ObjectiveTo compare the clinical characteristics,high-resolution computed tomography (HRCT) manifestations,pulmonary function results,serum autoantibodies and treatment modality of connective tissue diseases related interstitial lung diseases (CTD-ILDs) and idiopathic interstitial pneumonias (ⅡPs). MethodsPatients explicitly diagnosed with CTD-ILDs and ⅡPs were retrospectively selected from Nanjing Drum Tower Hospital between January 2004 and December 2012.The clinical features were abstracted,including age,gender,symptoms,signs,serum autoantibody results,HRCT findings,and treatment.Patient characteristics were compared between CTD-ILDs and ⅡPs using a Pearson's χ2 test for categorical variables,and a Student's t test for continuous variables. ResultsA total of 692 patients with complete data were included in this study,with 240 CTD-ILDs cases and 452 ⅡP cases.CTD-ILDs could exist in different types of CTDs,which were mainly shown in Sjogren's syndrome,rheumatoid arthritis,and dermatomyositis/polymyositis.Age,gender,connective tissue diseases related characteristics such as dry eyes,dry mouth,and arthralgia,and several autoantibodies such as ANA,SSA,SSB all showed significantly difference between CTD-ILDs and ⅡPs (P<0.05).However there were no significant differences in cough,dyspnea after exertion,velcro crackles on auscultation,or finger clubbing between two groups (P>0.05). The HRCT manifestations of CTD-ILDs were reticular opacities,patchy consolidation,band-like shadows,and pleural thickening.Pulmonary function tests commonly showed restrictive lung function and decreased diffusing capacity.The histopathologic findings of lung biopsies of CTD-ILDs were mostly chronic inflammatory cell infiltration,as well as hyperplasia of fibrous tissue and septal thickness.The finding of chronic inflammatory cell infiltration showed significant difference between CTD-ILDs and ⅡPs (P<0.05),while the HRCT manifestations,pulmonary function results or other histopathologic findings did not(P>0.05).The current treatment modality was corticosteroids plus immunosuppressants. ConclusionDespite the similarities,CTD-ILDs show distinct clinical,laboratory and imaging features from from ⅡPs in clinical practice.
ObjectiveTo highlight the characteristics of pulmonary MALT lymphoma with diffuse lung disease. MethodsThe clinical,radiological and pathological data of two patients with pulmonary MALT lymphoma were analyzed,and relevant literature was reviewed. ResultsOne patient was a 59-year-old male with cough for five years while antibiotic treatment was ineffective. The chest CT scan demonstrated diffuse lung disease,bilateral multiple consolidation and ground-glass opacities,small nodules and bronchiectasis. Thoracoscopy biopsy was performed and the pathology study confirmed the diagnosis of MALT lymphoma. Another case was a 50-year-old female,who suffered from fever,cough and dyspnea. The chest CT scan revealed bilateral multiple patchy consolidation,with air bronchogram. The eosinophils count in blood was high. Diagnosed initially as eosinophilic pneumonia,she was treated with corticosteroids. The clinical symptoms were improved,but the CT scan revealed no change. After the computed tomography guided percutaneous lung biopsy,pathological examination confirmed the diagnosis of MALT lymphoma. ConclusionMALT lymphoma with diffuse lung disease is rare and easy to be misdiagnosed. The positive rate of bronchoscopy is low and percutaneous lung biopsy or thoracoscopy biopsy is more useful for diagnosis.
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.
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.
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 improve clinicians' knowledge of hypersensitivity pneumonitis (HP). MethodsWe retrospectively analyzed the clinical data of 24 HP patients who were diagnosed in the Affiliated Drum Tower Hospital of Nanjing University Medical School during February 2005 to February 2013. The clinical,radiological and pathological features of those patients were summarized. ResultsAmong those 24 patients,15 were male and 9 were female,with mean age of (48±13) years. All patients had a history of environmental exposure. Two patients showed acute clinical manifestations,and there were 17 subacute and 5 chronic cases. The main clinical manifestations were dyspnea,cough,sputum,fever and weight loss with hypoxemia via blood gas analysis. Restrictive ventilatory impairment was the most frequent functional pattern,and the carbon monoxide diffusing capacity was decreased. Pulmonary function test showed restrictive ventilatory defect and gas interchange disturbance. The features of chest HRCT included diffuse ground-glass attenuation and/or patchy consolidation,centrilobular micronodules,mosaic sign,reticular and/or honeycombing lesions. Bronchoalveolar lavage fluid (BALF) demonstrated an increase of total cell counts with predominant lymphocytosis. The transbronchoscopic lung biopsy (TBLB) pathological examination revealed lymphocytic alveolitis,noncaseating granuloma,and interstial pneumonia. All patients were treated by corticosteroid and avoided antigen exposure and showed significant clinical and radiological improvement. ConclusionThe diagnosis of HP is difficult. In most cases (acute and subacute HP),a diagnosis can be made by combination history of exposure,chest HRCT manifestations,cell classification of BALF and pathological examination of TBLB. For atypical cases (chronic HP),a surgery lung biopsy is needed for multi-disciplinary diagnosis including pathologist,radiologist and pulmonologists.
