目的:肺泡蛋白沉积症(pulmonary alveolar proteinosis,PAP)是一种少见的肺部疾病,由于临床医生认识不足,容易误诊,而PAP通过灌洗治疗常常可获得较好的预后。本文通过分析PAP病例,总结PAP患者的临床特点、影像学表现和治疗方法,以提高该病的诊疗水平。方法:回顾性分析2003年2月～2008年5月四川大学华西医院呼吸科经病理确诊的15例PAP患者临床资料,并观察了全肺灌洗治疗PAP的效果。结果:PAP患者常见临床症状为咳嗽和进行性呼吸困难,体征正常或无特异性。胸部CT可表现为“地图样”、“铺路石样”或间质纤维化改变。全肺灌洗治疗的14例患者临床症状明显缓解。结论:肺泡蛋白沉积症虽然少见,但只要提高认识,诊断并不困难。支气管肺泡灌洗和(或)肺活检是确诊PAP的重要方法,全肺灌洗是治疗PAP的主要方法。
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.
ObjectiveTo highlight the characteristics of secondary pulmonary alveolar proteinosis (PAP) associated with malignant hematological diseases. MethodsThe clinical data of three patients with secondary PAP were analyzed and the related literature was reviewed. ResultsThree patients were diagnosed with secondary PAP by exclusion of primary or autoimmune PAP and denied the history of inhalation of occupational dusts. Two patients with secondary PAP were associated with chronic myelocytic leukemia, and the third one was associated with myelodysplastic syndrome. The performance on HRCT of the PAP associated with hematological malignancy was different from the primary PAP. Three patients were pathologically diagonised by brochoalveolar lavage fluid. One patient was successfully treated with inhalation of granulocyte-macrophage colony-stimulating factor (GM-CSF). ConclusionsSecondary PAP associated with hematological malignancy is very rare. The untypical HRCT is the main cause of misdiagnosis. Some patients may benefit from GM-CSF theatment.
ObjectiveTo determine the diagnostic value of serum KL-6 level in patients with interstitial lung diseases (ILD). MethodsAll the ILD patients enrolled were hospitalized from April 2013 to April 2014. Patients with other pulmonary diseases and healthy subjects were chosen as control groups simultaneously. Serum KL-6 concentrations were measured by chemiluminescent enzyme immunoassay. The association with serum KL-6 level and pulmonary function was analyzed. ResultsThere were 149 ILD patients, 155 patients with other pulmonary diseases, and 64 healthy subjects. The average serum levels of KL-6 were (1 801.86±2 831.36) U/mL, (267.00±124.41) U/mL, (201.28±81.18) U/mL in the patients with ILD, the patients with other pulmonary diseases and the healthy controls, respectively. The sensitivity and the specificity of the serum KL-6 for the diagnosis of ILD was 83.89% and 92.24% respectively when the cut-off level was set at 500 U/mL. The Kappa value was 0.767 (P < 0.001). The best cut-off value of KL-6 was 469.5 U/mL. Serum KL-6 levels in the patients with ILD were significantly higher compared with the patients with chronic obstructive pulmonary disease, pneumonia, tuberculosis, bronchiectasis and the healthy controls, respectively (all P < 0.001). The KL-6 levels in the pulmonary alveolar proteinosis patients were significantly higher compared with the patients with cryptogenic organizing pneumonia (COP), the patients with idiopathic pulmonary fibrosis (IPF) and the patients with connective tissue disease (CTD-ILD) (all P < 0.001). While the KL-6 concentration in IPF and CTD-ILD were significantly higher than that in COP (P=0.003 and P=0.008, respectively). Significant negative correlations were found between the levels of serum KL-6 and vital capacity as a percentage of the predicted value, forced vital capacity as a percentage of the predicted value, forced expiratory volume in one second as a percentage of the predicted value and carbon monoxide diffusing capacity as a percentage of the predicted value (all P < 0.001). Follow-up study showed the levels of serum KL-6 were consistent with clinical efficacy. ConclusionSerum KL-6 level is a reliable serum marker for ILD, and is related with the severity of disease and clinical efficacy.
