Hagiya Chihiro, Tsuboi Hiroto, Yokosawa Masahiro, Hagiwara Shinya, Hirota Tomoya, Takai Chinatsu, Asashima Hiromitsu, Miki Haruka, Umeda Naoto, Horikoshi Masanobu, Kondo Yuya, Sugihara Makoto, Ogishima Hiroshi, Suzuki Takeshi, Hiraoka Takahiro, Kaji Yuichi, Matsumoto Isao, Oshika Tetsuro, Sumida Takayuki
Departments of Internal Medicine, Faculty of Medicine, University of Tsukuba , Tsukuba , Japan.
Mod Rheumatol. 2014 May;24(3):471-6. doi: 10.3109/14397595.2013.844307. Epub 2013 Oct 21.
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.
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.
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.
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.
IgG4相关性疾病（IgG4-RD）的特征是各器官中存在IgG4阳性浆细胞浸润和纤维化。IgG4-RD累及眼眶包括泪腺、眼外肌、三叉神经和眼眶其他部位。免疫组织化学染色用于诊断眼眶炎症患者的IgG4-RD。本回顾性研究的目的是阐明IgG4-RD合并眼眶受累的临床病理特征。
我们检查了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IgG4-RD患者的临床特征、病理结果及治疗反应。
9例患者中，8例有泪腺炎，1例有眶下神经肿胀，另1例有IgG4相关性眼眶炎症。所有患者均发现有其他器官受累，包括唾液腺、淋巴结、肺、肾和腹主动脉旁。所有患者眼眶组织活检样本均显示淋巴细胞浆细胞浸润和纤维化，且IgG4阳性/IgG阳性浆细胞比例>40%。所有患者均接受泼尼松龙治疗（0.6mg/kg/天），早期反应良好，尽管2例患者在泼尼松龙减量后复发，表现为泪腺肿胀。
IgG4-RD合并眼眶受累的患者常伴有其他器官受累。眼眶组织的组织病理学表现符合IgG4-RD的特征。皮质类固醇对IgG4-RD的眼眶及全身受累有效。
