Anti-SRP antibody-associated necrotizing myopathy: 2 clinical cases

IF 3.2 4区 医学 Q2 CLINICAL NEUROLOGY
F. A. Abbasov, G. V. Zemtsova, P. A. Popov, K. I. Chekhonatskaya, D. V. Kukhno, M. Severova, M. Shmyreva, A. A. Kindarova, D. Schekochikhin
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Abstract

Necrotizing myopathies are a subtype of autoimmune myopathies characterized by muscle fiber necrosis with minimal infiltration by inflammatory cells on muscle biopsy. This group of myopathies is defined by flaccid palsies due to prima‑ ry skeletal muscle damage as well as extramuscular manifestations such as fever, rash, arthritis, Raynaud’s syndrome and interstitial lung disease. The presence of anti-SRP antibodies is associated with rapidly progressive refractory myositis predominantly affecting limb muscles and axial muscles.Objective of the work is to analyze the course of severe, refractory to several lines of immunosuppressive therapies anti-SRP associated necrotizing myopathy and to highlight an adequate treatment regime.Necrotizing myopathy was suspected in patients aged 39 and 56 years with rapidly progressive flaccid tetraparesis on the basis of clinical and anamnestic data, the results of needle electromyography and muscle magnetic resonance imaging, as well as the analysis of myositis-specific and myositis-associated autoantibodies. In both cases, a rapid development of atrophies, marked muscle weakness in the limbs, without involvement of the bulbar musculature, was observed. To achieve effective control of the disease progression, several lines of therapy were required: glucocorticosteroids, intravenous immunoglobulins, methotrexate and rituximab. Our observations are consistent with those in the literature.Our observations illustrate the clinical course of severe myopathy associated with anti-SRP antibodies. Early initiation of aggressive immunosuppression is crucial to control the disease progression. Treatment and rehabilitation allow achieving significant improvement of the patient’s condition.
抗srp抗体相关性坏死性肌病2例临床分析
坏死性肌病是自身免疫性肌病的一种亚型,其特征是肌纤维坏死,肌肉活检显示炎症细胞浸润极少。这组肌病的定义是由于原发性骨骼肌损伤引起的弛弛性麻痹,以及肌肉外表现,如发烧、皮疹、关节炎、雷诺综合征和间质性肺疾病。抗srp抗体的存在与快速进展的难治性肌炎有关,主要影响肢体肌肉和轴向肌肉。本研究的目的是分析几种抗srp相关坏死性肌病免疫抑制疗法的严重难治性病程,并强调适当的治疗方案。根据临床和记忆资料、针肌电图和肌肉磁共振成像结果,以及肌炎特异性和肌炎相关自身抗体分析,39岁和56岁的快速进展性弛缓性四瘫患者疑似坏死性肌病。在这两种情况下,观察到萎缩的迅速发展,四肢明显的肌肉无力,没有累及球肌肉组织。为了有效控制疾病进展,需要几种治疗方法:糖皮质激素、静脉注射免疫球蛋白、甲氨蝶呤和利妥昔单抗。我们的观察结果与文献中的一致。我们的观察说明了与抗srp抗体相关的严重肌病的临床病程。早期开始积极的免疫抑制是控制疾病进展的关键。治疗和康复使患者的病情得到显著改善。
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来源期刊
Journal of neuromuscular diseases
Journal of neuromuscular diseases Medicine-Neurology (clinical)
CiteScore
5.10
自引率
6.10%
发文量
102
期刊介绍: The Journal of Neuromuscular Diseases aims to facilitate progress in understanding the molecular genetics/correlates, pathogenesis, pharmacology, diagnosis and treatment of acquired and genetic neuromuscular diseases (including muscular dystrophy, myasthenia gravis, spinal muscular atrophy, neuropathies, myopathies, myotonias and myositis). The journal publishes research reports, reviews, short communications, letters-to-the-editor, and will consider research that has negative findings. The journal is dedicated to providing an open forum for original research in basic science, translational and clinical research that will improve our fundamental understanding and lead to effective treatments of neuromuscular diseases.
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