Unusual Presentation of Pulmonary Alveolar Proteinosis with Multiple Pulmonary Nodules: A Case Report

肺泡蛋白沉積症以多發性肺結節為不尋常之臨床表現：罕見病例報告及文獻回顧

陳逸燕(I-Yen Chen) ； 巫政霖(Cheng-Lin Wu) ； 李政宏(Cheng-Hung Lee) ； 張漢煜(Han-Yu Chang)

《胸腔醫學》 31卷1期 (2016/02) Pp. 21-28

摘要

肺泡蛋白沉積症為肺泡累積脂蛋白物質導致罕見性肺疾病，臨床上表現相當多元，由輕微感冒症狀到呼吸衰竭皆有。典型影像學上表現為中央分散之肺泡型浸潤，而胸部電腦斷層攝影多呈現毛玻璃樣的病灶以及不規則石板拼鋪型態（crazy-paving pattern）。以多發性肺結節作為臨床表現之肺泡蛋白沉積症相當少見，文獻回顧大多為栗粒狀肺結節，且大多好發于幼兒。在此，我們報告一位肺泡蛋白沉積症以多發性肺結節為影像學表現之罕見病例。

關鍵字

肺泡蛋白沉積症 ； 肺結節

並列摘要

Pulmonary alveolar proteinosis (PAP) is a rare disorder in which lipoproteinaceous material accumulates within alveoli. It has a variable clinical presentation and course. The typical chest radiograph reveals bilateral central and symmetric lung opacities (“bat-wing” distribution). HRCT findings reveal ground glass opacities with thickened alveolar septa (crazy-paving appearance). Other radiographic patterns such as interstitial, mixed alveolar, and interstitial, nodular, asymmetrical, and focal patterns have also been described. Few cases have been reported as pulmonary nodules in the literature. PAP may appear as a well-circumscribed nodular area on CT and microscopic examination. Nodules reported in the literature were smaller, like in a miliary pattern. We report this unusual presentation of multiple pulmonary nodules (maximum 13 mm in diameter) caused by PAP in a patient, and review related articles. Although very rare, we should take PAP into consideration when treating patients with multiple lung nodules, especially immunocompromised patients.

並列關鍵字

pulmonary alveolar proteinosis ； pulmonary nodule