Characteristics and Prognosis of Microscopic Polyangiitis Patients with Diffuse Alveolar Hemorrhage and Interstitial Lung Disease.

Objective To evaluate the clinical characteristics and prognostic predictors of patients with diffuse alveolar hemorrhage (DAH) and/or interstitial lung disease (ILD) secondary to microscopic polyangiitis (MPA) in a Chinese general hospital. Methods We retrospectively reviewed the medical records of MPA patients admitted to internal medicine departments between the year 2002 and 2012. The patients were divided into the ILD, DAH, DAH combined with ILD (DAHILD), and no pulmonary involvement (NPI) groups according to pulmonary involvement patterns. The clinical characteristics at diagnosis were analyzed. The risk factors associated with short-term death and long-term death were identified with Logistic regression and Cox analysis.Results Of 193 newly diagnosed MPA patients, 181 patients were enrolled in the research, of which 19 had DAH alone, 96 had ILD alone, 18 had DAH and DAH concurrently, and 48 had NPI. The median of serum creatine level in the DAH group was 449 µmol/L, significantly higher than that in the ILD group (123 µmol/L, Nemenyi = -35.215, P = 0.045) and DAHILD group (359 µmol/L, Nemenyi = -43.609, P = 0.007). The median follow-up time was 67 (range: 1-199) months. Patients in the ILD group were older than those in the DAH group (median: 69 years vs. 57 years, Nemenyi = 43.853, P= 0.005). The patients with both DAH and ILD had combined features of the two subtypes, and the highest mortality (72.2% at the end of follow-up). The elevated white blood cell count was a risk factor for short-term death (OR = 1.103, 95%CI: 1.008-1.207, P = 0.032 for one month; OR = 1.103, 95%CI: 1.026-1.186, P = 0.008 for one year). Old age (HR= 1.044, 95%CI: 1.023-1.066, P < 0.001), cardiovascular system involvement (HR = 2.093, 95%CI: 1.195-3.665, P = 0.010), poor renal function (HR = 1.001, 95%CI: 1.000-1.002, P = 0.032) were risk factors for long-term death. Pulmonary infections (38/54) were the leading causes of death, especially for the patients with ILD. Besides, 49 patients suffered from pulmonary infections in the first year after diagnosis. Conclusions MPA patients who presented with different pulmonary involvement patterns have completely different clinical features. These subtypes probably have different pathogenesis and should be studied separately.

[Clinical Features of Coccidioidomycosis:Analysis of 33 Chinese Cases].

Objective To summarize the characteristics of Chinese coccidioidomycosis cases, improve the diagnosis and treatment of this disease and prevent misdiagnosis as well as therapeutic error.Methods Search in databases including Medline,Wanfang,and CNKI using "Coccidioidomycosis" and "China" as index words yielded 23 articles that reported a total of 32 Chinese coccidioidomycosis cases.In addition,one patient with disseminated coccidioidomycos was treated in our center in April 2016.The demographic data,site of infection,clinical manifestations,past medical history,exposure history,imaging and laboratory findings,and pathological features of these 33 patients were analyzed.Results Among these 33 patients,7(21.2%)had visited an epidemic area and 6(18.2%)were immunocompromised.The disease involved the respiratory system,skin,bone,central nervous system,cornea,and stomach in 24,6,3,2,1,and 1 patients,respectively.Eight patients (24.2%) had multiple system involvement,and three of them died.The imaging findings included pulmonary nodules(n=14),mediastinal lymphadenopathy(n=5),solid shadow(n=4),cavity(n=4),pleural effusion(n=3),multiple plaques(n=2)and masses(n=2).Coccidiolys cysts were detected in the affected tissues(n=28)or in pus,exudate or pleural smear(n=3);in addition,coccidioides mycelium and spores were found in the sputum,pus,and tissue cultures in 4 cases,among whom only 2 cases were confirmed by serological examination.The treatments included triazoles(n=20),systemic or local administration of amphotericin B(n=13),surgical resection of the lesion(n=8),and intravenous gamma globulin(n=1).Five patients died,among whom three had underlying diseases that caused immunosuppression and one was an infant.The prognoses were relatively good in the remaining patients.Conclusions Early diagnosis and proper treatment can achieve good prognosis in coccidioidomycosis patients.Multi-system involvement and immunosuppression are risk factors for poor prognosis of coccidioidomycosis.For these patients,adequate and full-course medication may prevent rapid disease progression.

[Clinical characteristic analysis of 96 cases of hypersensitivity pneumonitis].

