Clinicopathological analysis of 25 patients with pulmonary alveolar proteinosis_West China Medical Journal

Authors：

LIU Qinglin ,  JIANG Lili

Department of Pathology, West China Hospital, Sichuan University, Chengdu, Sichuan 610041, P. R. China;

Corresponding?author：

JIANG Lili, Email: 879876047@qq.com

Keywords：

Pulmonary alveolar proteinosis; Alveolar lavage fluid; Lung biopsy

DOI：

10.7507/1002-0179.201812076

Video：

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ObjectiveTo explore the clinicopathological features of pulmonary alveolar proteinosis (PAP).MethodsA total of 25 patients with PAP who were pathologically diagnosed in West China Hospital of Sichuan University from 2014 to 2018 were collected as the study subjects.ResultsThe 25 patients with PAP were 18–73 years old, with an average age of (42.52±15.79) years. There were 20 males and 5 females. The most common type was autoimmune PAP (15 cases), and secondary PAP (10 cases) were found in patients with pneumonia, tuberculosis, nephrotic syndrome, and pneumoconiosis. The common clinical symptoms of PAP were cough (24 cases), expectoration (20 cases), and progressive difficulty in breathing (11 cases of shortness of breath and 5 cases of dyspnea). The chest CT manifestations included double lung grinding (19 cases), grid-like changes (11 cases), and map-like changes (3 cases). Periodic acid-schiff (PAS) staining and post-digestion PAS staining double positive lipoprotein-like deposits was observed in lung biopsy (in the alveolar cavity) and/or alveolar lavage fluids.ConclusionsPAP has no characteristic clinical symptoms and a long diagnosis period. Clinicians should combine clinical signs, imaging features, and lung biopsy and/or alveolar lavage fluid PAS staining and post-digestion PAS staining to confirm the diagnosis.

Citation： LIU Qinglin, JIANG Lili. Clinicopathological analysis of 25 patients with pulmonary alveolar proteinosis. West China Medical Journal, 2019, 34(1): 18-21. doi: 10.7507/1002-0179.201812076 Copy

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10.	李先鋒, 姜梹, 徐海鷹, 等. 胸部 CT 快速鑒別診斷支原體肺炎與鏈球菌肺炎價值研究. 臨床軍醫雜志, 2018, 46(3): 362-363.
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12.	李慧, 邱玉英, 黃妹, 等. 影像學表現為簇性微結節聚集征的肺結核一例. 中華結核和呼吸雜志, 2016, 39(9): 744-745.
13.	Su KC, Lay SL, Perng RP, et al. Lung cancer may develop subsequently or coincidently with pulmonary alveolar proteinosis. Lung Cancer, 2007, 58(1): 144-148.
14.	Uchiyama M, Nagao T, Hattori A, et al. Pulmonary alveolar proteinosis in apatient with Behcet’s disease. Respirology, 2009, 14(2): 305-308.
15.	孟芝蘭, 劉鴻瑞, 梁智勇, 等. 肺泡蛋白沉積癥的病理學特點和診斷. 中華病理學雜志, 2005, 34(9): 575-578.

1. 佘巍巍, 王昌明, 莫碧文, 等. 肺泡蛋白沉積癥 32 例臨床和病理及影像學對照研究. 重慶醫學, 2010, 39(19): 2576-2577, 2580.
2. Trapnell BC, Whitsett JA, Nakata K. Pulmonary alveolar proteinosis. N Engl J Med, 2003, 349(26): 2527-2539.
3. 田光, 張燕, 韓志海, 等. 肺泡蛋白沉積癥臨床觀察及文獻回顧分析. 中國臨床醫生雜志, 2016, 44(3): 63-65.
4. Zhang D, Tian X, Feng R, et al. Secondary pulmonary alveolar proteinosis: a single-center retrospective study (a case series and literature review). BMC Pulm Med, 2018, 18(1): 15.
5. 許巍, 申昆玲. 兒童肺泡蛋白沉積癥. 中華實用兒科臨床雜志, 2018, 33(4): 317-320.
6. shah PL, Hansell D, Lawson PR, et al. Pulmonary alveolar proteinosis: clinical aspects and current concepts on pathogenesis. Thorax, 2000, 55(1): 67-77.
7. Johkoh T, Itoh H, Müller NL, et al. Crazy-paving appearance at thin-section CT: spectrum of disease and pathologic findings. Radiology, 1999, 211(1): 155-160.
8. 許傳禮, 許靜, 馬嚴剛. 肺泡蛋白沉著癥的 CT 診斷與鑒別診斷. 中國中西醫結合影像學雜志, 2018, 16(3): 289-292.
9. 李菲, 唐笑先, 師建強, 等. 肺泡蛋白沉積癥的臨床特點和多層螺旋 CT 表現. 實用醫學影像雜志, 2017, 18(5): 406-409.
10. 李先鋒, 姜梹, 徐海鷹, 等. 胸部 CT 快速鑒別診斷支原體肺炎與鏈球菌肺炎價值研究. 臨床軍醫雜志, 2018, 46(3): 362-363.
11. Devakonda A, Raoof S, Sung A, et al. Bronchiolar disorders: a clinical-radiological diagnostic algorithm. Chest, 2010, 137(4): 938-951.
12. 李慧, 邱玉英, 黃妹, 等. 影像學表現為簇性微結節聚集征的肺結核一例. 中華結核和呼吸雜志, 2016, 39(9): 744-745.
13. Su KC, Lay SL, Perng RP, et al. Lung cancer may develop subsequently or coincidently with pulmonary alveolar proteinosis. Lung Cancer, 2007, 58(1): 144-148.
14. Uchiyama M, Nagao T, Hattori A, et al. Pulmonary alveolar proteinosis in apatient with Behcet’s disease. Respirology, 2009, 14(2): 305-308.
15. 孟芝蘭, 劉鴻瑞, 梁智勇, 等. 肺泡蛋白沉積癥的病理學特點和診斷. 中華病理學雜志, 2005, 34(9): 575-578.

West China Medical Journal

Clinicopathological analysis of 25 patients with pulmonary alveolar proteinosis

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