以眼球运动障碍为首要表现的IgG4相关疾病的临床特征

Clinical features of IgG4-related diseases with oculomotor impairment as first sign

目的总结以眼球运动障碍为首要表现的IgG4相关疾病(IgG4-RD)的临床特征。设计回顾性病例系列。研究对象2020年12月至2021年12月北京同仁医院以眼球运动障碍为首要表现的IgG4-RD患者8例。方法回顾患者的病历资料,包括眼位、眼球运动障碍方向、血清IgG4水平、眼眶磁共振成像(MRI)眼外肌表现、病理检查结果、治疗方案等。主要指标临床表现、眼眶MRI及病理结果。结果8例(100%)患者均表现为眼球运动障碍,其中外展受限87.5%、上转受限75.0%、下转受限37.5%、内收受限37.5%;视力下降5例(62.5%),头痛/眼痛3例(37.5%)。眼眶MRI显示局限性眼上肌群和外直肌增粗伴强化2例(25.0%),局限性眼上肌群和外直肌受压2例(25.0%),弥漫性眼外肌群增粗伴强化3例(37.5%),眼外肌正常但颅脑MRI示脑膜增厚伴强化1例(12.5%)。患者均合并神经系统外部位受累,表现为泪腺增大5例(62.5%)、鼻窦黏膜增生肥厚4例(50.0%)、淋巴结反应性增生4例(50.0%)。眼眶肿物、鼻窦黏膜、胸腺组织病理中均可见大量IgG4阳性浆细胞,伴组织纤维化或闭塞性静脉炎。结论以眼球运动障碍为首要表现的IgG4-RD的临床特征以眼球内下斜位、外展受限、上转受限常见,影像学多示眼上肌群和外直肌增粗或受压伴强化。

Objective To summarize the clinical features of patients with IgG4-related diseases(IgG4-RD)with oculomotor impairment as first sign.Design Retrospective case series.Participants From December 2020 to December 2021,8 IgG4-RD patients with oculomotor impairment as first sign were collected in Beijing Tongren Hospital.Method The medical record data of all patients were reviewed,including primary eye position,the directions of oculomotor impairment,serum IgG4 level,abnormal extraocular muscles according to orbital MRI and treatment methods.Pathological examination results were also reviewed from orbital masses,sinus mucosa or thymus tissue.Main Outcome Measures Clinical features,abnormal extraocular muscles according to orbital MRI,pathological results.Results All 8 patients(100%)presented with oculomotor impairment,including abduction impairment 87.5%,upgaze impairment 75.0%,downgaze impairment and addcution impairment 37.5%respectively.The other main clinical manifestations were visual declines(62.5%)and headaches(37.5%).According to orbital MRI,the local supraocular muscles and lateral rectus muscle thickened with enhancement in 2 patients(25.0%),the local supraocular muscles and lateral rectus muscle were oppressed in 2 patients(25.0%),the diffused extraocular muscles thickened with enhancement in 3 patients(37.5%),extraocular muscles were normal but meningeal thickened with enhancement in 1 patient(12.5%).All patients were accompanied with other part involvements besides nervous system,including lacrimal gland enlargements(62.5%),paranasal mucosa hypertrophies(50.0%)and lymph node reactive hyperplasia(50.0%).All biopsy specimens from orbital masses,nasal mucosae or thymus tissue revealed a large number of IgG4 positive plasma cells with tissue fibrosis or obliterans phlebitis.Conclusion Hypotropia and esotropia in primary eye position and abduction impairment and upgaze impairment are the most common clinical features of IgG4-RD with oculomotor impairment as the first sign.The supraocular muscles and the lateral rectus muscle usually thickened or were oppressed with enhancement in imaging examination.