Pulmonary Granulomatous Inflammation and Unexplained Repeated Infections-A Case of Chronic Granulomatous Disease

肺部肉芽性發炎及不明原因反覆感染－慢性肉芽腫病個案報告

陳泓丞(Hung-Cheng Chen) ； 楊崑德(Kuender D. Yang) ； 林安伸(An-Shen Lin) ； 王金洲(Chin-Chou Wang) ； 黃琬婷(Wan-Ting Huang) ； 賴建豪(Chien-Hao Lie)

《胸腔醫學》 26卷3期 (2011/06) Pp. 133-139

https://doi.org/10.29806/TM.201106.0003

摘要

慢性肉芽腫病(CGD)是一種罕見因細胞內superoxide製造功能衰竭所造成的遺傳性及先天免疫功能不全症候群疾病。它通常於出生後第一年即有嚴重反覆性細菌和黴菌感染。我們提出一個案為一年輕男性患有慢性肉芽腫病於初就診的胸部X-ray表現為左下肺實質化與空洞化。患者曾有反覆不明原因感染病史（包括肝膿瘍，蜂窩組織炎分別在患者17歲和19歲）。此患者切割活檢標本顯示為肺肉芽腫發炎。在台灣肺肉芽腫發炎常於肺結核病患者出現，此患者的痰標本及切割活檢檢體中抗酸桿菌鑑定皆呈陰性表現。相關自體免疫疾病檢測亦呈現陰性。在刺激後的多發性多形性白血球功能測試及chemiluminance測試顯示為低反應，加上趨化檢測為陰性結果，故診斷為慢性肉芽腫病。慢性肉芽腫病罕見在成年才開始表現，推測延遲診斷原因是目前強效抗生素藥物治療及在成年胸腔科相關知識的缺乏。因此，任何青少年或成人不明原因的肺部反覆感染，並伴隨著肉芽腫的形成，應考慮顆粒球白血細胞缺陷所導致的免疫不全症候群及慢性肉芽腫病。對於慢性肉芽腫病早期診斷給予及時治療和預防感染是相當重要的。

關鍵字

慢性肉芽腫病 ； 慢性肉芽腫性發炎

並列摘要

Chronic granulomatous disease (CGD) is a rare inherited disorder caused by a failure of intracellular superoxide production by phagocytes. It is usually identified in early childhood with severe recurrent bacterial and fungal infections. We present a case of CGD in a young male adult in whom the disease initially presented with left middle lung consolidation with cavitation. The patient had a history of unexplained repeated infection (including liver abscess and submandibular cellulitis at the age of 17 and 19 years). A specimen of cutting biopsy of the lung showed granulomatous inflammation. Pulmonary granuloma is a common manifestation of tuberculosis in Taiwan, but no acid-fast bacilli were identified by Ziehl-Neelsen staining in a specimen of sputum and cutting biopsy. In addition, no autoimmune disease was detected. The patient had negative respiratory burst activity in the polymorphonuclear leukocyte function test, a low response in the chemiluminescence test, and a normal finding in the chemotaxis assay, so the diagnosis of CGD was finally established. CGD rarely starts presenting in adulthood, either because it is not well-recognized in non-pediatric chest wards or because of the administration of potent antimicrobials that unintentionally treat many CGD-associated infections, postponing the diagnosis until more severe infections occur. Therefore, any adolescent or adult with unexplained and repeated infections that are accompanied by granuloma formation should be checked for phagocyte function defects. Early diagnosis of CGD is important because of the benefits of timely treatment and infection prophylaxis.

並列關鍵字

chronic granulomatous disease ； granulomatous inflammation