Department of Radiology, Massachusetts General Hospital, Boston, Massachusetts 02114, USA.
Ophthalmic Plast Reconstr Surg. 2013 Jul-Aug;29(4):261-6. doi: 10.1097/IOP.0b013e31829165ad.
Immunoglobin G4 (IgG4)-related disease is a systemic condition characterized by lymphoplasmacytic infiltrates that can involve the orbit. The purpose of this study was to identify the various patterns of orbital IgG4-related disease on imaging.
Retrospective review of radiologic examinations including CT, MRI, and positron emission tomography was performed in patients with proven cases of IgG4-related disease.
A total of 9 patients with orbital IgG4-related disease were identified, including 9 with CT, 4 with MRI, and 4 with 18-fluorodeoxyglucose positron emission tomography. Patterns of involvement included lacrimal gland enlargement, lacrimal sac involvement, extraocular muscle thickening, preseptal involvement, orbital fat involvement, and cranial nerve involvement, many of which occurred simultaneously. Associated demineralization of the orbital wall was evident on CT in 2 cases. On T2-weighted MRI, the lesions appeared as hypointense in 2 cases, heterogeneously hypointense to isointense in 1 case, and hyperintense in 1 case. Diffuse enhancement was present in all 3 cases in which postcontrast T1-weighted sequences were available. The lesions were hypermetabolic on positron emission tomography in 3 of 4 cases. There was definite extraorbital involvement by IgG4-related disease in 3 of the 9 patients and suspected involvement in another 3 of the 9 patients.
IgG4-related disease displays a wide variety of imaging manifestations in the orbit. Extraorbital disease is often present and can help suggest the diagnosis.
免疫球蛋白 G4（IgG4）相关疾病是一种以淋巴浆细胞浸润为特征的系统性疾病，可累及眼眶。本研究的目的是确定眼眶 IgG4 相关疾病的各种影像学表现模式。
对经证实的 IgG4 相关疾病患者的影像学检查（包括 CT、MRI 和正电子发射断层扫描）进行回顾性分析。
共发现 9 例眼眶 IgG4 相关疾病患者，其中 9 例有 CT 检查，4 例有 MRI 检查，4 例有 18-氟脱氧葡萄糖正电子发射断层扫描。受累模式包括泪腺增大、泪囊受累、眼外肌增厚、眶隔前间隙受累、眶内脂肪受累和颅神经受累，许多患者同时存在多种受累模式。2 例 CT 显示眶壁有明确的脱钙。在 T2 加权 MRI 上，2 例病变呈低信号，1 例呈不均匀低信号至等信号，1 例呈高信号。3 例有增强后 T1 加权序列的患者均有弥漫性强化。4 例中有 3 例在正电子发射断层扫描上呈高代谢。9 例患者中有 3 例存在明确的眼眶外 IgG4 相关疾病受累，另有 3 例疑似受累。
IgG4 相关疾病在眼眶中表现出广泛的影像学表现。眼眶外疾病常存在，并有助于提示诊断。