OBJECTIVE: To improve understanding of the clinical characteristics and diagnosis of hypersensitivity pneumonitis (HP). METHODS: We retrospectively analyzed the clinical data, including clinical symptoms, laboratory tests, exposure, pulmonary function tests, chest CT imaging and cytological classification of bronchoalveolar lavage (BAL) of 96 patients with HP from Jan 2001 to Jun 2011 in Peking Union Medical College Hospital. We divided the patients into 2 groups: a pathologically-confirmed group and a clinically-suspected group. RESULTS: There were 58 females and 41 males. The median age at the diagnosis was 53 years. The most common exposures were low-molecular-weight chemicals (42.7%) and animal proteins (37.5%). Common clinical symptoms included dyspnea on exertion (90.6%) and cough (76.0%). Pulmonary function test showed diffusion abnormality (73.5%) and restrictive ventilatory impairment (59.7%). Chest CT scan revealed patchy or diffuse bilateral ground-glass opacities (64.6%), centrilobular nodules (21.9%), and air trapping (15.6%). Reticulation (45.8%), traction bronchiectasis (21.9%) and honeycombing(9.4%) were present in chronic HP. BAL lymphocyte counts > 0.2 and CD4/CD8 < 0.9 were more commonly seen in patients with a disease course of less than 1 year. The pathologically-confirmed group and the clinically-suspected group shared many similar characteristics including age at diagnosis, gender, clinical manifestation, pulmonary function impairments and imaging findings, but significant differences existed in certain parameters. In the pathologically- confirmed group, the duration of disease was longer (24 months vs 6 months, Z = -2.492, P = 0.013) and clubbed fingers were more common (23.4% vs 8.2%, χ(2) = 4.227, P = 0.040). Diffusion abnormality was present in more patients of this group (90.7% vs 44.0%, χ(2) = 35.219, P < 0.01). By CT scan, reticulation, traction bronchiectasis and honeycombing (57.5% vs 26.5%, χ(2) = 9.434, P < 0.01) were more evident as compared to the clinically-suspected group. The value of transbronchial lung biopsy for diagnosing HP was limited, with a positive result of only 8.2%. Surgical lung biopsy was needed in uncertain cases. CONCLUSION: The diagnosis of HP was difficult. In some cases a clinical diagnosis can be made by combination of history of exposure, CT manifestations and cell classification of BAL. For atypical cases a multi-disciplinary approach including pathologists, radiologists and pulmonologists is needed.

[The clinicopathological analysis of 4 cases of IgG4-related nonspecific interstitial pneumonia].

OBJECTIVES: To observe the immunohistochemical staining of IgG4 in nonspecific interstitial pneumonia (NSIP) and to study the clinicopathological features of IgG4-related NSIP. METHODS: Retrospective analysis was carried out for 32 patients with NSIP who had been admitted into Peking Union Medical College Hospital from November 2002 to October 2010. The diagnosis of NSIP was established by surgical lung biopsy and all specimens were fixed in neutral formalin and embedded in paraffin. Sections were cut for HE and immunohistochemical stain. According to the diagnostic criteria for IgG4-related disease, 4 cases were confirmed to be IgG4-related NSIP. The clinicopathological features including clinical history, laboratory examination, and pathologic evaluation were studied. RESULTS: The 4 patients with IgG4-related NSIP included 1 man and 3 women, with a median age of 48 years (range, 44 - 56 years). The presenting symptoms were dry cough or shortness of breath. One patient (1/4, 25.0%) was found to have a positive autoantibody but no cases showed positive RF in serum. The histological finding of the 4 cases was characterized by inflammatory cell infiltration in interstitium with fibrosis, and 1 case showed obliterative arteritis. The numbers of IgG4-positive plasma cells in the 4 cases were 42/hpf, 22/hpf, 11/hpf, and 33/hpf respectively, while the percentages of IgG4-positive to IgG-positive plasma cells were 70%, 71%, 57%, 43% respectively. CONCLUSIONS: IgG4-related interstitial pulmonary disease can be characterized as the NSIP pattern. The pathological features of IgG4-related NSIP include infiltration of lympho-plasmacytes and eosinophils in interstitium with fibrosis, and lymphoid follicles are frequently identified in the area of lymphocyte aggregation, but obliterative arteritis is infrequently identified in the lesion. Immunohistochemical staining of IgG and IgG4 is very helpful for a definite diagnosis of IgG4-related disease.

[The diagnostic value of CT-guided percutaneous needle lung biopsy in diffuse parenchymal lung diseases].

