IgG4相关性疾病眼部病变的临床及影像学特征分析

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。 （眼科，2015, 24： 309-312）  

Analysis of the clinical and imaging features of ocular lesions of IgG4-related disease

Objective To investigate the clinical and imaging features of ocular lesions of IgG4-related disease(IgG4-RD) . Design Retrospective case series. Participants Twenty-five patients presented to ophthalmology department with IgG4-RD confirmed by pathology and／or laboratory examinations. Methods The clinical manifestations and imaging characteristics were analyzed. Main Outcome Measures Clinical manifestation, orbit magnetic resonance imaging (MRI), Somatostatin receptor scintigraphy (SRS) single-photon emission computed tomography (SPECT/CT). Results The main clinical manifestations were eyelids swelling and eyeballs protrusion in 25 cases with bilateral involvement happened in 23 cases, and 22 patients had 2 or more organs involved. Salivary gland (13 cases) and nose (19 cases) were most commonly involved organs besides eyes. Radiographic findings showed diffuse enlargement of lacrimal gland (22 cases), extraocular muscle hypertrophy (14 cases), infiltration of orbital soft tissue (10 cases). SRS indicated that the uptakes of tracer in orbits were high (UR=1.87±0.43) before treatment. After treatment by glucocorticoids and immunosuppressants, the uptakes in orbits reduced or disappear(UR=1.51±0.24). Conclusions Diffuse enlargement of bilateral lacrimal glands was the most common imaging finding of ocular lesions of IgG4-RD. Immune activity in orbits involvement can be objectively assessed with SRS, which is valuable for assessing clinical therapeutic effect.  (Ophthalmol CHN, 2015, 24: 309-312)