Diagnosis and Treatment of Pulmonary Alveolar Proteinosis:Analysis of 34 Cases_Chinese Journal of Respiratory and Critical Care Medicine

Authors：

 LiYing , YanWei , ZhangXiaohua , LiuLang

Hunan Prevention and Treatment Center for Occupational Diseases, Changsha, Hunan, 410007, China;

Corresponding?author：

LiYing, Email: liying9858@163.com

Keywords：

Pulmonary alveolar proteinosis; Inhalation of dust; Whole lung lavage; Recurrence

DOI：

10.7507/1671-6205.2016093

Video：

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Objective To improve the diagnosis and treatment of pulmonary alveolar proteinosis (PAP). Methods The clinical data of 34 PAP cases diagnosed between May 2008 and June 2015 in Hunan Prevention and Treatment Center for Occupational Diseases were retrospectively analyzed. Results There were 34(52.9%)patients with a history of dust exposure. Cough and progressive dyspnea were common clinical symptoms. The incidence rate of hypoxemia and lung function damage was 87.9% and 90.9%,respectively. High resolution CT clearly demonstrated the characteristic map-like changing and paving-stone sign. Arterial blood oxygen partial pressure increased from (62.5±12.9)mm Hg to (73.0±12.2)mm Hg,and D_LCO%pred increased from (50.1±14.9)% to (64.6±14.4)% after large-volume whole lung lavage. The recurrence rate was 38.2%. Conclusion The etiology of PAP is unknown,dust exposure may be associated with secondary PAP. The effect of whole lung lavage is remarkable but the recurrence rate is high. It is needed to study further on etiological treatment of PAP.

Citation： LiYing, YanWei, ZhangXiaohua, LiuLang. Diagnosis and Treatment of Pulmonary Alveolar Proteinosis:Analysis of 34 Cases. Chinese Journal of Respiratory and Critical Care Medicine, 2016, 15(4): 398-400. doi: 10.7507/1671-6205.2016093 Copy

1.	余巍巍, 曾錦榮,王昌明,等.國內20年肺泡蛋白沉積癥誤診原因匯總分析.中國綜合臨床,2009,25:1276-1278.
2.	Rosen SH,Castleman B,Liebow AA.Pulmonary alveolar proteinosis. N Engl J Med,1958,258:1123-1142.
3.	張弘, 邵池,彭敏,等.肺泡蛋白沉積癥84例誤診誤治分析.中國實用內科雜志,2009,29:426-428.
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7.	Arai T,Inoue Y,Sugimoto C,et al.CYFRA 21-1 as a disease severity marker for autoimmune pulmonary alveolar proteinosis. Respirology,2014,19:246-252.
8.	劉娟, 向萍.可疑慢性粉塵接觸致肺泡蛋白沉積癥1例報道.重慶醫學,2014,43:4415-4416.
9.	葉紹色. 接塵工人肺泡蛋白沉積癥10例臨床分析.應用預防醫學,2010,16:300-301.
10.	Bonella F,Bauer PC,Griese M,et al.Pulmonary alveolar proteinosis:New insigh from a single-center cohort of 70 patients.Respir Med,2011,105:1908-1916.
11.	Cummings KJ,Nakano M,Omae K. Indium lung disease.Chest,2012,141:1512-1521.
12.	王志兵, 張向左.肺泡蛋白沉積癥的影像學特點分析.華北煤炭醫學院學報,2010,12:352.
13.	Sihoe AD,Ng VM,Liu RW,et al. Pulmonary alveolar proteinosis in extremis:the case for aggressive whole lung lawage with extracorporeal membrane oxygenation suppo.Heart Lung Circ,2008,17:69-72.
14.	徐凱峰, 陳燕,郭子健,等.肺泡蛋白沉積癥患者血清中抗粒-巨噬細胞集落刺激因子抗體等血清學指標的臨床意義.中華結核和呼吸雜志,2004,27:824-828.
15.	聞勝蘭, 吳曉虹,應可凈,等.大容量全肺灌洗治療重度肺泡蛋白沉積癥五例分析.中華結核和呼吸雜志,2004,27:598-600.
16.	楊俊, 江丹,盧進昌,等.大容量全肺灌洗治療肺泡蛋白沉積癥10例療效分析.浙江臨床醫學,2014,16:397-398.
17.	Ioachimescu OC,Karuru MS.Pulmonary alveolar proteinosis. Chron Respire Dis,2006,3:149-159.
18.	Tazawa R,Trapnell BC,Inoue Y,et al.Inhaled granulocyte/macrophage-colony stimulating factor as therapy for pulmonary alveolar proteinosis.Am J Respir Crit Care Med,2010,181:1345-1354.

