IgG4-related Lung and Pleural Disease: A Clinicopathologic Study of 21 Cases
IgG4-related Lung and Pleural Disease: A Clinicopathologic Study of 21 Cases
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DOI：
10.1097/pas.0b013e3181bd535b
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发表时间：
2009-12-01
影响因子：
5.6
通讯作者：
Nakanuma, Yasuni
中科院分区：
文献类型：
--
作者：
Zen, Yoh;Inoue, Dai;Nakanuma, Yasuni
免疫球蛋白G4（IgG 4）相关疾病可发生在呼吸系统。然而，其临床病理特征尚未得到很好的阐明。在这项研究中，我们研究了临床和病理特征，并随访数据，IgG 4相关的肺和胸膜病变。患者组包括17名男性和4名女性，平均年龄69岁（范围：42至76岁）。16例肺部病变和5例胸膜病变。组织学上，所有病变均表现为弥漫性淋巴浆细胞浸润。不规则纤维化和闭塞性血管改变在实性区域更常见。9例（43%）有嗜酸性粒细胞浸润，每个高倍视野超过5个细胞。免疫染色显示炎症区域有大量IgG 4阳性浆细胞。硬化性炎症分布于肺内结缔组织。肺部病变表现出各种形态学变化，根据炎症的优势领域。11例受试患者中有9例血清IgG 4浓度升高（平均6.9 g/L;范围0.3 - 18.0 g/L，正常范围< 1.35 g/L）。9例患者（43%）发现肺外和胸膜外IgG 4相关病变。并在随访期间同时或异步发展。所有接受类固醇治疗的患者均有反应，但3例患者仍存在超声放射学异常。有趣的是，在随访期间，发现1例患者在IgG 4相关肺部疾病背景下患有原发性腺癌。总之，IgG 4相关疾病的肺部和胸膜病变的种类比以前认为的要多。因此，了解这种疾病的形态多样性和临床病理特征是很重要的。
Immunoglobulin G4 (IgG4)-related disorders can occur in the respiratory system. However, the clinicopathologic characteristics have not been well clarified. In this study, we examined clinical and pathologic features of, and follow-up data on, IgG4-related lung and pleural lesions. The patients group consisted of 17 mates and 4 females with an average age of 69 years (range: 42 to 76). Pulmonary lesions in 16 patients and pleural lesions in 5 patients were examined. Histologically, all lesions showed diffuse lymphoplasmacytic infiltration. Irregular fibrosis and obliterative vascular changes were more common in solid areas. Nine cases (43%) had eosinophilic infiltration with more than 5 cells per high-power field. Immunostaining revealed numerous IgG4-positive plasma cells in inflamed areas. Sclerosing inflammation was distributed with intrapulmonary connective tissue. Pulmonary lesions showed a variety of morphologic changes according to the predominant area of inflammation. Serum IgG4 concentrations were elevated in 9 of 11 patients tested (average 6.9 g/L; range 0.3 to 18.0 g/L normal range < 1.35 g/L). Extra-pulmonary and extra-pleural IgG4-related lesions were identified in 9 patients (43%). and developed simultaneously or asynchronously during follow up. All patients treated with steroids responded, but sonic radiologic abnormalities remained in 3 patients. Interestingly, 1 patient was found to have a primary adenocarcinoma against a background of IgG4-related lung disease during follow up. In conclusion, IgG4-related diseases show a greater variety of pulmonary and Pleural lesions than Previously thought. It is important, therefore, to know the morphologic variety and clinicopathologic characteristics of this disorder.