Ocular adnexal IgG4-related disease has uniform clinicopathology
Ocular adnexal IgG4-related disease has uniform clinicopathology
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DOI：
10.1111/j.1440-1827.2008.02257.x
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发表时间：
2008-08-01
影响因子：
2.2
通讯作者：
Yoshino, Tadashi
中科院分区：
文献类型：
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作者：
Sato, Yasuharu;Ohshima, Koh-ichi;Yoshino, Tadashi
IgG 4相关疾病是最近提出的具有几个独特的临床病理特征的临床实体。然而，眼附属器IgG 4相关疾病尚未得到很好的阐明。本研究的目的是检查21例眼附属器IgG 4相关疾病患者（10例男性，11例女性;年龄范围，39-86岁）。21例患者中有17例（81%）累及泪腺，经常观察到双侧泪腺肿胀（n = 12; 70.6%）。与此相反，任何患者的结膜均未受累。组织学是均匀的，有明显的淋巴浆细胞浸润混合致密的纤维化，类似于以前的报告IgG 4相关的疾病。免疫染色检测到大量IgG 4阳性浆细胞聚集。在接受检测的13名患者中，有10名患者的血清IgG 4高于正常水平，尽管几乎所有病例都是在治疗后测量的。有趣的是，免疫球蛋白重链基因重排检测在两个17例（12%）检查。目前的研究结果表明，眼附属器IgG 4相关疾病具有统一的临床病理学：即，疾病涉及双侧泪腺淋巴样增生和纤维化，而不是结膜。免疫球蛋白重链基因重排的存在提示B细胞淋巴瘤可能是在IgG 4相关慢性炎症背景下发生的。
IgG4-related disease is a recently proposed clinical entity with several unique clinicopathological features. Ocular adnexal IgG4-related disease, however, has not well been clarified. The purpose of the present study was to examine 21 patients (10 men, 11 women; age range, 39-86 years) with ocular adnexal IgG4-related disease. In 17 out of 21 patients (81%), the lacrimal glands were involved and bilateral lacrimal gland swelling was frequently observed (n = 12; 70.6%). In contrast, the conjunctiva was not involved in any of the patient. Histology was uniform with marked lymphoplasmacytic infiltration admixed with dense fibrosis, similar to previous reports of IgG4-related disease. Immunostaining detected numerous aggregates of IgG4-positive plasma cells. Serum IgG4 was higher than normal in 10 of the 13 patients tested, although it was measured after treatment in almost all cases. Interestingly, immunoglobulin heavy chain gene rearrangement was detected in two of 17 patients (12%) examined. The present results show that ocular adnexal IgG4-related disease has uniform clinicopathology: that is, disease involving the bilateral lacrimal glands with lymphoid hyperplasia and fibrosis, but not the conjunctiva. And presence of immunoglobulin heavy chain gene rearrangement suggests the possibility of B-cell lymphoma arising in a background of IgG4-related chronic inflammation.