:adverse effects; D016896:Treatment Outcome
D007166:Immunosuppressive Agents; D009124:Muscle Proteins; D008773:Methylphenazonium Methosulfate; D011239:Prednisolone; D003402:Creatine Kinase; C105884:CAPN3 protein, human; D002154:Calpain; D001379:Azathioprine