Objective To evaluate therapeutic efficacy and safety of recombinant human granulocyte-macrophage colony-stimulating factor (GM-CSF) inhalation in patients with recurrent pulmonarv alveolar proteinosis (PAP). Methods Three cases of recurrent PAP were treated by GM-CSF inhalation after whole lung lavage. The clinical data of the pulmonary function and SpO 2, the clinical symptoms and pulmonary lesions were compared before and after treatment. Results The pulmonary function and manifestations were improved obviously after GM-CSF inhalation. Also the ground-glass opacity was improved in high-resolution CT. The pulmonary function and SpO 2 increased obviously after received GM-CSF inhalation. There were no any adverse reactions in 3 cases. Conclusion GM-CSF inhalation therapy is effective and safe in recurrent PAP, but the long-term effect remains to be seen.
ObjectiveTo explore the differential diagnosis value of subpleural bandlike ground-glass opacity (GGO) in thoracic CT in paraquat poisoning pneumonia. MethodsA retrospective study was carried out by retrieving the patients CT database from March 2013 to March 2015. The patients with paraquat poisoning pneumonia, interstitial pneumonia and pulmonary alveolar proteinosis (PAP) were recruited and their radiological characteristics of thoracic CT were analyzed. ResultsA total of 698 newly diagnosed interstitial pneumonia patients were finally enrolled in this study, 392 of them (56.2%) presented with GGO in thoracic CT. A total of 38 newly diagnosed PAP patients and 14 paraquat poisoning patients were enrolled, and GGO presented in thoracic CT of 100.0% and 42.9% of them respectively. Subpleural bandlike GGO was mostly commonly found in 83.3% of the paraquat poisoning pneumonia patients with GGO in thoracic CT, followed by 18.4% of the PAP patiens and 5.6% of the interstitial pneumonia patients with GGO in thoracic CT, which were significantly lower than that in the paraquat poisoning pneumonia patients (P < 05). GGO associated crazy paving pattern in thoracic CT was mostly commonly found in 94.7% of the PAP patients, followed by 0.5% of the interstitial pneumonia patients and none of the paraquat poisoning pneumonia patients. All the PAP patients with subpleural bandlike GGO were found associated with crazy paving pattern, while none of such association was found in the interstitial pneumonia or the paraquat poisoning pnuemonia patients. GGO coexisting with honeycombing and subpleural line were respectively found in 22.7% and 11.2% of the interstitial pneumonia patients, and none of such association was found in the PAP or the paraquat poisoning pneumonia patients. ConclusionsSubpleural bandlike GGO is mostly commonly found in paraquat poisoning pneumonia patients and rarely in PAP and interstitial pneumonia patients. Combined with crazy paving pattern and subpleural line, subpleural bandlike GGO may be a valuable feature in the diagnosis of paraquat poisoning pneumonia patients.
ObjectiveTo describe the clinical,radiographic,and laboratory features of autoimmune pulmonary alveolar proteinosis (PAP) from a single center. MethodsConsecutive autoimmune PAP cases diagnosed in the Nanjing Drum Tower Hospital between January 2006 and December 2012 were recruited in the study. The clinical,radiographic and laboratory data of the PAP patients were analyzed to explore the clinical significance of serum GM-CSF autoantibody (GMAb) and serum cytokeratin (CYFRA21-1). ResultsThe median serum GMAb level of the 26 cases was 28.64 μg/mL (interquartile range,19.2-75.4 μg/mL),which were diagnosed as autoimmune PAP based on the serum GMAb levels of these patients all above the cut-off value of 2.39 μg/mL while the serum GMAb levels of 30 normal controls were 0.10(0.05-0.15)μg/mL and all below the cut-off value. 34.6% of all recruited 26 autoimmune PAP patients had identified occupational inhalational exposure. There was no significant correlation in the serum GMAb in autoimmune PAP patients with disease severity scores (DSS),lung function parameters,chest high resolution computed tomography (HRCT) scores,or PaO2 (P>0.05). There was significant correlation of DSS of autoimmune PAP patients with PaO2,FVC%pred,TLCO%pred,opacity extent score of chest HRCT,and opacity severity score of chest HRCT (P<0.05). The median serum level of CYFRA21-1 of the autoimmune PAP patients was 9.9(4.3-19.5)ng/mL,which was significant higher than that of the normal control group (P<0.05). However there was no significant correlation in the serum CYFRA21-1 in the autoimmune PAP patients with DSS,lung function parameters,and chest HRCT scores. 92.3% of the chest HRCT of 26 autoimmune PAP patients had crazy paving sign,while 100% of them had geographic sparing sign. ConclusionSerum GMAb and CYFRA21-1 may be important biomarkers for diagnosis of autoimmune PAP. The PAP with occupational inhalational exposure constitutes a high proportion of autoimmune PAP patients.