OBJECTIVE: This study was to evaluate the efficacy and limitation of CT-guided percutaneous cutting needle lung biopsy in the diagnosis of diffuse parenchymal lung diseases (DPLD). METHODS: A total of 481 patients admitted in Peking Union Medical College Hospital from January 2000 to December 2008 underwent CT-guided percutaneous cutting needle lung biopsy. The patients were evaluated by clinical history, physical examination and lung HRCT. Those with localized opacity or lesions in a single lung in the CT scan were excluded. Finally, 248 patients with DPLD in HRCT were enrolled for this study. RESULTS: The study patients included 114 males and 134 females, and the mean (± SD) age at diagnosis was 50 ± 16 (range from 13 - 78) years. Confirmed diagnosis by percutaneous needle lung biopsy was obtained in 130 patients (52.4%), including pulmonary infection (35.4%, 46/130), pulmonary malignant diseases (25.4%, 33/130), bronchiolitis obliterans organizing pneumonia/organizing pneumonia (22.3%, 29/130), pulmonary vasculitis (6.2%, 8/130), granulomatous lesions (4.6%, 6/130), pulmonary sarcoidosis (2.3%, 3/130), acute interstitial pneumonia (1.5%, 2/130), pulmonary amyloidosis (1.5%, 2/130), and pulmonary alveolar proteinosis (0.8%, 1/130). Open lung biopsy/video-assisted thoracoscopic surgery was performed in 37 out of 118 cases for which the diagnosis was undetermined by percutaneous lung biopsy. Confirmed diagnosis was obtained in 36 patients, including non-specific interstitial pneumonia (NSIP, 33.3%, 12/36), usual interstitial pneumonia (UIP, 8.3%, 3/36), pulmonary infection (16.7%, 6/36), neoplasm (8.3%, 3/36), lymphoid interstitial pneumonia, pulmonary vasculitis (5.6% 2/36), hypersensitivity pneumonitis (5.6%, 2/36), and pulmonary sarcoidosis, allergic bronchopulmonary aspergillosis, pulmonary hyalinizing granuloma, pneumoconiosis, Castleman's disease, and lymphoproliferative disorder (1 case respectively). CONCLUSION: CT-guided percutaneous cutting needle lung biopsy can provide confirmed diagnosis in half of patients with DPLD, and has a high diagnostic yield in patients with infectious or neoplastic diseases, but it is not a good method for diagnosis of interstitial lung diseases such as NSIP and UIP.

Pathological and high resolution CT findings in Churg-Strauss syndrome.

OBJECTIVE: To investigate the Churg-Strauss syndrome (CSS) associated lung involvement, concentrating on clinical characteristics, pathological findings of lung involvements, response to treatment, and prognosis. METHODS: We retrospectively analyzed the characters of the clinical manifestations, thin-section CT and pathological findings of CSS. The study involved 16 patients. Clinical data were obtained by chart review. All patients underwent transbronchial lung biopsy (TBLB). Six of them underwent surgical lung biopsy as well. RESULTS: The patients included 7 men and 9 women, aged from 14 to 61 years (median, 47.5 years). Extrathoracic organs involved included nervous system (7/16) and skin (5/16). Respiratory symptoms included cough (12/16), exertional dyspnea (11/16), hemoptysis (4/16), and chest pain (3/16). CT findings included bilateral ground-glass opacities (12/16), bilateral patchy opacities (12/16), and centrilobular nodules (6/16). The pathological findings of TBLB demonstrated increased eosinophils (3/16), vasculitis (3/16), and interstitial pneumonia (16/16). The pathological findings of surgical lung biopsy of 6 cases showed necrotizing vasculitis in 4 cases, capillaries in 5, eosinophilic pneumonia in 3, granulomas in 2, and airway abnormalities in 3. All patients improved in symptoms after therapy during the study period (range, 3 to 51 months; median, 15 months). CONCLUSIONS: Asthma may be present in CSS patient when there is bronchial involvement. Ground-glass opacities and consolidation seen on high-resolution CT reflect the presence of eosinophilic pneumonia, vasculitis, and pulmonary alveolar hemorrhage. TBLB has significant limitations for the diagnosis of CSS. Early diagnosis and therapy can result in satisfactory prognosis.

[Clinical features of pulmonary involvement in patients with microscopic polyangiitis].

