Objective To summarize the clinical characteristics of patients with diffuse parenchymal lung disease (DPLD) combined with hematological diseases in order to improve the clinicians’ knowledge of these diseases. Methods The clinical data of 46 patients was collected, who were hospitalized in Nanjing Drum Tower Hospital from January 2010 to October 2020 for DPLD combined with hematological diseases. Their clinical manifestations, laboratory tests, imaging features, diagnostic methods, treatment and prognosis were analyzed retrospectively. Results Among the 46 patients, there were 26 males and 20 females, with an average age of 60±13 years old. The main symptoms were cough and sputum, dyspnea, fever, chest tightness, and so on. Laboratory tests showed that some patients had pancytopenia or two-line cytopenia, and increase in lactate dehydrogenase, C-reactive protein, erythrocyte sedimentation rate and β2-microglobulin. Bilateral ground glass opacity, consolidations, big or small nodules, reticular shadows, and traction bronchiectasis were showed on chest high-resolution computed tomography. Among the 13 patients who were diagnosed clearly by pathology, they had 5 cases of organizing pneumonia, 4 cases of pulmonary alveolar proteinosis, 2 cases of acute fibrinous and organizing pneumonia, 1 case of diffuse alveolar hemorrhage, and 1 case of lung amyloidosis. Thirty-three patients were clinically diagnosed, including 3-case drug-induced interstitial lung disease, and 1-case exogenous allergic alveolitis. The patients with diffuse pulmonary lesions as the first manifestation and subsequently diagnosed with hematological diseases accounted for 65.2% (30/46). Among these patients, 2 of them had two kinds of hematological diseases at the same time. In the rest of the 16 cases, hematological diseases were diagnosed before DPLD. Among the 46 cases, 26 patients improved after treatment, 18 of them were treated with glucocorticoid, 8 with N-acetylcysteine and pirfenidone, 4 with granulocyte-macrophage colony stimulating factor inhaling and/ or whole lung alveolar lavage, and 2 with clarithromycin for immune regulation, etc. Fifteen patients refused treatment and transferred back to local hospital after the diagnosis of hematological diseases. Five patients died, 2 of them died of respiratory failure and 3 of them died of diseases progression. Conclusions DPLD includes many kinds of diseases, with known or unknown etiology and lack of specificity in clinical manifestations. Therefore, diagnosis for them is quite difficult. Hematological diseases themselves can be the causes of DPLD. At the same time, the treatment for hematological diseases and the related immunosuppression after treatment can also cause DPLD. In the clinical practice, careful screening and systematic differentiation are urgently needed in order to treat different causes precisely, control the conditions and improve the prognosis.
ObjectiveTo improve clinicians' awareness of cryptogenic organizing pneumonia (COP).MethodsThirty-three inpatients with COP, who had been diagnosed by pathology in Nanjing Drum Tower Hospital during January 2013 to December 2016 were collected. Their clinical manifestations, laboratory tests and imaging data were reviewed and analyzed retrospectively.ResultsThirty-three cases consisted of 18 males and 15 females, and the mean age was (58.7±13.5) years old. Most patients had subacute or insidious onset. The common symptoms were cough, fever, shortness of breath and chest tightness. About half of patients revealed inspiratory crackles or velcroes. Autoantibodies and anti-neutrophil cytoplasmic antibodies were negative. High-resolution computerized tomography findings of COP included bilateral patchy areas of air-space consolidation that showed predominantly subpleural or peri-bronchovascular distribution, focal nodules, enlarged hilar or mediastinal lymph nodes and pleural effusion. 25 patients were treated with glucocorticoid, 6 with macrolid, and 2 were only followed up without drug treatment.ConclusionsClinical manifestations, laboratory tests and imaging features are important clues to diagnose COP. Diagnosis depends on pathology. Meanwhile, definite pathogen and potential underlying diseases must be excluded.
ObjectiveTo summarize the clinical, radiological and pathological characteristics of acquired immune deficiency syndrome (AIDS) combined with Pneumocystis carinii pneumonia (PCP), so as to improve the clinicians' understanding of the disease. MethodsThe clinical data of 50 AIDS patients combined with PCP admitted between February 2006 and May 2015 were retrospectively analyzed, including medical history, physical signs, laboratory examination, chest high resolution CT (HRCT), pathological characteristics, treatment and prognosis, etc. ResultsThe clinical features of AIDS patients combined with PCP included cough, dyspnea and fever, without obvious positive signs in the lung.The patients were divided as a mild group, a moderate group and a severe group according to the levels of PaO2.There was significant difference among three groups in serum albumin level [(23±3) g/L vs. (30±5) g/L and (28±6) g/L, P < 0.01].There were no significant differences among three groups in CD4+ T lymphocyte and lactate dehydrogenase (LDH) (P > 0.05).The typical chest radiograph feature of HRCT was ground-glass shadows in both lungs, and may be associated with reticular shadows or "gravel sign" and cyst.Of 50 patients, 16 patients were diagnosed via pathology of transbronchial lung biopsy(TBLB) and only 5 patients were diagnosed via silver staining of the bronchoalveolar lavage fluid (BALF).The other patients were clinically diagnosed.100% of the patients were treated with sulfamethoxazole (SMZco), 64%with caspofungin, and 72% with glucocorticoid.All the patients relieved with no death in hospital. ConclusionWhen a patient got cough, dyspnea and fever, especially ground glass on HRCT in both lungs, AIDS combined with PCP should be highly considered, and diagnostic treatment with SMZco and CD4+ T lymphocyte measurement should be conducted as soon as possible, so as to reduce misdiagnosis and mortality.
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.
ObjectivesTo compare the clinical features of combined pulmonary fibrosis and emphysema (CPFE) and idiopathic pulmonary fibrosis (IPF).MethodsEighty-three patients diagnosed as CPFE or IPF for the first time were retrospectively analyzed from June 2014 to July 2018 in Nanjing Drum Tower Hospital, including 47 patients in the CPFE group and 36 in the IPF group. The demographic characteristics, clinical manifestations, pulmonary function, cardiac ultrasound, blood gas analysis and prognosis of the two groups were compared.ResultsThe proportion of smokers in the CPFE group was higher than IPF group (P<0.05), but dyspnea was lower (P<0.05). The FVC, FVC%pred, FEV1, FEV1%pred and VC% of the CPFE group were higher than IPF group (P<0.05), while FEV1/FVC%pred in the IPF group was higher than CPFE group (P<0.05). DLCO/VA%pred of CPFE group decreased more significantly than IPF group (P<0.05), RV/TLC%pred of CPFE group increased annually, while decreased annually in IPF group (P<0.01). The RV%pred of CPFE increased annually, while that of IPF group decreased annually (P<0.05). There was no significant difference in arterial oxygen pressure and pulmonary artery pressure between the two groups. As for prognosis, the 1- and 3-year survival rate of the CPFE group were 87.9% and 73.8% respectively, those of the IPF group were 84.1% and 65.8% respectively, and no significantly difference was observed between two groups (P=0.95).ConclusionsCompared with IPF, patients with CPFE usually have more smokers, less proportion of dyspnea, almost normal lung volume, more rapidly decreased DLCO/VA%pred, and no significant difference in prognosis.
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.