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。
Objective To evaluate the efficacy and safety of whole lung lavage in the treatment of pulmonary alveolar proteinosis ( PAP) .Methods Twelve patients who were diagnosed as PAP from September 2008 to October 2011 in Hunan Occupational Disease Hospital were recruited in the study. The changes of dyspnea symptom, lung-function, arterial blood gas, and chest image were compared before and after whole lung lavage treatment. Meanwhile, the safety of lung lavage was evaluated. Results All patients were relieved from dyspnea. The lung function, hypoxia, hyperventilation, and chest image were all obviously improved. The vital signs in the process of lung lavage were stable without serious complications. Conclusion Whole lung lavage is an effective and safe treatment for PAP.
ObjectiveThree cases of pulmonary tuberculosis misdiagnosed as pulmonary alveolar proteinosis were reported and analyzed in combination with literatures, so as to improve the ability of differential diagnosis of these two diseases. MethodsThe clinical data of 3 cases of pulmonary tuberculosis patients which were diagnosed by pathology whose imaging manifestations were similar to those of pulmonary alveolar proteinosis were collected and reviewed in combination with relevant literature. ResultsAll the 3 patients were male, with a chronic course , no typical clinical manifestations of pulmonary tuberculosis, CT imaging showed diffuse glass grinding shadow, thickened pulmonary lobular septa ,showed "Crazy-paving pattern". ALL the three patients were considered as " pulmonary alveolar proteinosis" initially, and finally confirmed by lung biopsy or acid-resistant bacilli found by bronchoalveolar lavage. Reviewing 8 literature reports with similar imaging findings, 1 case was misdiagnosed as pulmonary alveolar proteinosis, 3 cases were pulmonary alveolar proteinosis combined with pulmonary tuberculosis, and 4 cases were secondary pulmonary alveolar proteinosis. It was found that most patients had systemic or respiratory symptoms of pulmonary tuberculosis. CT images mainly showed diffuse ground glass shadows in bilateral lungs with thickening of lobular septa, and 3 patients also showed clustered small nodulars. Most patients improved after anti-tuberculosis treatment, with only one patient dying. ConclusionsThe imaging manifestations of atypical pulmonary tuberculosis are various, which are easy to be misdiagnosed when they are similar to " pulmonary alveolar proteinosis". Clinicians should raise their awareness of tuberculosis with this imaging characteristic.
Objective To investigate the colonization, risk factors and prognosis of Pneumocystis jirovecii (P.jirovecii) colonization in patients with Pulmonary alveolar proteinosis (PAP). Methods The patients with Pulmonary alveolar proteinosis who were admitted to the Department of Respiratory and Critical Care Medicine, Nanjing Drum Tower Hospital from March 2019 to December 2022 were retrospectively analyzed. Polymerase chain reaction/next-generation metagenomic sequencing were used to detect the colonization of P. jirovecii in bronchoalveolar lavage fluid, and then to investigate the colonization rate, risk factors and outcome of P. jirovecii in PAP patients. Results A total of 25 patients were included in the study, of which 7 were colonized by P. jirovecii (28.0%). The rate of using antibiotics before admission in the colonizing group was significantly higher than that in the non-colonizing group (85.7% vs 33.3%, P=0.030). Total blood lymphocytes (1.4×109/L vs. 1.8×109/L, P=0.048), CD3+T cells (0.83×109/L vs. 1.34×109/L, P=0.010), CD4+T cells (0.48×109/L vs. 0.85×109/L, P=0.010) were significantly lower than those in the non-colonizing group, lactate dehydrogenase (469.9 U/L vs. 277.3 U/L, P=0.005) was significantly higher than those in the non-colonizing group. A higher proportion of colonizing group required combination therapy (57.1% vs. 11.1%, P=0.032); but there was no significant difference in the percentage of whole-lung ground-glass opacification, lung function, oxygen index and outcome. Lactate dehydrogenase was positively correlated with the percentage of whole-lung ground-glass opacification of PAP, but negatively correlated with oxygen index, percentage of predicted forced vital capacity and percentage of predicted diffusion capacity for carbon monoxide. Conclusions The colonization rate of P. jirovecii in PAP patients was high. Reduced lymphocyte count in peripheral blood of PAP patients and antibiotic use before diagnosing were risk factors for P. jirovecii colonization.
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.
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 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.