OBJECTIVE: To explore the clinical features of pulmonary involvement in patients with microscopic polyangiitis (MPA). METHODS: We retrospectively investigated the clinical data of 50 patients hospitalized with MPA in Peking Union Medical College Hospital from January 2008 to December 2009, the data included clinical manifestation, laboratory parameters, echocardiography, pulmonary function test, chest computed tomography, and histopathology of kidney. RESULTS: Pulmonary involvements were observed in 46 patients, common symptoms include cough (34/46), expectoration (30/46), dyspnea (19/46) and hemoptysis (16/46). Pulmonary involvement was the initial manifestation in 14 patients, five cases had radiographic evidences of usual interstitial pneumonia before MPA was diagnosed. The prevalence of positive MPO-ANCA antibodies in MPA patients was 96%. The prevalence of positive PR3-ANCA antibodies was 6%. Radiographic manifestations included ground glass attenuation (16/37), interstitial changes (16/37), infiltrates (12/37) and pleural effusion (7/37). The most frequent abnormality in pulmonary function test was reduced carbon monoxide diffusing capacity (12/15) and restrictive ventilation dysfunction (4/15). The incidences of pulmonary hypertension was 33% (13/39), the average pulmonary artery systolic pressure was (48 ± 8) mm Hg (1 mm Hg = 0.133 kPa). CONCLUSION: The prevalence of pulmonary involvement in patients with MPA was high, pulmonary involvement was the initial manifestation in 28% patients. The clinical manifestations were nonspecific, radiographic manifestations included ground glass attenuation, interstitial changes, infiltrates and pleural effusion. The short term prognosis was well in patients with pulmonary involvement treated with systemic corticosteroids and cyclophosphamide, infection was a leading cause of death in patients with pulmonary involvement.

[Non-Hodgkin's lymphoma with diffuse ground-glass opacity on chest CT: a report of 6 cases].

OBJECTIVE: To investigate the clinical, pathological and imaging characteristics, misdiagnosis and treatment of pulmonary non-Hodgkin's lymphoma with diffuse ground-glass opacities (GGO). METHODS: Six cases of pulmonary non-Hodgkin's lymphoma with diffuse GGO on chest CT diagnosed from January 2008 to March 2010 were retrospectively analyzed. RESULTS: There were 5 males and 1 female with average age of 52 years old (range: 30-59). The course had a range of 2-36 months. Most patients presented with dyspnea (n=5) and loss of weight (n=5). Enlargement of superficial lymph nodes (n=2) and hepatosplenomegaly (n=2) were also found. Laboratory tests showed that average hemoglobin decreased to 25 g/L and average serum LDH was 755 U/L. Chest CT showed diffuse GGO (n=2), diffuse GGO with consolidations (n=3), with wide lung septum (n=3), with multiple nodules (n=2), with enlargement of mediastinal lymph nodes (n=2). Diagnosis of the 6 cases were made by lung biopsy. Histological findings including intravascular lymphoma (n=2), diffuse large B cell lymphoma (n=2) and T cell lymphoma (n=2). The average follow-up period was 4 months (range: 2-6). Chemotherapy was administered in 4 patients with B cell lymphoma and all of them improved or remained stable. One patient with T cells lymphoma was lost to follow-up and another patient with T cell lymphoma died due to lung infection. CONCLUSIONS: Non-Hodgkin's lymphoma with diffuse GGO on chest CT scan is rare. And its misdiagnosis is common due to a lack of specific clinical manifestations. And lung biopsy is necessary for an early diagnosis.

[Clinical analysis of acute pulmonary thromboembolism in 68 elderly patients].

OBJECTIVE: To explore the clinical characteristics of acute pulmonary thromboembolism (PTE) in elderly patients, in order to improve the diagnosis and treatment of the disease. METHOD: The clinical data were reviewed for patients aged over 60 years old hospitalized with acute PTE in Peking Union Medical College Hospital from January 2006 to January 2009. RESULTS: The average age of the 68 patients was (72 +/- 6) years old. Deep vein thromboembolism was found in 33 cases. Hypertension (55.9%), cancer (32.4%), surgery (29.4%), immobility (29.4%), diabetes (23.5%) and obesity (20.6%) were the most common risk factors, with 70.6% (48/68) of these patients having more than 2 risk factors. The common symptoms were dyspnea (77.9%), cough (26.5%), chest pain (13.2%), palpitation (13.2%), faint (13.2%) and asymmetric edema of the lower extremities (30.9%). All of the arterial blood gas in 61 cases showed hypoxemia. The positive rate of blood D-dimer elevation was 84% (47/56). The main signs in chest X-ray were infiltrates (37.5%) and pleural effusions (18.8%). Non-specific ST-T changes (59.3%) and sinus tachycardia (23.7%) were the most common abnormalities in electrocardiogram. Forty seven cases (69.1%) were diagnosed through CT pulmonary angiography (CTPA), and 20 cases (29.4%) were through ventilation-perfusion lung scintigraphy. Sixty one cases received anticoagulant therapy and 5 received thrombolytic therapy. Vena cava filters were implanted in 5 patients. Fifty six cases improved after treatment, and the case fatality ratio was 18% (12/68). CONCLUSION: The most common risk factors for PTE in elderly patients were chronic diseases, cancer, surgery and immobility. The symptoms and auxiliary examination of these patients were not specific, and CTPA was the most useful diagnosing tool. Anticoagulation was the basic treatment for PTE in elderly patients and it was safe and effective, while the overuse of thrombolytic therapy and vena cava filter should be avoided.