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 analyze the clinical characteristics and survival of pulmonary fibrosis (PF) patients complicated with lung cancer (LC) (PL-LC). MethodsFifty-three patients with PF diagnosed as LC from January 2008 to March 2014 in Nanjing Drum Tower Hospital were included in this study. Univariate analysis and Cox regression analysis were used to detect the effects of clinical variables on survival. Kaplan-Meier method was used to calculate the median survival time (MST) and overall survival (OS). ResultsMale patients (n=48, 90.6%) and patients with a history of smoking (n=42, 79.2%) were more easily suffered from PF-LC. The average age was 68.6±9.5 years. Cox multivariate analysis revealed that Velcro crackles (P=0.009) and clinical stage (P=0.013) were the independent risk factors of survival in the patients with PF-LC. The MST of 53 patients was 6.0 months.The survival rates of 1-year and 2-year were 34.1% and 22.0%, respectively. Forty-two (79.2%) patients were idiopathic pulmonary fibrosis (IPF) complicated with LC, and 11 (20.8%) patients were secondary pulmonary fibrosis (SPF) complicated with LC. OS difference between two groups was not significant (P=0.610). OS of NSCLC group (n=37) was significantly prolonged than that of SCLC group (n=6) and unclassified pathological pattern group (n=10) (P=0.035). OS of Ⅰ and Ⅱstage patients (n=13) was significantly longer than that of Ⅲ and Ⅳ stage patients (n=40) (P=0.002). MST and OS of patients with LC treated (n=31) were significantly better than those of untreated patients (n=22) (P < 0.001) and OS of patients treated by comprehensive therapy (n=11) was significantly prolonged than that of patients treated by mono-therapy (n=20) (P=0.036). ConclusionsVelcro crackles and clinical stages are the independent risk factors of prognosis in PF-LC patients. It is beneficial to survival if the PF patients with LC were treated by comprehensive therapy.
Objective To analyze the risk factors of invasive pulmonary aspergillosis (IPA) in patients with interstitial pneumonia. Methods The clinical data of 770 cases of interstitial pneumonia admitted between December 2010 and August 2015 were collected. Among them, 46 cases were combined with IPA and 724 cases were not ombined with IPA. The clinical data was analyzed to explore the risk factors of IPA in patients with interstitial pneumonia. Results Univariate analysis showed that in the aspects of age (t=3.348, P=0.001), serum albumin level (t=8.381, P < 001), broad-spectrum antibiotic used within 3 months (χ2=87.157, P < 001), long-term administration of glucocorticoid (χ2=57.462, P < 001), long-term administration of immunosuppressive agents (χ2=31.715, P < 001), imaging in UIP type (χ2=20.632, P < 001), diabetes mellitus (χ2=9.737, P=0.002) and heart failure (χ2=9.300, P=0.002), there were significant differences between two groups. After multivariate logistic regression analysis, broad-spectrum antibiotic used within 3 months (OR=4.773, P < 001), long-term administration of glucocorticoid (OR=9.195, P < 001), long-term administration of immunosuppressive agents (OR=2.662, P=0.046), imaging in UIP type (OR=5.725, P < 001), and diabetes mellitus (OR=3.847, P=0.003) were found to be the risk factors of IPA in patients with interstitial pneumonia. Serum albumin level was negatively correlated with the occurrence of IPA in patients with interstitial pneumonia. Conclusions Various factors contribute to the occurrence of IPA in patients with interstitial pneumonia. Miscellaneous appropriate measures should be taken to reduce the incidence of IPA.