1. 余巍巍, 曾錦榮,王昌明,等.國內20年肺泡蛋白沉積癥誤診原因匯總分析.中國綜合臨床,2009,25:1276-1278.
2. Rosen SH,Castleman B,Liebow AA.Pulmonary alveolar proteinosis. N Engl J Med,1958,258:1123-1142.
3. 張弘, 邵池,彭敏,等.肺泡蛋白沉積癥84例誤診誤治分析.中國實用內科雜志,2009,29:426-428.
4. Shah PL,Hansell D,Lawson DR,et al. Pulmonary alveolar proteinosis:clinical aspects and current concepts on pathogenesis. Thorax,2000,55:67-77.
5. 朱元玨, 陳文彬,主編.呼吸病學.北京:人民衛生出版社,2002,1106-1111.
6. 張新靜, 肖永龍,蔡后榮,等.自身免疫性肺泡蛋白沉積癥患者臨床影像學特征與生物學標志的關系.中國呼吸與危重監護雜志,2014,13:389-393.
7. Arai T,Inoue Y,Sugimoto C,et al.CYFRA 21-1 as a disease severity marker for autoimmune pulmonary alveolar proteinosis. Respirology,2014,19:246-252.
8. 劉娟, 向萍.可疑慢性粉塵接觸致肺泡蛋白沉積癥1例報道.重慶醫學,2014,43:4415-4416.
9. 葉紹色. 接塵工人肺泡蛋白沉積癥10例臨床分析.應用預防醫學,2010,16:300-301.
10. Bonella F,Bauer PC,Griese M,et al.Pulmonary alveolar proteinosis:New insigh from a single-center cohort of 70 patients.Respir Med,2011,105:1908-1916.
11. Cummings KJ,Nakano M,Omae K. Indium lung disease.Chest,2012,141:1512-1521.
12. 王志兵, 張向左.肺泡蛋白沉積癥的影像學特點分析.華北煤炭醫學院學報,2010,12:352.
13. Sihoe AD,Ng VM,Liu RW,et al. Pulmonary alveolar proteinosis in extremis:the case for aggressive whole lung lawage with extracorporeal membrane oxygenation suppo.Heart Lung Circ,2008,17:69-72.
14. 徐凱峰, 陳燕,郭子健,等.肺泡蛋白沉積癥患者血清中抗粒-巨噬細胞集落刺激因子抗體等血清學指標的臨床意義.中華結核和呼吸雜志,2004,27:824-828.
15. 聞勝蘭, 吳曉虹,應可凈,等.大容量全肺灌洗治療重度肺泡蛋白沉積癥五例分析.中華結核和呼吸雜志,2004,27:598-600.
16. 楊俊, 江丹,盧進昌,等.大容量全肺灌洗治療肺泡蛋白沉積癥10例療效分析.浙江臨床醫學,2014,16:397-398.
17. Ioachimescu OC,Karuru MS.Pulmonary alveolar proteinosis. Chron Respire Dis,2006,3:149-159.
18. Tazawa R,Trapnell BC,Inoue Y,et al.Inhaled granulocyte/macrophage-colony stimulating factor as therapy for pulmonary alveolar proteinosis.Am J Respir Crit Care Med,2010,181:1345-1354.

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