[Clinical analysis of 45 cases with pulmonary thromboembolism after surgical procedures].

OBJECTIVE: To analyze the occurrence of pulmonary thromboembolism (PTE) after surgical procedures to attract more attention to the prevention, diagnosis and treatment of this disease. METHODS: Retrospectively analyze the clinical data of the hospitalized patients with post-surgical PTE from June 2004 to February 2009. The average age of the 45 cases was (60 +/- 16) years old, 35 cases received anticoagulant therapy and 6 cases received thrombolytic therapy, the other 4 cases only received emergency medical treatment. Analyze the data about the surgery category, duration, anaesthetic way, risk factors, clinical symptoms, auxiliary examinations, diagnosis, treatment and turnover of these patients. RESULTS: Among the total 45 cases of post-surgical PTE, 37 cases (82.2%) occurred within 2 weeks, it accounted for 13.2% (45/341) of the hospitalized PTE patients during that period. PTE was often seen in patients after major surgical operation such as general (35.6%), gynecological (13.3%), orthopedic (13.3%) and chest surgery, especially the surgery related to malignant tumor (57.8%). The average surgical duration was (220 +/- 124) min, 37 cases (82.2%) was given general anaesthesia. The clinical manifestations and auxiliary examinations results of post-surgical PTE were not typical. Thirty-six cases improved after treatment, 9 cases died and the case fatality ratio was 20.0% (9/45). CONCLUSIONS: Surgical procedure is an important risk factor of PTE. The prevention diagnosis and treatment of post-surgical PTE should be paid more attention to.

Comparing different venous thromboembolism risk assessment machine learning models in Chinese patients.

OBJECTIVE: Venous thromboembolism (VTE) is a fatal complication and the most common preventable cause of death in hospitals. The risk-to-benefit ratio of thromboprophylaxis depends on the performance of the risk assessment model. A linear model, the Padua model, is recommended for medical inpatients in the United States but is not suitable for Chinese inpatients due to differences in race and disease spectrum. Currently, machine learning (ML) methods show advantages in modeling complex data patterns and have been applied to clinical data analysis. This study aimed to build VTE risk assessment ML models among Chinese inpatients and compare the predictive validity of the ML models with that of the Padua model. METHODS: We used 376 patients, including 188 patients with VTE, to build a model and then evaluate the predictive validity of the model in a consecutive clinical dataset from Peking Union Medical College Hospital. Nine widely used ML methods were trained on the model derivation set and then compared with the Padua model. RESULTS: Among the nine ML methods, random forest (RF), boosting-based methods, and logistic regression achieved a higher specificity, Youden index, positive predictive value, and area under the receiver operating characteristic curve than the Padua model on both the test and clinical validation sets. However, their sensitivities were inferior to that of the Padua model. Combined with the receiver operating characteristic curve, RF, as the best performing model, maintained high specificity with relatively better sensitivity and captured VTE patients' patterns more precisely. CONCLUSIONS: Advances in ML technology provide powerful tools for medical data analysis, and choosing models conforming to the disease pattern would achieve good performance. Popular ML models do not surpass the Padua model on all indicators of validity, and the drawback of low sensitivity should be improved upon in the future.

Pulmonary manifestations of Sjögren's syndrome.

BACKGROUND: Primary Sjögren's syndrome (PSS) is associated with various histological patterns of interstitial lung disease. Although chest images and lung function studies showed that lung involvement predominantly occurs in small airways, pathological findings were not consistent with the results of high-resolution CT (HRCT) and lung function tests. OBJECTIVES: To investigate the pathological characteristics of PSS-associated interstitial lung disease (PSS-ILD) and their relationship with HRCT lung function tests. METHODS: Fourteen patients diagnosed as PSS who underwent surgical lung biopsy in Peking Union Medical College Hospital from October 2000 to October 2006 were reviewed. Histopathologic findings, radiologic findings and lung function tests were analyzed. RESULTS: The study included 13 women. The median age was 46 years. Most patients presented with dyspnea and cough. CT scans revealed bilateral ground-glass, consolidative, reticular and nodular opacities and cyst lesions. The histological patterns included nonspecific interstitial pneumonia (NSIP) cellular pattern associated with organizing pneumonia (OP), NSIP mixed pattern associated with OP, noncaseating granulomas, chronic bronchiolitis, follicular bronchiolitis, constrictive bronchiolitis, lymphocytic interstitial pneumonia associated with follicular bronchiolitis, NSIP mixed pattern associated with follicular bronchiolitis, NSIP mixed pattern coexisting with chronic bronchiolitis, OP associated with chronic bronchiolitis, and noncaseating granulomas coexisting with OP. Treatment included prednisone and cyclophosphamide. During the follow-up period (median 38 months), most patients improved or remained stable. The patient with constrictive bronchiolitis died from progression of primary disease. CONCLUSIONS: The histopathologic patterns of PSS-ILD included lung interstitial involvement and small airway involvement or both. Corticosteroid therapy combined with cyclophosphamide was administered with a favorable response in the majority of patients.

