肺泡蛋白沉积症11例临床分析

Eleven cases analysis of pulmonary alveolar proteinosis

目的探讨肺泡蛋白沉积症（PAP）的临床特点及治疗方法。方法回顾性分析11例确诊的PAP患者。比较支气管肺泡灌洗（BAL）及全肺灌洗前后血气分析、肺功能及影像学变化。结果11例患者均为隐匿起病，主要症状和体征：咳嗽（11例），咳痰（11例），进行性呼吸困难（9例）；8例测定血清乳酸脱氢酶（LDH）的患者中，有4例血清LDH增高；6例患者经肺功能测定，5例表现为限制性通气功能障碍及弥散功能下降，部分患者表现为呼吸衰竭；9例患者的CT表现：7例为双肺弥漫性分布片状磨玻璃样影，1例主要表现为双肺网状纤维条索影，仅有少量片状磨玻璃样，另有1例表现为两肺弥漫分布的斑点状影伴少量网状纤维条索影；肺灌洗后大部分患者临床症状和影像表现有所改善。用力肺活量（FVC）由（67．75±18．21）％提高到（77．06±19．37）％，P〉0．05；DLCO由（53．95±22．11）％提高到（59．98±11．76）％，P〉0．05。结论CT扫描有助于早期诊断PAP，肺灌洗可以改善患者的症状影像表现。

Objective To Investigate clinical features and therapy methods of a series of 11 patients with PAP. Methods We conducted a retrospective analysis of 11 confirmed cases, comparing the change of pulmonary function, arterial blood gas measurement, CT scan before and after Bronchoalveolar Lavage（BAL） or whole lung lavage. Results All the eleven patients belonged to hiding sickness and the most common symptoms were cough and sputum（ 11 patients） ,dyspnea（9 patients）. Eight patients underwent the serum lactate dehydrogenase （LDH）test, elevated LDH in four patients. Six patients underwent the pulmonary function test,five of them present restrictive ventilatory disorder and declining in the diffusing capacity of lung,part of them had respiratory failure. The CT image of nine patients showed：seven of them had ground glass infiltration in both lungs, one patient mainly presented reticular interstitial shadows with few ground glass infiltration and another patient presented widespread mottling shadows with few interstitial reticular shadows. In this group,eleven patients were confirmed by pathological results. After whole lung lavage or bron- choalveolar lavage,most patients＇ symptom and chest imageologic presentation were improved：FVC% was increased from （67.75 ±18.21）% to （77.06 ± 19.37）%,P 〉0.05 and DLCO from （53.95 ±22.11）% to （59.98 ± 11.76） ,P 〉 0.05. Conclusion CT scan was important in the diagnosis of PAP in early stage. BAL and whole lung lavage could improve the clinical symptom and chest imageologic presentation.