肺泡蛋白沉积症继发感染九例临床分析

A clinical analysis of 9 cases of pulmonary alveolar proteinosis with secondary infection

目的 通过分析9例特发性肺泡蛋白沉积症（PAP）继发感染患者的临床资料，以提高陔病的诊治水平。方法 同顾性分析北京协和医院1990年1月至2010年1月因继发感染需要住院诊疗的特发性PAP患者的临床资料。结果 1990年1月1日至2010年1月1日北京协和医院共收治特发性PAP患者97例，男69例，女28例，其中9例㈨继发感染需要住院诊疗，男5例，女4例，年龄22～7l（46．4±14．6）岁。6例曾误诊为间质性肺炎，均用过糖皮质激素治疗（3例仍在使用糖皮质激素，另3例已停用糖皮质激素3～15．5个月）。5例曾接受单侧和（或）双侧肺灌洗，与此次感染相距1个月、2个月、9个月、14个月、24个月不等。发热8例，咳嗽9例，咳痰8例，咯血2例，胸痛1例，肺部听诊有湿哕音1例。影像学以弥漫性磨玻璃影（9例）、空洞影（4例）为主要表现，合并胸腔积液少见（1例）。感染灶均局限在胸腔内：9例均有肺部感染，1例合并胸膜受累。病原学：结核分枝杆菌感染4例，真菌感染3例（白念珠菌、青霉菌及烟曲霉各1例），诺卡菌感染2例（其中1例合并巨细胞病毒感染）。9例患者6例治愈，1例好转，2例死亡。结论 对特发性PAP患者，尤其是州糖皮质激素者，fl；现发热、近期加重的呼吸困难，胸部影像学有空洞、结节影等表现时，需警惕PAP继发感染的可能，尽早获取病原学资料，早期、足疗程治疗可以改善预后。

Objective To describe the clinical characteristics of 9 cases of idiopathic pulmonary alveolar pmteinosis （iPAP） with secondary infections. Method The clinical and radiological data of 9 patients with iPAP and secondary infections admitted into Peking Union Medical College Hospital from 1 st January 1990 to 1st January 2010 were retrospectively analyzed. Results In that period, them were 97 patients of iPAP were admitted in our hospital. There were 9 patients of iPAP with secondary infections ,aged （46.4±14.6）y. There were 5 males and 4 females. Among them, 6 patients were misdiagnosed as interstitial pneumonia and cortieosteroids were given to them. When the infection appeared, cortieosteroids were still given to 3 patients, and the other 3 patients had stopped corticosteroids for 3 to 15 and a half months. Five patients had accepted mono-lung or whole lung lavage before 1,2, 9, 14,24 months. The clinical manifestations were fever （ 8 cases）, cough （ 9 cases） , expectoration （ 8 cases）, hemoptysis （ 2 cases） , chest pain（ l case） and moist rales （ 1 ease）. Glass-ground opacities （9 cases） and cavitations （4 case） were the main manifestations of chest radiology. Pleural effusions（ 1 case） was not common. The locations of infection was limited in chest：9 cases had puhnonary infection and one case was associated with pleurisy. The infectious pathogens were the acid-fast tubercle bacillus（4 cases）, fungus （3 eases, candida albicans, penicillium and aspergillus fumigatus for each one） and norcardia（2 eases, one ease was associated with cytomegalovirus infection）. Follow-up： 6 patients were cured, 1 patient was improved and 2 patients were died. Conclusions For patients with iPAP, especially when they had been receiving cortieosteroids, if they had fever and/or recently exaggerated dyspnea, especially whose chest radiology showed nodules and cavitations, the clinicians should be aware of infections diseases for them. Further specific microbiological studies and sufficient therapy should be obtained as quickly as possible.