Immunoglobulin G4-related lung disease: Clinicoradiological and pathological features
Immunoglobulin G4-related lung disease: Clinicoradiological and pathological features
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DOI：
10.1111/resp.12016
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发表时间：
2013-04-01
期刊：
影响因子：
6.9
通讯作者：
Ogura, Takashi
中科院分区：
文献类型：
--
作者：
Matsui, Shoko;Hebisawa, Akira;Ogura, Takashi
背景和目的：免疫球蛋白G4（IgG 4）相关疾病是一种多器官疾病，可包括肺。IgG 4相关的肺部疾病可以呈现在各种形式，与这种疾病的患者的临床，放射学和病理特征进行了assessed.Methods：48例疑似IgG 4相关的肺部疾病，与高浓度的血清IgG 4和丰富的IgG 4阳性浆细胞浸润到胸腔内器官，进行了回顾性评价。结果：48例患者中，有18例有胸外表现，诊断为IgG 4相关性肺部疾病。这些患者大多数是中年至老年男性。IgG 4相关性肺病的特征是血清IgG和IgG 4浓度高，白色血细胞计数和血清C反应蛋白浓度正常，对皮质类固醇反应良好。常见的影像学表现包括纵隔淋巴结病和淋巴管周围增厚，伴或不伴胸膜下和/或支气管血管周围实变。病理检查显示大量的淋巴浆细胞浸润与纤维化的淋巴管周围的路线，与分布以及相关的放射学表现。结论：研究结果表明，IgG 4相关的肺部疾病的胸腔内表现通过淋巴管途径的肺，并显示出各种临床特征。由于一些淋巴组织增生性疾病表现出类似的结果，临床放射学和病理学特征的相关性是至关重要的IgG 4相关性肺部疾病的诊断。
Background and objective: Immunoglobulin G4 (IgG4)-related disease is a multi-organ disorder that can include the lungs. IgG4-related lung disease can present in various forms; the clinical, radiological and pathological features of patients with this disease have been assessed.Methods: Forty-eight patients suspected of having IgG4-related lung disease, with a high serum concentration of IgG4 and abundant IgG4-positive plasma cell infiltration into the intrathoracic organs, were retrospectively evaluated. Their clinical features, chest imaging findings and pathological findings were examined, with final diagnoses made by an open panel conference.Results: Of the 48 patients, 18 with extrathoracic manifestations were diagnosed as having IgG4-related lung disease. Most of these patients were middle-aged to elderly men. IgG4-related lung disease was characterized by high serum concentrations of IgG and IgG4, normal white blood cell count and serum C-reactive protein concentration and a good response to corticosteroids. Common radiological findings included mediastinal lymphadenopathy and thickening of the perilymphatic interstitium, with or without subpleural and/or peribronchovascular consolidation. Pathological examination showed massive lymphoplasmacytic infiltration with fibrosis in and around the lymphatic routes, with distribution well correlated with radiological manifestations.Conclusions: The findings suggest that the intrathoracic manifestations of IgG4-related lung disease develop through lymphatic routes of the lungs and show various clinical characteristics. Because some lymphoproliferative disorders show similar findings, the correlation of clinicoradiological and pathological characteristics is crucial for the diagnosis of IgG4-related lung disease.