Tumefactive IgG4-related Disease in Otolaryngology Field

IgG4相關性疾病合併病理tumefactive發炎變化在耳鼻喉科的臨床表現

孫萬烜(Wan-Hsuan Sun) ； 李育睿(Yu-Ruei Li) ； 羅承裕(Cheng-Yu Lo) ； 張燕良(Yen-Liang Chang)

《台灣耳鼻喉頭頸外科雜誌》 54卷2期 (2019/06) Pp. 57-67

https://doi.org/10.6286/jtohns.201906_54(2).057

摘要

BACKGROUND: Immunoglobulin G4-related disease involves chronic fibrotic inflammation accompanied by lymphocytes and immunoglobulin G4-positive plasma cell infiltration. Mikulicz's disease, Kuttner tumor, Riedel thyroiditis, and eosinophilic angiocentric fibrosis of the upper respiratory tract are all located on the spectrum of immunoglobulin G4-related disease. The purpose of this study is to discuss the clinical presentation and treatment of immunoglobulin G4-related disease within the field of otolaryngology. We will also discuss the relationship between tumefactive fibroinflammatory lesions and immunoglobulin G4-related disease. METHOD: In this study, we retrospectively reviewed 12 patients who were suspected of having or diagnosed to have immunoglobulin G4-related disease between October 2005 and October 2018 in Taipei Cathay General Hospital's otorhinolaryngology department. For each case, we analyzed clinical manifestations, laboratory tests, images, and pathological specimens. RESULT: The first case was initially diagnosed as chronic sinusitis, but was actually a tumefactive IgG4-related disease. After several surgeries, the patient's symptoms were improved. Nevertheless, earlier awareness of the IgG4-related disease would have been beneficial. The second case was suspected to be cervical lymphadenitis. After the symptoms progressed to an orbital pseudotumor and sinusitis, the treating physicians considered immunoglobulin G4-related sialadenitis and cervical lymphadenopathy. A better understanding of immunoglobulin G4-related diseases will allow this kind of patient to be diagnosed more accurately in the future. The third case suffered from enlarged masses in the bilateral upper neck, and an excisional biopsy showed immunoglobulin G4-positive plasma cell infiltration. As a result, an immunoglobulin G4-related disease was diagnosed. No symptoms remained after the patient underwent glucocorticoid therapy. The remaining nine cases with immunoglobulin G4-related sialadenitis were first diagnosed as a Kuttner tumor or sclerosing sialadenitis. CONCLUSION: Immunoglobulin G4-related disease generally involves the salivary glands. Cervical lymph nodes, the middle ear, the thyroid, sinuses, and the nasal cavity may also be affected. Tumefactive fibroinflammatory lesions should be considered a pathological presentation of immunoglobulin G4-related disease. Physicians should administer early glucocorticoid therapy if symptoms persist or progress.

關鍵字

IgG4-related disease ； tumefactive fibroinflammatory lesion

並列摘要

背景：IgG4相關性疾病是一種慢性纖維性發炎疾病伴隨淋巴及免疫球蛋白G4血漿細胞浸潤。Mikulicz's disease、Kuttner tumor、Riedel thyroidits以及eosinophilic angiocentric fibrosis近日都經研究討論後歸類在lgG4相關性疾病，本研究將整理IgG4相關性疾病在耳鼻喉科領域的臨床表現以及治療。另外tumefactve纖維發炎性病灶和IgG4相關性疾病的關聯性也會深入討論。方法：從2005年10月到2018年10月共13年間，在台北國泰醫院耳鼻喉科共搜集12名IgG4相關性疾病的病患。其臨床症狀；血液、影像、手術及病理發現；以及疾病治療等都會進一步討論。結果：第一名病患一開始以慢性鼻竇炎的症狀來求診。經過多次手術後症狀改善。由於近日的諸多研究對IgG4相間性疾病有更進一步了解，此病患仔細討論後認為是tumefactive IgG4相關性疾病。第二名病患一開始有兩側頸部淋巴及兩側下頷腺腫大等症狀。後期疾病進展到鼻竇以及眼眶都腫脹。經過近期許多研究更加了解lgG4相關性疾病。回顧其病史，IgG4相關性唾液腺炎及頸部淋巴炎為其正確診斷。第三名病患初期同樣呈現兩側頸部淋巴及兩側下頷腺腫大。經切除性切片病理檢查後確診為IgG4相關性唾液腺炎及頸部淋巴炎，經過類固醇治療後症狀大幅改善。其餘9名病患最初診斷為Kuttner tumor或是sclerosing sialadenitis，如今也皆被歸類在immunoglobulin G4-related sialadenitis。結論：IgG4相關性疾病最常侵犯唾液腺。此外，頸部淋巴腺、中耳、甲狀腺、鼻竇以及鼻腔等都是耳鼻喉科領域可能受侵犯的位置。經過相關研究整理及討論，我們認為Tumefactive fbroinflammatory lesions屬於IgG4相關性疾病的一種病理表現。類固醇治療在疾病初期能有良好的治療效果。

並列關鍵字

IgG4-related disease ； tumefactive fibroinflammatory lesion（IgG4相關性疾病 ； 腫瘤樣纖維發炎性病灶）