特发性炎性肌病的恶性特征：Dr Cipto Mangunkusumo医院的回顾性研究

IF 2.8 4区医学 Q2 CLINICAL NEUROLOGY

Neuromuscular Disorders Pub Date : 2025-09-01 DOI:10.1016/j.nmd.2025.105493

L. Indrawati , M. Putri , W. Isaac , N. Shafitha , S. Simatupang , A. Safri , N. Fadli , A. Harsono , W. Wiratman , A. Budikayanti , F. Octaviana , M. Hakim

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引用次数: 0

摘要

特发性炎症性肌病（IIMs）可能与恶性肿瘤有关，因此伴随的恶性肿瘤治疗将改善IIM对免疫抑制剂的反应。本研究旨在研究Dr. Cipto Mangunkusumo医院IIM患者的恶性肿瘤特征。我们确定了2018年至2025年4月期间诊断为IIM的106例患者，使用国际肌炎评估和临床研究（IMACS） 2023标准、恶性发生率（证实或怀疑）和肌炎特异性抗体（MSA）收集恶性肿瘤风险分层数据。患者以女性（75例；70.7%）为主，年龄≥40岁（57例；53%）。皮肌炎（DM）是最常见的亚型（48例；45.3%），其次是多发性肌炎（PM）（25例；23.6%）、免疫介导坏死性肌病（IMNM）（19例；17.9%）、ASSD（3例；2.8%）和重叠性肌炎（OM）（24例；22.64%）。根据IMACS， 34例（32.7%）和30例（28.3%）患者分别处于高危和中度恶性肿瘤风险。恶性肿瘤16例（14.9%），包括肝细胞癌、卵巢癌、胃肠道腺癌、急性髓系白血病、淋巴瘤、肺癌、甲状腺癌和鼻咽癌。DM是最常见的相关亚型（13/16,81.25%）；在本组中，1例患者为青少年发病的糖尿病。2例恶性肿瘤发生在HCC和甲状腺癌的重叠肌炎（OM）患者中。大多数癌症（16例中有13例）在IIM发病后三年内被发现。TIF1-γ是恶性肿瘤中最常见的MSA。此外，12例患者（11.32%）接受了疑似肿瘤的评估，其中DM是最常见的相关亚型（41.67%）。与总体人群研究相似，DM是与恶性肿瘤相关的最普遍的IIM亚型，TIF1-γ是主要的MSA。及时的癌症筛查是必要的，特别是在高风险的IMACS患者中，因为即使是OM和SLE患者也可能有增加的恶性肿瘤风险。

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30PMalignancy characteristics among idiopathic inflammatory myopathies: a retrospective study at Dr Cipto Mangunkusumo hospital

Idiopathic inflammatory myopathies (IIMs) can be associated with malignancy, therefore concomitant malignancy management will improve the response of IIM to immunosuppressive agents. This study aimed to study the characteristics of malignancy among IIM patients at Dr. Cipto Mangunkusumo Hospital. We identified 106 patients diagnosed with IIM between 2018 and April 2025, collected data on malignancy risk stratification using International Myositis Assessment and Clinical Studied (IMACS) 2023 criteria, malignancy occurrence (proven or suspected), and myositis-specific antibodies (MSA). The patients were predominantly female (75; 70.7%) with (57; 53%) aged over 40 years. Dermatomyositis (DM) was the most common subtype (48; 45.3%), followed by Polymyositis (PM) (25; 23.6%), Immune-mediated necrotizing myopathy (IMNM) (19; 17.9%), ASSD (3; 2.8%), and Overlap myositis (OM) (24; 22.64%). According to IMACS, 34 (32.7%) and 30 (28.3%) patients were at high risk and moderate risk for malignancy, respectively. Malignancy was confirmed in 16 patients (14.9%), including cases of Hepatocellular carcinoma, Ovarian and Gastrointestinal adenocarcinoma, Acute myeloid leukemia, Lymphoma, Lung, Thyroid, and Nasopharyngeal cancers. DM was the most frequently associated subtype (13/16,81.25%); within this group, one patient was juvenile-onset DM. Two malignancies occurred in overlap myositis (OM) patients with HCC and Thyroid cancer. Most cancers (13 out of 16) were detected within three years after IIM onset. TIF1-γ was the most commonly detected MSA in malignancy cases. Additionally, 12 patients (11.32%) underwent evaluation for suspected neoplasm, with DM being the most common associated subtype (41.67%). Similar to overall population studies, DM was the most prevalent IIM subtype linked to malignancy, with TIF1-γ as the primary MSA. Prompt cancer screening is essential, especially in high-risk IMACS patients, as even those with OM and SLE may have an increased risk of malignancy.

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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.