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.	李先锋, 姜梹, 徐海鹰, 等. 胸部 CT 快速鉴别诊断支原体肺炎与链球菌肺炎价值研究. 临床军医杂志, 2018, 46(3): 362-363.
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12.	李慧, 邱玉英, 黄妹, 等. 影像学表现为簇性微结节聚集征的肺结核一例. 中华结核和呼吸杂志, 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.	孟芝兰, 刘鸿瑞, 梁智勇, 等. 肺泡蛋白沉积症的病理学特点和诊断. 中华病理学杂志, 2005, 34(9): 575-578.

1. 佘巍巍, 王昌明, 莫碧文, 等. 肺泡蛋白沉积症 32 例临床和病理及影像学对照研究. 重庆医学, 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, 张燕, 韩志海, 等. 肺泡蛋白沉积症临床观察及文献回顾分析. 中国临床医生杂志, 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. 许巍, 申昆玲. 儿童肺泡蛋白沉积症. 中华实用儿科临床杂志, 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. 许传礼, 许静, 马严刚. 肺泡蛋白沉着症的 CT 诊断与鉴别诊断. 中国中西医结合影像学杂志, 2018, 16(3): 289-292.
9. 李菲, 唐笑先, 师建强, 等. 肺泡蛋白沉积症的临床特点和多层螺旋 CT 表现. 实用医学影像杂志, 2017, 18(5): 406-409.
10. 李先锋, 姜梹, 徐海鹰, 等. 胸部 CT 快速鉴别诊断支原体肺炎与链球菌肺炎价值研究. 临床军医杂志, 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, 黄妹, 等. 影像学表现为簇性微结节聚集征的肺结核一例. 中华结核和呼吸杂志, 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. 孟芝兰, 刘鸿瑞, 梁智勇, 等. 肺泡蛋白沉积症的病理学特点和诊断. 中华病理学杂志, 2005, 34(9): 575-578.

West China Medical Journal

Clinicopathological analysis of 25 patients with pulmonary alveolar proteinosis

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