Clinical features and prognosis in 21 patients with extrinsic allergic alveolitis.

OBJECTIVE: To assess the spectrum of causes, clinical features, differences between disease phases, and prognosis of extrinsic allergic alveolitis (EAA). METHODS: Patients with EAA diagnosed at Peking Union Medical College Hospital from August 1983 to May 2007 were analyzed retrospectively. Their medical records were examined to gather clinical, laboratorial, radiological, and histopathological data. Patients were divided to three phases (acute, subacute, and chronic) according to clinical presentations. Follow-up data regarding treatment response, subsequent radiological and pulmonary function studies, and clinical outcomes were collected. RESULTS: A total of 21 cases were enrolled. Among them, 11 were subacute, 10 were chronic. The most common exposure was pet birds (6 cases, 28.6%). The primary abnormality of pulmonary function was restriction and/or reduction in diffusing capacity (12 cases, 63.2%). The most common findings on high-resolution computed tomography (HRCT) were ground-glass opacities (13 cases, 68.4%) and centrilobular nodules (8 cases, 42.1%). Airway obstruction in pulmonary function test, emphysema, lung cysts, and fibrosis on HRCT were more frequently seen in chronic than in subacute patients, though the differences were not statistically significant. Bronchoalveolar lavage fluid (BALF) showed lymphocytosis. The total cell count and the percentage of neutrophils were significantly higher in subacute than in chronic patients (P<0.05). Nonnecrotizing granulomas were seen in 8 (47.1%) cases. Improvement or normalization in symptoms, radiography, and pulmonary function test after treatment were seen in all 18 patients with available follow-up data. Five patients recurred. CONCLUSIONS: The characteristic abnormalities of pulmonary function, findings on HRCT, and pathology are essential for all phases of EAA, and the atypical manifestations such as obstruction and fibrosis can also be present frequently, particularly in chronic cases. Differential cell counts of BALF are related to the phase of the disease. The treatment response and prognosis of EAA are good.

[The results of transbronchial needle aspiration in 164 cases with enlarged mediastinal and/or hilar lymph nodes].

OBJECTIVE: To evaluate the role of transbronchial needle aspiration (TBNA) in the diagnosis of patients with enlarged mediastinal and/or hilar lymph node. METHODS: Patients with mediastinal and/or hilar lymphoadenopathy proven by CT scan were eligible for TBNA as reported. All specimens were directly and instantly smeared for pathological examination. RESULTS: From June 1 2004 to December 31 2007, 164 patients were examined: including 80 lung cancers, 69 lung benign diseases, 2 other malignancy tumor, and 13 without definite diagnosis. Total 260 lymph nodes were punctured. TBNA procedures were successfully carried out in 445/463 (96.1%). Sensitivity of TBNA was 82.5% (66/80) in patients who had been proven to suffer from bronchogenic carcinoma. There were 25 patients that diagnosis of lung cancer was pathologically determined by TBNA only. A total of 122 lymph nodes in the 80 lung cancer patients were aspirated by TBNA with a positive rate of 65.6% (80/122). Severe complications were rare except small amount of bleeding at the TBNA site (100/164, 61.0%). From June 1 2006 to December 31 2007, lymph node tissues able to make histology diagnosis were yield in 73.5% (64/87) patients. Through histology pathology, the sensitivities of TBNA were 53.3% (8/15) for sarcoidosis and 78.6% (33/42) for lung cancer. CONCLUSION: TBNA is quite safe and helpful in diagnosis and staging of bronchogenic carcinoma, and in diagnosis of benign lung diseases.

[T-bet gene modified dendritic cells abrogate and reverse the airway inflammation in allergic asthma: experiment with mice].

