19PClinico-patho-serological correlation for classification of idiopathic inflammatory myopathies and therapeutic outcomes - a real-world retrospective cohort

IF 2.8 4区医学 Q2 CLINICAL NEUROLOGY

Neuromuscular Disorders Pub Date : 2025-09-01 DOI:10.1016/j.nmd.2025.105483

S. Sivaraman Nair, J. George, R. Poyuran, D. Narasimhaiah

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Abstract

The classification of idiopathic inflammatory myopathies (IIMs) has undergone a paradigm shift with the application of muscle specific antibodies (MSAs) and biology-specific immunohistochemical stains. Many of these techniques are being gradually adopted in developing nations. We aimed to study the spectrum of subtypes in a retrospective cohort of IIMs with specific focus on the roles of clinical, pathological and serological evaluation and the therapeutic choices. We screened the electronic medical records between April 2017 to March 2024 for patients diagnosed as IIM with a follow up of at least one year. Patients where an alternate diagnosis could not be ruled out were excluded. We applied the European Neuromuscular Centre criteria for classification into dermatomyositis (DM), immune-mediated necrotizing myopathy (IMNM), antisynthetase syndrome (ASyS), and sporadic inclusion body myositis (sIBM). Those with a well-defined systemic autoimmune disease were classified as overlap myositis and the rest were labelled unclassified. Descriptive analysis of the clinical, investigational and therapeutic aspects of the subcategories were done. We identified 124 case records of IIM of whom 67 (53.7% females) were included for analysis. The mean age of onset was 42.1 (± 16.9) years with pediatric onset in 7 (10.4%). Symptom duration at presentation was 18.9 (35.3) months. Extramuscular features were seen in 19 (28.4%); 16 (23.9) with cutaneous manifestations, 3 (4.5%) each with arthritis and pulmonary disease and 1 (1.5%) with cardiomyopathy. Four (6%) had association with malignancies. The patient subclassification was DM in 14 (20.9%), IMNM in 11 (16.4%), ASyS in 3 (4.5%), sIBM in 15 (22.4%), and OM in 6 (8.9%) while 18 (26.9) were unclassified. Serology profile was incomplete in 7(38.9%) unclassified. Muscle biopsy was available for 61. Among DM, 6 were Mi2+, 3 NXP2+, and 2 MDA5+. Classical DM muscle pathology was recorded in 6. IMNM has 2 SRP+ and 3 HMGCR+ while ASyS had 2 Jo1+ and 1 PL-7+. Glucocorticoids were given in 97%, oral immunotherapies in 87.9%, and cyclophosphamide or rituximab were given in 25.3%. Excluding sIBM, 15.4% had a favourable immunotherapy response. The immunotherapy response did not differ between unclassified and other groups. Nearly three-fourths of the cohort could be classified into a specific IIM subcategory with judicious application of the criteria. Use of serology and biopsy were suboptimal in seronegative and unclassified groups. Higher therapeutic options were needed in a quarter.

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特发性炎性肌病分类与治疗结果的临床病理血清学相关性——一个真实世界的回顾性队列研究

随着肌肉特异性抗体（msa）和生物特异性免疫组织化学染色的应用，特发性炎症性肌病（IIMs）的分类经历了范式转变。其中许多技术正在发展中国家逐步采用。我们的目的是研究IIMs回顾性队列的亚型谱，特别关注临床、病理和血清学评估以及治疗选择的作用。我们筛选了2017年4月至2024年3月期间诊断为IIM的患者的电子病历，随访至少一年。不能排除其他诊断的患者被排除在外。我们应用欧洲神经肌肉中心的标准将其分为皮肌炎（DM）、免疫介导的坏死性肌病（IMNM）、抗合成酶综合征（ASyS）和散发性包涵体肌炎（sIBM）。那些有明确的系统性自身免疫性疾病的患者被归类为重叠肌炎，其余的被标记为未分类。对亚类的临床、研究和治疗方面进行描述性分析。我们收集了124例IIM病例，其中67例（53.7%为女性）纳入分析。平均发病年龄为42.1（±16.9）岁，其中儿科发病7例（10.4%）。症状持续时间为18.9（35.3）个月。肌肉外征19例（28.4%）；皮肤表现16例（23.9），关节炎和肺病各3例（4.5%），心肌病1例（1.5%）。4例（6%）与恶性肿瘤有关。其中DM 14例（20.9%），IMNM 11例（16.4%），ASyS 3例（4.5%），sIBM 15例（22.4%），OM 6例（8.9%），未分型18例（26.9%）。7例（38.9%）未分类患者血清学资料不完整。61例进行了肌肉活检。DM中Mi2+ 6例，NXP2+ 3例，MDA5+ 2例。6.典型DM肌肉病理记录。IMNM有2个SRP+和3个HMGCR+，而ASyS有2个Jo1+和1个PL-7+。糖皮质激素占97%，口服免疫治疗占87.9%，环磷酰胺或利妥昔单抗占25.3%。除sIBM外，15.4%的患者有良好的免疫治疗反应。免疫治疗反应在未分类组和其他组之间没有差异。近四分之三的队列可以被分类到一个特定的IIM子类别与明智的应用标准。在血清阴性和未分类组中，血清学和活检的使用是次优的。四分之一的人需要更高的治疗方案。

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来源期刊

Neuromuscular Disorders 医学-临床神经学

CiteScore

4.60

自引率

3.60%

发文量

543

审稿时长

53 days

期刊介绍： This international, multidisciplinary journal covers all aspects of neuromuscular disorders in childhood and adult life (including the muscular dystrophies, spinal muscular atrophies, hereditary neuropathies, congenital myopathies, myasthenias, myotonic syndromes, metabolic myopathies and inflammatory myopathies). The Editors welcome original articles from all areas of the field: • Clinical aspects, such as new clinical entities, case studies of interest, treatment, management and rehabilitation (including biomechanics, orthotic design and surgery). • Basic scientific studies of relevance to the clinical syndromes, including advances in the fields of molecular biology and genetics. • Studies of animal models relevant to the human diseases. The journal is aimed at a wide range of clinicians, pathologists, associated paramedical professionals and clinical and basic scientists with an interest in the study of neuromuscular disorders.