The composition of cellular infiltrates in anti‐HMG‐CoA reductase‐associated myopathy

Chung, Tae Hwan; Christopher‐Stine, Lisa; Paik, Julie J; Corse, Andrea M.; Mammen, Andrew L.

doi:10.1002/mus.24642

Cited by 86 publications

(66 citation statements)

References 24 publications

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“…18 None of our SRP-IgG-and HMGCR-IgG-positive patients had cancer, despite reported cancer associations in the literature. 23,27,29 The largest risk factor we identified for NAM was statin exposure, which was documented in one-third of our patients, 12 positive for HMGCR-IgG and 4 for SRP-IgG (1 patient was dual seropositive). As previously reported, 6,9 atorvastatin and simvastatin were the most common statins, likely reflecting prescribing patterns.…”

Section: Discussionmentioning

confidence: 94%

Clinical Features and Treatment Outcomes of Necrotizing Autoimmune Myopathy

et al. 2015

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IMPORTANCE Necrotizing autoimmune myopathy (NAM) is characterized pathologically by necrotic muscle fibers with absent or minimal inflammation. It is often accompanied by statin therapy, connective tissue diseases, cancer, and autoantibodies specific for signal recognition particle (SRP) or 3-hydroxy-3-methylglutaryl-coenzyme A reductase (HMGCR). Data are limited concerning differences among etiologic subgroups and treatment outcomes in NAM. OBJECTIVES To describe the clinical, serologic, and electrophysiologic characteristics of NAM, compare patient subgroups, and determine clinical outcome predictors. DESIGN, SETTING, AND PARTICIPANTS We conducted a retrospective review of medical records for 63 adult Mayo Clinic patients assigned the clinical and histopathologic diagnosis of NAM from January 1, 2004, through December 31, 2013. Patients were stratified by presumed cause and autoantibody status. MAIN OUTCOMES AND MEASURES Clinical, electrophysiologic, and pathologic characteristics were collected and compared among patient subgroups. Predictors of response to treatment were identified by univariate logistic regression. RESULTS Lower extremity weakness predominated (46 [73%]). Distal weakness (26 [41%]), dysphagia (22 [35%]), and dyspnea (23 [37%]) were common. Twenty-two patients (35%) were receiving a statin medication at onset, 6 had cancer, and 3 had a connective tissue disease. The median creatine kinase level was 5326 U/L. In 13 patients (24%), SRP-IgG was detected, and in 17 patients (34%), HMGCR-IgG was detected (one-third of whom had not received statin medication). One patient was dual seropositive. Facial weakness was more common in SRP-IgG-positive patients. Myotonic discharges were more common in statin-associated NAM. Prednisone monotherapy was insufficient to control disease in most patients; 30 (90%) of 32 patients required 2 or more immunotherapeutic agents. Relapse occurred in 16 (55%) of 29 patients during immunosuppressant taper or discontinuation. Predictors of favorable outcome were male sex and use of 2 or more immunotherapeutic agents within 3 months of onset.CONCLUSIONS AND RELEVANCE Necrotizing autoimmune myopathy was idiopathic in half of this cohort with clinical and histopathologically defined disease. In the remainder, NAM was associated with statin medication, cancer, or connective tissue disease. One in 4 patients was SRP-IgG positive, and 1 in 3 was HMGCR-IgG positive. The disease was usually not controlled by corticosteroid monotherapy. Presentation, course, and outcomes did not differ significantly in seropositive, seronegative, and statin-associated cases. Early aggressive immunosuppressant therapy improved outcomes, and risk of relapse was high during medication dose reduction or withdrawal.

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Section: Discussionmentioning

confidence: 94%

Clinical Features and Treatment Outcomes of Necrotizing Autoimmune Myopathy

et al. 2015

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“…Muscle biopsy findings in NAM generally show necrotic and regenerating fibres with minimal inflammatory infiltrate, and no evidence of vasculitis [10,11].…”

Section: Introductionmentioning

confidence: 99%

Treatment and outcomes in necrotising autoimmune myopathy: An Australian perspective

Ashton

Junckerstorff

Bundell

et al. 2016

Neuromuscular Disorders

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“…6 In addition, the predominance of cytotoxic T-cells and myofiber MHC class I expression indicated an underlying cell-mediated autoimmune mechanism for the inflammation.…”

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confidence: 99%