IgG4-Related Disease with Pleural Involvement Presenting as Progressive Dyspnea

IgG4相關疾病以肋膜侵犯和呼吸喘表現

林珮瑜(Pei-Yu Lin) ； 古世基(Shih-Chi Ku)

《胸腔醫學》 32卷6期 (2017/12) Pp. 272-278

摘要

Immunoglobulin G4 (IgG4)-related disease is a recognized fibroinflammatory condition. In its pathology, we can see lymphoplasmacytic infiltration, storiform fibrosis and IgG4- positive plasma cells. The disease can involve multiple organ systems, including the lung. In this article, we reported the case of a patient with an initial presentation of hydronephrosis and retroperitoneal mass. The computed tomography-guided biopsy revealed IgG4-related disease. The patient then began to receive weekly dexamethasone treatment (4 mg/week). The mass size decreased gradually, with the disease stabilizing during follow-up. However, a new onset of left pleural effusion developed after 7 years of a stable condition. The pleural fluid analysis revealed lymphocyte-predominant exudate, and the pleural biopsy showed clustered plasma cells and lymphocytes. These plasma cells were mostly IgG4-positive (more than 50 per high-power field). The serum IgG4 level also elevated to 2,490 mg/dL. IgG4- related disease with pleural involvement was diagnosed. The pleural effusion almost totally subsided 1 month after increasing the dose of Predonine to 15 mg/day.

關鍵字

IgG4-related disease ； retroperitoneal mass ； pleural effusion

並列摘要

IgG4相關疾病的致病機轉雖未完全確定，但目前一致認為它和自體免疫相關，全身的器官都有可能被侵犯，IgG4相關疾病的肺部病例報告相對較少，並不像其對於胰臟或者唾液腺的案例多。本文我們報告一位八十六歲的男性，一開始以腎水腫、後腹腔腫塊來表現，病人經電腦斷層導引切片證實為IgG4相關疾病，血液中IgG4數值也上升，腫塊在類固醇治療後逐漸變小，然而卻在治療穩定時，病人出現新的肋膜積液，肋膜積液分析顯示為淋巴球為主的滲出液，其餘細胞學檢查和培養皆為陰性，病人最後接受肋膜切片才發現一樣是IgG4相關疾病導致。

並列關鍵字

IgG4相關肺部疾病 ； 肋膜積液 ； 肋膜切片