14 .慢性免疫介导坏死性肌病的临床病理特点

IF 2.8 4区医学 Q2 CLINICAL NEUROLOGY

Neuromuscular Disorders Pub Date : 2025-09-01 DOI:10.1016/j.nmd.2025.105478

S. Komaki , A. Kubota , M. Maeda , J. Shimizu , A. Yamanaka , I. Nishino , T. Toda

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引用次数: 0

摘要

一些免疫介导的坏死性肌病（IMNM）表现为慢性病程；然而，其临床病理特征尚不清楚。我们纳入339例HMGCR-IMNM和481例SRP-IMNM，比较慢性病例（病程≥2年）和急性病例（病程≤3个月）的临床特征、MRI表现和病理表现。对于MRI评估，评估水肿，脂肪替代和萎缩的存在。病理检查采用苏木精-伊红染色和免疫染色（HLA-ABC、p62、C5b9）进行半定量评价。本研究纳入HMGCR-IMNM慢性88例、急性66例，SRP-IMNM慢性56例、急性80例。在慢性病例中，发病年龄较年轻，肌肉萎缩更为普遍，血清CK水平较低，肌肉症状较轻。在病理检查中，慢性病例表现出更突出的慢性改变，如肌内膜纤维化、脂肪浸润、纤维肥大，而纤维大小变化和坏死纤维较少。矛盾的病理结果表明，慢性病例是一个异质组。接下来，我们对慢性病例进行了聚类分析。因此，病例分为三组：有明显的慢性肌病改变、肌肉萎缩和严重下肢无力的病例（第1组）；轻度肌病改变和轻度肌肉症状（聚类2）；慢性肌病改变伴炎性细胞浸润（第3组）。影像学分析显示慢性病例表现为更多的脂肪替代和萎缩，水肿较少。其中，集群1表现出明显的下肢萎缩。总之，慢性IMNM是一个异质性群体，可分为三组，具有不同的临床病理特征。需要进一步的调查来评估各组之间的差异。

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14PClinicopathological characteristics of chronic cases of immune-mediated necrotizing myopathy

Some cases of immune-mediated necrotizing myopathy (IMNM) show chronic courses; however, their clinicopathological features are not well understood. We included 339 cases of HMGCR-IMNM and 481 cases of SRP-IMNM and compared the clinical features, the MRI findings, and the pathological findings between chronic cases (disease duration ≥ 2 years) and acute cases (disease duration ≤ 3 months). For MRI evaluation, the presences of edema, fatty replacement, and atrophy were evaluated. For the pathological examination, hematoxylin-eosin staining and immunostaining (HLA-ABC, p62, and C5b9) were semi-quantitatively evaluated. The study included 88 chronic and 66 acute cases of HMGCR-IMNM and 56 chronic and 80 acute cases of SRP-IMNM. In chronic cases, the age of onset was younger, muscle atrophy was more prevalent, serum CK levels were lower, and muscle symptoms were milder. On the pathological examinations, chronic cases showed more prominent chronic changes such as endomysial fibrosis, fatty infiltration, and hypertrophic fibers, whereas fiber size variation and necrotic fibers were less frequent. The paradoxical pathological findings suggested that chronic cases are a heterogeneous group. Next, we performed a cluster analysis on chronic cases. Consequently, cases were classified into three groups: cases with marked chronic myopathic changes, muscle atrophy and severe lower limb weakness (Cluster 1); cases with mild myopathic changes and mild muscle symptoms (Cluster 2); and cases with chronic myopathic changes with inflammatory cell infiltration (Cluster 3). Imaging analysis revealed that chronic cases showed more fatty replacement and atrophy, with less edema. Among the clusters, Cluster 1 showed prominent atrophy in the lower limbs. In conclusion, chronic IMNM is a heterogeneous group and classified into three groups with distinct clinicopathological features. Further investigation is needed to assess the differences among groups.

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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.