肺泡蛋白沉着症肺功能检查特点:附27例分析

目的 肺泡蛋白沉着症(PAP)在临床上极为罕见,其肺功能检查特点的报道也极少,本研究目的在于探讨肺功能检查在PAP诊断和治疗中的价值.方法 回顾性分析本院呼吸疾病研究所经肺组织病理(过碘酸雪夫反应阳性)确诊的27例PAP患者的临床资料.全部病例均给予肺通气和弥散功能测试,其中12例进行了肺容积测试.比较了10例行支气管肺泡灌洗(BAL)治疗的患者在灌洗前、后肺功能的改变情况.结果 27例患者入院后的基础通气肺功能情况:用力肺活量(FVC):(79.67±16.21)%;第1秒用力呼气容积(FEV1):(83.94±16.07)%,一秒率(FEV1/FVC):(89.20±5.50)%;最大呼气流量(PEF):(107.64±17.73)%;肺一氧化碳弥散量(DLCO):(49.27±21.83)%;DLCO与肺泡通气量比值(DLCO/VA):(69.92±20.11)%.肺总量(TLC):(80.60±19.56)%;残气容积(RV):(86.03±38.10)%;残总比(RV/TLC):(32.73±9.48)%;功能残气量(FRC):(84.91±28.08)%.27例患者基础肺功能下降的异常率:FVC:55.6%(15例);FEV1:44.4%(12例);FEV1/FVC:0%(0例);PEF:3.7 %(1例);DLCO:88.9%(24例);DLCO/VA:70.4%(19例).12例患者肺容积的异常率:TLC下降者占50%(6例);RV下降者占41.7%(5例),升高者占16.7%(2例);RV/TLC升高者占50%(6例);FRC下降者占33.3%(4例).10例进行BAL治疗的患者术后肺功能改善率:FVC:5.47%;FEV1:5.50%;DLCO:31.07%;DLCO/VA:20.35%.灌洗前后DLCO及DLCO/VA差异有统计学意义(t=-3.551,-3.159;P=0.006,0.012).结论 PAP的肺功能检查以肺限制性通气功能障碍及肺弥散功能障碍为常见,尤其为弥散功能障碍.PAP经BAL治疗后肺弥散功能有显著性改善.

Characters of pulmonary function testing in pulmonary alveolar proteinosis: analysis of 27 patients

Objective Pulmonary alveolar proteinoais (PAP) is a rare respiratory disorder featured with lipoproteinaceous material accumulating within alveoli. Few studies on the characters of pulmonary function testing in PAP have been addressed. The purpose of this study was to investigate the clinical features of lung function testing in the diagnosis and therapy of PAP. Methods Twenty-seven patients with PAP (15 to 54 years) were enrolled in the institute and histopathologically diagnosis was proven by antiperiodic acid Schiff positive reaction. Spirometry and diffusion capacity were measured in all patients, among them 12 were tested with total lung volume. Ten patients with PAP were treated with bronchoalveolar lavage (BAL). Results of pulmonary function tests were compared before and after the treatment. Results Baseline percentage of prediction in FVC, FEV1, FEV1/FVC, PEF, DLCO and DLCO/VA were (79.67±16.21 ) %, (83.94±16.07)%,(89.20±5.50)%, (107. 64±17.73)%, (49.27±21.83)%, (69.92±20.11)%, respectively. Baseline percentage of prediction in TLC, RV, RV/TLC and FRC were (80. 60±19.56) %,(86.03±38. 10)%, (32. 73±9.48)% and (84.91±28.08 ) %, respectively. The incidence of impaired pulmonary function in 27 patients was 55.6% in FVC and 44.4% in FEV1 and 0% in FEV1/FVC and 3. 7% in PEF and 88. 9% in DLCO and 70.4% in DLCO/VA. For the abnormal lung volumes in 12 patients,the decreased TLC was found in 6 patients, decreased RV in 5 patients,increased RV in 2 patients and increased RV/TLC in 6 patients,decreased FRC in 4 patients. FVC, FEV,1 ,DLCO and DLCO/VA were improved after whole lung lavage by 5.47%, 5.50%, 31.07% and 20. 35%, respectively. Significant improvements were found in DLCO and DLCO/VA after treatment (t=-3.551,-3. 159, P=0.006 and 0. 012). Conclusions The impairment of pulmonary function in PAP is featured by restrictive ventilation disorder and reduction of diffusion capacity, with more severe of the latter. After being treated with BAL, the pulmonary function of PAP could be markedly improved.