OBJECTIVE: To explore the feasibility of the therapeutic strategy to use T-bet gene modified dendritic cells (DCs) to reverse the course of asthma. METHODS: (1) Mature DCs were derived from mononuclear cells obtained from femur of BALB/c mouse and divided into 3 groups, T-bet group transfected with recombinant adenovirus Ad-T-bet containing T-bet gene, LacZ group transfected with recombinant adenovirus Ad-LacZ containing LacZ gene, and control group. Seven days later ELISA was used to detect the interferon (IFN)-gamma level in the culture fluid. (2) Airway inflammation abrogating trial. Twenty-four BALB/c mice were sensitized with intraperitoneal injection of ovalbumin (OVA) (on day 1 and 15) to establish asthma models, and then randomly divided into 3 equal groups: T-bet group injected intravenously with T-bet-modified DCs on day 27, LacZ group injected with LacZ-modified DCs, and model control group without intravenous injection. Two days later the model mice began to undergo challenge by inhalation of OVA twice (on day 29 - 31). Eight mice were used as control group treated with PBS. On day 37 all mice were killed, ELISA was used to detect the blood interleukin (IL)-4 and IFN-gamma levels, and microscopy was conducted to observe the airway inflammation. (3) Airway inflammation reversing trial. Another 24 model mice were divided into 3 equal groups as well: re-challenged T-bet group injected intravenously with T-bet-modified DCs on day 27 and 42, re-challenged LacZ group injected intravenously with LacZ-modified DCs on day 27 and 42, and model control group. Since the day 45 OVA inhalation was given once a day for successive 3 days. On day 49 these mice were all killed to undergo the tests as mentioned above. RESULTS: The IFN-gamma level in the culture fluid of the T-bet gene modified DCs was (15.24 +/- 4.75) ng/ml, significantly higher than that of the LacZ gene modified DCs and control DCs [(3.08 +/- 0.61) and (2.35 +/- 0.41) ng/ml respectively, both P < 0.01]. The IFN-gamma in mice blood plasma of T-bet groups in abrogating and reversing trial were (130.2 +/- 10.5) and (145.7 +/- 16.7) pg/ml respectively, both significantly higher than those of the abrogating and reversing trial normal control groups [(25.0 +/- 6.5) and (24.6 +/- 5.9) pg/ml respectively], asthmatic model control groups [(20.7 +/- 4.5) and (16.5 +/- 7.0) pg/ml respectively] and LacZ groups [(17.6 +/- 7.0) and (24.2 +/- 9.0) pg/ml respectively] (all P < 0.01). However, the IL-4 levels in mice blood plasma of T-bet groups were both significantly lower than those of asthmatic model control groups and LacZ groups (all P < 0.01). The airway inflammation of T-bet groups were remarkable milder than those of the model control groups and LacZ groups. CONCLUSION: The asthma management strategy based on T-bet gene modified DCs is feasible with the plausible mechanism that the T-bet gene modified DCs regulate the T cells differentiation and polarization on the antigen presenting level.

[Pathologic study of diffuse pulmonary interstitial fibrosis caused by chronic hypersensitivity pneumonitis].

OBJECTIVE: To study the pathologic characteristics of chronic hypersensitivity pneumonitis, especially the pattern of pulmonary interstitial fibrosis; and to compare the histologic features with those of idiopathic interstitial pneumonitis. METHODS: The HE-stained paraffin sections of 10 cases of chronic hypersensitivity pneumonitis encountered during the period from 2000 to 2008 were retrospectively analyzed. RESULTS: There were altogether 6 males and 4 females, with age of patients ranging from 23 to 59 years (mean=47.2 years). Clinically, the patients presented with chronic cough and shortness of breath for 4 months to 6 years. Histologically, 7 cases showed usual interstitial pneumonitis (UIP)-like fibrosis. Patchy fibrosis was observed under the pleura, adjacent to interlobular septa and around bronchioles. In all of the 7 cases, foci of fibroblastic proliferation, as well as bronchiolar metaplasia of peribronchiolar alveoli and mild bronchiolitis, were noted. Three cases presented with mild honeycomb changes of lung and 3 cases showed non-specific interstitial pneumonitis (NSIP)-like fibrosis, in which the alveolar septa were expanded by fibrous tissue and collagen, with relative preservation of alveolar architecture. Bronchiolitis and lymphocytic infiltrates in alveolar septa were seen. Schaumann bodies were identified in 1 case. In general, patients with chronic hypersensitivity pneumonitis were younger than patients with idiopathic UIP. Computed tomography often showed upper and middle lobar involvement and mosaic attenuation. Compared with idiopathic UIP, the UIP-like fibrosis of chronic hypersensitivity pneumonitis often occurred not only under the pleura and adjacent to interlobular septa, but also around bronchioles and was accompanied by bronchiolar metaplasia. CONCLUSIONS: Chronic hypersensitivity pneumonitis can mimic other types of lung conditions with interstitial fibrosis, especially UIP and NSIP. As a result, some cases of chronic hypersensitivity pneumonitis may be misdiagnosed as such.

[Clinical manifestations and radiological features of hepatopulmonary syndrome.].

