Mixed Connective Tissue Disease Presenting with Chylothorax-A Case Report and Literature Review

混合性結締組織疾病合併乳糜胸－病例報告及文獻回顧

陳相如(Shung-Ru Chen) ； 洪敏德(Min-De Hung) ； 沈光漢(Gwan-Han Shen) ； 許正園(Jeng-Yuan Hsu)

《胸腔醫學》 22卷3期 (2007/06) Pp. 168-173

https://doi.org/10.29806/TM.200706.0003

摘要

混合性結締組織疾病是一種結締組織異常的疾病，合併有高價且明確的自體抗體，臨床上常有類似全身性紅斑性狼瘡，硬皮症及多發性肌炎的表現。
混合性結締組織疾病早期的表現並不具特異性，常只是全身倦怠，關節肌肉酸痛及低熱，較特異的發現是抗核抗體的產生。肺部的浸潤相當常見(85%)，但少見助膜侵犯。而以乳糜胸來表現的，在目前文獻中仍未被報導。
乳糜胸的病因如下：腫瘤（54%，其中淋巴腫瘤占四分之三），創傷（25%，手術占大部分），原因不明(15%)，其它病因(6%)。本篇報告一個呈現乳糜胸的混合性結締組織疾病病案，在以前的文獻中，鮮見類似個案的報導。乳糜胸的鑑別診斷中必須將混合性結締組織疾病納入考慮。

關鍵字

乳糜胸 ； 混合性結締組織疾病 ； 全身性紅斑性狼瘡 ； 自體免疫疾病

並列摘要

Mixed connective tissue disease (MCTD) was defined as a connective tissue disorder characterized by the presence of high titers of a distinct autoantibody in combination with clinical features commonly seen in systemic lupus erythematosus (SLE), scleroderma, and polymyositis (referred to as overlap syndrome).
The early clinical features of MCTD are nonspecific, and may consist of general malaise, arthralgias, myalgias, and low-grade fever. A specific clue that these symptoms are caused by a connective tissue disease is the discovery of a positive antinuclear antibody (ANA) and high RNP (ribonucleoprotein). The lung is usually involved, but pleural involvement is rare, and chylothorax has not been reported before.
The etiologies of chylothorax are: tumor (54%, with lymphoma responsible for three-quarters), trauma (25%, with surgical trauma responsible for most), idiopathic (15%), and miscellaneous (6%). Rheumatoid arthritis and systemic lupus erythematosus have been reported to have chylothorax, but in only a limited number of cases. Herein, we report a case of MCTD presenting with chylothorax, which should be considered in the differential diagnosis of chylothorax.

並列關鍵字

chylothorax ； MCTD mixed connective tissue disease ； SLE systemic lupus erythematosus ； autoimmune disease