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目的探讨IgG4相关性疾病眼部病变的临床及影像学特征。设计回顾性病例系列。研究对象25例在2010年10月-2014年5月期间首诊于眼科并经实验室和(或)活组织检查证实的IgG4相关性疾病患者。方法回顾性分析25例患者眼部病变的临床及影像学表现。主要指标临床表现,眼眶MRI,生长抑素受体SPECT/CT显像(SRS)。结果 25例患者双眼睑肿胀、眼球突出为主要临床表现,其中23例为双眼受累,22例患者有≥2个器官受累,眼外受累器官多见于鼻部(19例)和涎腺(13例)。眼眶MRI主要表现为弥漫性泪腺增大(22例)、眼外肌增粗(14例)、眶周软组织浸润(10例)等。SRS显示治疗前眼眶摄取示踪剂显著增高(UR=1.87±0.43);经免疫抑制治疗后活动期眼眶示踪剂摄取减少(UR=1.51±0.24)。结论 IgG4相关性疾病的眼部病变具有较典型的临床特征,泪腺弥漫性增大为眼眶MRI最常见表现,SRS能客观反映受累眼眶的免疫活动程度,对临床疗效评价有较好的价值。
Objective To investigate the clinical and imaging features of ocular lesions in IgG4-related diseases. Design retrospective case series. Participants Twenty-five patients with IgG4-related disease who were first diagnosed with ophthalmology and confirmed by laboratory and / or biopsy between October 2010 and May 2014 were enrolled. Methods The clinical and radiographic findings of ocular lesions in 25 patients were retrospectively analyzed. The main clinical manifestations, orbital MRI, somatostatin receptor SPECT / CT imaging (SRS). Results Twenty - five patients had double eyelid swelling and prominent eyeballs as the main clinical manifestations. Among them, 23 patients had binocular involvement, 22 patients had more than 2 organs involved, extraocular organs were mostly found in nasal region (19 cases) and salivary gland (13 cases ). Orbital MRI mainly showed diffuse lacrimal gland enlargement (22 cases), extraocular muscle thickening (14 cases), periorbital soft tissue infiltration (10 cases) and so on. SRS showed significantly increased orbital uptake tracer before treatment (UR = 1.87 ± 0.43); decreased orbit tracer uptake after active immunosuppression (UR = 1.51 ± 0.24). Conclusions The ocular lesions of IgG4-related diseases have typical clinical features. The diffuse increase of lacrimal gland is the most common manifestation of orbital MRI. SRS can objectively reflect the degree of orbital immune activity, which has a good value for clinical evaluation.