Marotta Dario A, Zadourian Adena, Jabaay Maxwell J, Kesserwani Ali, Kesserwani Hassan
Department of Research, Alabama College of Osteopathic Medicine, Dothan, USA.
Department of Neurology, Division of Neuropsychology, University of Alabama, Birmingham, USA.
Cureus. 2020 Apr 25;12(4):e7827. doi: 10.7759/cureus.7827.
Immune-mediated necrotizing myopathy (IMNM) is a rare idiopathic disease that is further classified by the presence of serum antibodies. A modicum of patients lack serum autoantibodies. Significantly elevated creatine kinase (CK) is highly characteristic of IMNM. The pathophysiology of IMNM is partially understood, and effective treatment options are limited, particularly in patients without serum autoantibodies. In this case, we report a 76-year-old male presenting with a four-month history of proximal muscle weakness. Muscle biopsy and serology confirmed the diagnosis of autoantibody-negative IMNM. Early and aggressive treatment with high-dose steroids and a course of intravenous immunoglobulin significantly reduced the patient's symptoms and CK within three months. This case serves as an example of an effective treatment outcome in a patient with this rare idiopathic necrotizing myopathy.
免疫介导性坏死性肌病（IMNM）是一种罕见的特发性疾病，可根据血清抗体的存在进一步分类。少数患者缺乏血清自身抗体。肌酸激酶（CK）显著升高是IMNM的高度特征。IMNM的病理生理学部分已知，有效的治疗选择有限，尤其是在无血清自身抗体的患者中。在此病例中，我们报告一名76岁男性，有4个月近端肌无力病史。肌肉活检和血清学检查确诊为自身抗体阴性的IMNM。大剂量类固醇和一个疗程的静脉注射免疫球蛋白的早期积极治疗在三个月内显著减轻了患者症状并降低了CK水平。该病例是这种罕见特发性坏死性肌病患者有效治疗结果的一个实例。
