Histopathologic Findings in Idiopathic Orbital Myositis

Histopathologic Findings in Idiopathic Orbital Myositis
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DOI:
10.1016/j.ophtha.2020.09.012
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发表时间:
2021-03-18
期刊:
影响因子:
13.7
通讯作者:
McNab, Alan A.
McNab, Alan A.
中科院分区:
医学1区
文献类型:
--
作者:
Ben Artsi, Elad;Mckelvie, Penelope A.;McNab, Alan A.

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目的:报告 20 年来接受眼外肌 (EOM) 活检的特发性眼眶肌炎 (IOM) 患者的组织学和临床特征,为预期的组织病理学结果提供第一个系统参考,并讨论与常见鉴别诊断的组织病理学差异。设计:队列研究。参与者:包括 2000 年至 2019 年期间接受 EOM 活检的所有诊断为 IOM 的患者。排除最终诊断不同的患者。方法:回顾所有参与者的 EOM 组织样本和病历。主要指标:肌肉活检的组织病理学特征,包括组织形态特征和细胞组成。结果:13 名患者符合纳入和排除标准,并对他们的组织样本进行了重新审查。 9 名患者的组织病理学结果表明 IOM 的结论性诊断,研究重点针对他们。就诊时的平均年龄为 49 岁,66.7% 的患者为女性。最常进行活检的 EOM 是内直肌 (44.4%)。活检最常见的指征是未解决的眼眶疾病,对皮质类固醇反应不足(44.4%),或由于已知预先存在的全身恶性肿瘤或除了肌肉增大外还存在非典型眼眶肿块(44.4%)而高度怀疑恶性肿瘤。提示 IOM 诊断的组织病理学结果是肌内膜中炎症浸润 (n = 9) 和轻度纤维化 (n = 8) 导致肌纤维张开或替换肌纤维,无肉芽肿或血管炎。确定的炎症浸润是慢性炎症细胞,由淋巴细胞 (n = 9)、浆细胞 (n = 6) 和组织细胞 (n = 6) 组成。其他不太常见的细胞是嗜酸性粒细胞 (n = 4)、多形核细胞 (n = 1) 和巨细胞 (n = 1)。 5 名患者出现明显的肌纤维变性或再生。最初队列中的 4 名患者在翻修时表现出不确定的组织学结果,并被重新分配为疑似 IOM。 结论:IOM 中受累肌肉的组织病理学特征类似于特发性眼眶炎症中所见的特征,但与常见鉴别诊断中所见的特征不同。当眼眶肌炎表现不典型或可能存在重大潜在疾病时,应强烈考虑进行眼外肌活检。 (C) 2020 年美国眼科学会
Purpose: To report the histologic and clinical features of idiopathic orbital myositis (IOM) patients who underwent extraocular muscle (EOM) biopsy over 20 years, to provide the first methodical reference to the expected histopathologic findings, and to discuss the histopathologic differences from common differential diagnoses.Design: Cohort study.Participants: All patients with a diagnosis of IOM who underwent EOM biopsy from 2000 through 2019 were included. Patients who had a different final diagnosis were excluded.Methods: Tissue samples of EOM and medical records of all participants were reviewed.Main Outcome Measures: Histopathologic features of muscle biopsy, including tissue morphologic features and cellular composition.Results: Thirteen patients met both inclusion and exclusion criteria, and their tissue samples were revisited. Nine patients showed histopathologic findings that suggested a conclusive diagnosis of IOM, and the study focused on them. The average age at presentation was 49 years, and 66.7% of patients were women. The most commonly biopsied EOM was the medial rectus (44.4%). The most common indications for biopsy were nonresolving orbital disease with inadequate response to corticosteroids (44.4%) or a high suspicion of malignancy because of known pre-existing systemic malignancy or the presence of an atypical orbital mass in addition to enlarged muscles (44.4%). The histopathologic findings that suggested a diagnosis of IOM were splaying of muscle fibers by inflammatory infiltrates (n = 9) and mild fibrosis (n = 8) in the endomysium or replacing muscle fibers, with no granulomas or vasculitis. The inflammatory infiltrates identified were of chronic inflammatory cells, consisting of lymphocytes (n = 9), plasma cells (n = 6), and histiocytes (n = 6). Other less commonly identified cells were eosinophils (n = 4), polymorphonuclears (n = 1), and giant cells (n = 1). Muscle fiber degeneration or regeneration was evident in 5 patients. Four patients from the initial cohort showed inconclusive histologic findings on revision and were reassigned as suspected IOM.Conclusions: The histopathologic features of involved muscles in IOM resemble those seen in idiopathic orbital inflammation and differ from those seen in common differential diagnoses. Extraocular muscle biopsy should be strongly considered whenever the presentation of orbital myositis is not typical or when significant underlying conditions are a possibility. (C) 2020 by the American Academy of Ophthalmology