Immunoglobulin G4-Related Disease with Isolated Lung Involvement: A Case Report and Literature Review

IgG4相關性疾病僅侵犯肺部之病例報導與文獻回顧

陳彥霖(Yen-Lin Chen) ； 黃俊凱(Chun-Kai Huang) ； 鄭媚方(Mei-Fang Cheng) ； 王振源(Jann-Yuan Wang)

《胸腔醫學》 32卷2期 (2017/04) Pp. 105-114

摘要

Immunoglobulin G4-related disease (IgG4-RD) is a new concept in systemic disease. Isolated lung involvement is, however, rarely reported. In this report, we describe a 64-yearold man with chronic cough and worsened dyspnea. Chest computed tomography (CT) revealed multiple areas of consolidation in bilateral lung fields. The pathological report on the tissue biopsy from the left lower lung indicated a dense lymphoplasmacytic infiltrate rich in IgG4-positive plasma cells and obliterative phlebitis, which met the diagnostic criteria of IgG4-RD. Positron emission tomography (PET) and abdominal magnetic resonance imaging excluded extra-thoracic organ involvement. Through our report of this case and the literature review, we emphasize the concept of IgG4-RD having a broad spectrum of manifestation, including targeting of the lung as the primary organ. A thorough investigation via high-yield tools, such as PET, can assist in evaluating disease extent, and in clinical decision-making for treatment and follow-up.

關鍵字

IgG4 ； lung involvement ； positron emission tomography

並列摘要

免疫球蛋白G4相關性疾病（IgG4-related disease）是近年發現的一種系統性疾病。其中僅以肺部作為單一侵犯器官之臨床案例極少。此報導病例為一位六十四歲男性，主訴慢性咳嗽與日漸嚴重之呼吸困難，經胸部電腦斷層檢查發現雙側肺葉多處實質化病灶，經左下肺病灶切片檢查後，病理報告顯示為緻密的淋巴漿細胞浸潤（dense lymphoplasmacytic infiltrate），其中以富含IgG4陽性的漿細胞為主，同時亦發現阻 塞性靜脈炎（obliterative phlebitis）。上述發現均符合IgG4相關性疾病之診斷，病人並接受正子攝影與腹部核磁共振檢查，確定無胸腔外器官受到侵犯。藉由此病例報導與文獻回顧，我們確認IgG4相關性疾病的不同病程中，可以僅以肺部為唯一侵犯器官。透過如正子攝影等有效的全身評估工具，臨床醫師能更加瞭解病人疾病侵犯範圍，以協助擬定臨床治療與追蹤方針。

並列關鍵字

免疫球蛋白G4 ； 肺侵犯 ； 正子攝影