54VPDistinct histopathological features of juvenile idiopathic inflammatory myopathies: a quantitative comparative study

IF 2.8 4区医学 Q2 CLINICAL NEUROLOGY

Neuromuscular Disorders Pub Date : 2025-09-01 DOI:10.1016/j.nmd.2025.105517

F. Hakim , S. Sakka , M. Snoussi , O. Boudawara , K. Moalla , N. Bouattour , S. Daoud , N. Charfi , L. Sellami , S. Marzouk , T. Boudawara , M. Damak

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引用次数: 0

Abstract

Juvenile idiopathic inflammatory myopathies (JIIM), defined by onset before 18 years of age, are rare autoimmune disorders that can present with distinct clinical and serological profiles. However, specific histopathological features in JIIM remain underexplored. This study aims to identify histological characteristics distinct to JIIM through a quantitative comparative analysis. We conducted a retrospective study on patients diagnosed with IIM at a Tunisian neurology center, following the 2017 ACR-EULAR criteria. Histopathological features were analysed through a review of available slides, including fiber atrophy, necrosis, ischemia, regeneration, fibrosis, and inflammatory infiltrates, graded with a predefined scoring system. Available muscle samples were collected and examined for immunohistochemical markers (CD4, CD8, CD20, CD31, CD68, anti-MxA). Histological findings in juvenile patients were collected compared to those in adults. We included 13 juvenile cases from 100 IIM patients (13%). The mean age of JIIM was 12± 5,2 years (range: 3–17). Sex ratio: 0.18. JIIM included 10 dermatomyositis patients (2 anti-Mi2, 1 anti-NXP-2, 1 seronegative) and 3 overlap myositis (1 scleromyositis, 1 lupus-associated myositis, 1 anti-Jo-1 anti-synthetase syndrome). Juvenile onset was correlated with severe fiber atrophy (38.4%, p=0.024) with a perifascicular atrophy extended into the endomysium (53.8%, p=0.01). It was also associated with severe inflammatory infiltrates (46%, p=0.02), primarily in the perivascular region (84.6%, p=0.23). However, inflammatory cell types, vascular injury, and MXA staining did not show a specific pattern related to juvenile onset. JIIM is characterized by distinct histopathological features, including pronounced inflammatory infiltrates and perifascicular atrophy. These findings highlight the age-specific mechanisms in JMII, which may be associated with more severe outcomes.

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青少年特发性炎性肌病的不同组织病理学特征：一项定量比较研究

青少年特发性炎症性肌病（JIIM），定义为18岁前发病，是一种罕见的自身免疫性疾病，具有独特的临床和血清学特征。然而，JIIM的具体组织病理学特征仍未得到充分研究。本研究旨在通过定量比较分析，找出与JIIM不同的组织学特征。我们根据2017年ACR-EULAR标准，对突尼斯神经病学中心诊断为IIM的患者进行了回顾性研究。通过回顾现有的切片分析组织病理学特征，包括纤维萎缩、坏死、缺血、再生、纤维化和炎症浸润，并按照预先设定的评分系统进行分级。收集可用肌肉样本，检测免疫组织化学标志物（CD4、CD8、CD20、CD31、CD68、抗mxa）。收集了青少年患者与成人患者的组织学结果。我们从100例IIM患者中选取了13例青少年病例（13%）。JIIM的平均年龄为12±5.2岁（范围3-17岁）。性别比：0.18。JIIM纳入10例皮肌炎患者（2例抗mi2, 1例抗nxp -2, 1例血清阴性）和3例重叠肌炎患者（1例硬化肌炎，1例狼疮相关性肌炎，1例抗jo -1抗合成酶综合征）。青少年发病与严重纤维萎缩相关（38.4%,p=0.024），筋束周围萎缩延伸至肌内膜（53.8%,p=0.01）。它还与严重的炎症浸润（46%,p=0.02）相关，主要发生在血管周围区域（84.6%,p=0.23）。然而，炎症细胞类型、血管损伤和MXA染色并没有显示出与幼年发病相关的特定模式。JIIM具有明显的组织病理学特征，包括明显的炎症浸润和筋膜周围萎缩。这些发现强调了JMII的年龄特异性机制，这可能与更严重的结果有关。

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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.