Sridhar S, Nashi Saraswati, Kulanthaivelu Karthik, Vengalil Seena, Baskar Dipti, Polavarapu Kiran, Preethish-Kumar Veeramani, Padmanabha Hansashree, Bardhan Mainak, Unnikrishnan Gopikrishnan, Huddar Akshata, Menon Deepak, Nittur Vidya, Rajanna Manoj, Bevinahalli Nandeesh, Thomas Aneesha, Keerthipriya Muddasu Suhasini, Gangadhar Yashwanth, Pratyusha P V, Saini Jitender, Mahadevan Anita, Nalini Atchayaram
Department of Neurology, Neuroscience Faculty Center, National Institute of Mental Health and Neurosciences (NIMHANS), Bengaluru 560029, India.
Department of Neurology, Neuroscience Faculty Center, National Institute of Mental Health and Neurosciences (NIMHANS), Bengaluru 560029, India.
Neuromuscul Disord. 2025 Feb;47:105257. doi: 10.1016/j.nmd.2024.105257. Epub 2024 Nov 30.
Idiopathic inflammatory myopathies (IIMs) constitute a group of immune-mediated disorders, affecting muscles. Our study aims to investigate the specific patterns of muscle involvement in subgroups of IIM. An ambispective and observational study was conducted. The evaluation encompassed clinical characterization, scales of MMT8, MDAAT, assessment of severity of edema/ fatty replacement using Modified Stramare (grades 1-5) & Modified Goutallier-Lamminen-Mercuri (GLM) scores, respectively on the muscle MRI of 125 patients with IIM. A total of 125 patients were categorized into four subgroups: dermatomyositis (DM), immune- mediated necrotizing myopathy (IMNM), overlap myositis (OM), & seronegative groups. Median age at presentation was 35 years. Median duration of illness was 8 months. In DM, 53.12% of patients showed grade 3 to grade 5 edema in adductor group. In the leg, 46.8% showed grade 3-5 edema in the deep flexors of leg & peronei, and 65.6% had significant fasciitis. In IMNM, 52.1% displayed grade 4-5 edema in rectus femoris & semimembranosus. Interestingly, in the leg, 78.2% of IMNM patients lacked edema in peroneii. For OM subgroup, there was no specific pattern of involvement. In seronegative subset, vastus lateralis showed grade 4-5 edema, while in the leg, deep posterior compartment and peroneal muscle group exhibited subtle to no edema. The involvement of peroneus muscles in leg emerged as a characteristic feature of DM, they were conspicuously spared in other subgroups. Mild muscle fatty replacement was observed in all groups except for IMNM, where it was more pronounced. The research broadens the imaging spectrum of inflammatory myositis & introduces innovative concepts of selective involvement of muscles.
特发性炎性肌病(IIM)是一组影响肌肉的免疫介导性疾病。我们的研究旨在调查IIM亚组中肌肉受累的具体模式。进行了一项前瞻性和观察性研究。评估包括临床特征、MMT8量表、MDAAT量表,分别使用改良的斯特拉马雷(1 - 5级)和改良的古塔利耶 - 拉明宁 - 梅尔库里(GLM)评分对125例IIM患者的肌肉MRI进行水肿/脂肪替代严重程度评估。总共125例患者被分为四个亚组:皮肌炎(DM)、免疫介导的坏死性肌病(IMNM)、重叠性肌炎(OM)和血清阴性组。就诊时的中位年龄为35岁。疾病的中位持续时间为8个月。在DM组中,53.12%的患者在内收肌群显示3至5级水肿。在腿部,46.8%的患者在小腿深层屈肌和腓骨肌显示3 - 5级水肿,65.6%有明显的筋膜炎。在IMNM组中,52.1%的患者在股直肌和半膜肌显示4 - 5级水肿。有趣的是,在腿部,78.2%的IMNM患者腓骨肌无水肿。对于OM亚组,没有特定的受累模式。在血清阴性亚组中,股外侧肌显示4 - 5级水肿,而在腿部,后深部间隙和腓骨肌群显示轻度至无水肿。腿部腓骨肌受累是DM的一个特征性表现,在其他亚组中明显未受累。除IMNM组外,所有组均观察到轻度肌肉脂肪替代,IMNM组更为明显。该研究拓宽了炎性肌炎的影像学谱,并引入了肌肉选择性受累的创新概念。