Clinicopathological features of IgG4-related disease complicated with orbital involvement
Clinicopathological features of IgG4-related disease complicated with orbital involvement
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DOI：
10.3109/14397595.2013.844307
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发表时间：
2014-05-01
影响因子：
2.2
通讯作者：
Sumida, Takayuki
中科院分区：
文献类型：
--
作者：
Hagiya, Chihiro;Tsuboi, Hiroto;Sumida, Takayuki
Objective. IgG 4相关疾病（IgG 4-RD）的特征是各种器官中IgG 4阳性浆细胞浸润和纤维化。IgG 4-RD累及的眼眶包括泪腺、眼外肌、三叉神经和眼眶的其他部分。免疫组织化学染色用于诊断眼眶炎症患者中的IgG 4-RD。本研究旨在探讨IgG 4-RD合并眼眶病变的临床病理特征。我们研究了2010年4月至2012年8月在筑波大学医院接受眼眶组织活检的9例IgG 4-RD患者的临床特征、病理学结果和治疗反应。在9名患者中，8名患有泪腺炎，1名患有眶下神经肿胀，另1名患有IgG 4相关的眼眶炎症。所有患者均发现其他器官受累，包括唾液腺、淋巴结、肺、肾和主动脉旁受累。在所有患者中，眼眶组织活检样本显示淋巴浆细胞浸润和纤维化，IgG 4阳性/IgG阳性浆细胞比率> 40%。所有患者均接受泼尼松龙（0.6 mg/kg/d）治疗，早期反应良好，但有2例患者在泼尼松龙减量后复发，表现为泪腺肿胀。IgG 4-RD合并眼眶受累者常合并其他器官受累。眼眶组织病理学检查结果符合IgG 4-RD的特征。皮质类固醇对IgG 4-RD的眼眶和全身受累有效。
Objective. IgG4-related disease (IgG4-RD) is characterized by IgG4-positive plasmacytic infiltration and fibrosis in various organs. Orbital involvement in IgG4-RD includes lacrimal glands, extra-ocular muscles, trigeminal nerve and other parts of the orbit. Immunohistochemical staining is used to diagnose IgG4-RD in patients with orbital inflammation. The purpose of this retrospective study was to clarify the clinicopathological features of IgG4-RD complicated with orbital involvement.Methods. We examined the clinical features, pathological findings and response to treatment in nine patients with IgG4-RD who underwent orbital tissue biopsy between April 2010 and August 2012 at the University of Tsukuba Hospital.Results. Among the nine patients, eight had dacryoadenitis, one had infraorbital nerve swelling, and another one had IgG4-related orbital inflammation. Involvement of other organs was identified in all patients, including involvement of the salivary glands, lymph nodes, lung, kidney and para-aorta. In all patients, biopsy samples from orbital tissues showed lymphoplasmacytic infiltration and fibrosis, and IgG4-positive/IgG-positive plasmacyte ratio of > 40%. All patients were treated with prednisolone (0.6 mg/kg/day) and responded well in early phase, although relapse was noted in two patients following tapering of prednisolone, evident by swelling of lacrimal glands.Conclusion. Patients with IgG4-RD complicated with orbital involvement often present with involvement of other organs. The histopathological findings of orbital tissue match the characteristic features of IgG4-RD. Corticosteroid is effective for orbital and systemic involvement in IgG4-RD.