OBJECTIVE: To investigate the clinical and radiological features of hepatopulmonary syndrome (HPS). METHODS: Twenty two cases diagnosed as HPS in Peking Union Medical College Hospital from Nov, 1996 to Nov, 2008 were reviewed. Their clinical features and radiological findings were analyzed. RESULTS: The study included 12 males and 10 females with a mean age of (44 +/- 17) years and mean course of (54 +/- 52) months. Abnormalities on chest CT scans were found in 12 cases, which included intrapulmonary artery dilatation in 10 cases, with subpleural small nodular, reticular and patchy opacities; bilateral lower basal segmental artery dilatation in 3 cases; main pulmonary artery dilation in 1 case; right ventricular enlargement in 1 case and bilateral pleural effusion in 1 case. The chest CT was normal in 6 cases. CTPA and pulmonary artery angiogram were performed in 15 cases and artery dilatation was found in 4. Diffuse liver diseases were found in 12 cases by CT scans, 10 of them presented as typical hepatic cirrhosis and 2 cases as liver hepatomegaly, while esophagus varix and(or) fundus gastricus varix were found in 12 cases and splenomegaly in 6 cases, hydroperitonia in 3 cases. CONCLUSION: Clinical manifestations combined with CT features were helpful for the diagnosis of HPS.

[Clinical and radiological features of pulmonary tuberculosis manifested as interstitial lung diseases.].

OBJECTIVE: The purpose of this paper was to investigate the clinical and radiological features of pulmonary tuberculosis presenting as interstitial lung diseases (ILD). METHODS: We analyzed the data of cases suspected of diffuse parenchyma lung diseases at this hospital between October 2003 and October 2007. The diagnosis of active pulmonary tuberculosis was based on epithelioid granuloma or positive acid-fast bacilli in lung biopsy and changes on serial radiographs obtained during treatment. RESULTS: The data of a series of 230 consecutive patients with suspected ILD were retrospectively analyzed. The diagnosis was confirmed by lung biopsy. Twelve patients were confirmed to have pulmonary tuberculosis. There were 5 males and 7 females with a mean age of 38 +/- 11 years (range, 17 - 68). The median course of disease in these patients was 3 months (range, 0.5 - 18 months). Patients with pulmonary tuberculosis presented with fever (11/12), cough (9/12), weight loss (7/12), dyspnea (7/12), lymphadenopathy (4/12), and splenohepatomegaly (2/12). On chest CT scan, ground-glass attenuation was identified in 4, bilateral patchy infiltration in 5, tree-in-bud appearance 1, and centrilobular lesions in 2 of the 12 patients. During the follow-up period (median, 9 month, range from 3 to 12 month), 11 patients improved, but 1 died of diabetic ketoacidosis. CONCLUSION: The diagnosis of pulmonary tuberculosis should be considered in suspected ILD patients presenting with fever, splenohepatomegaly and lymphadenopathy.

[Intravascular lymphomatosis presenting in the lung].

OBJECTIVE: To investigate the clinical, radiographic and pathological characteristics of intravascular lymphomatosis primarily manifested in the lung, without skin and central nervous system involvements. METHODS: A case of T cell intravascular lymphomatosis presenting with fever and multiple pulmonary shadows on chest radiograph was described and 14 similar cases reported in the English literature were reviewed. RESULTS: We described a case of T cell intravascular lymphomatosis, who was a 36 year old man, complained of fever and multiple pulmonary shadows on chest radiograph and admitted to Peking Union Medical College Hospital in march, 2008. Open lung biopsy showed features characteristic of intravascular lymphomatosis. He received CHOP chemotherapy, but died 20 days after diagnosis. Most cases of intravascular lymphomatosis primarily manifested in the lung occurred in older patients, ranging from 36 to 79 years of age (mean 59 years), with a male predominance (M : F = 11 : 4). The chief complaints were dyspnoea (10/15), cough (9/15), fever (5/15) and weight loss (5/15). Pulmonary function tests usually revealed some degree of decreased diffusing capacity. Eight cases had high serum lactate dehydrogenase levels. Chest CT scan showed multiple reticular or/and nodular density. Immunophenotypically, 10 cases were B cell lineage, 3 cases were T cell lineage. Six cases of B cell intravascular lymphomatosis were followed, of whom 4 and were alive, and 2 died of respiratory failure. Three cases of T cell intravascular lymphomatosis showed poor prognosis, both of whom died of respiratory failure. CONCLUSIONS: Intravascular lymphomatosis primarily manifested in the lung is a rare malignant disease. Its pulmonary symptoms were nonspecific, and pulmonary function tests and chest CT scan manifested as those of interstitial pneumonia or pulmonary infection. It can be pathologically diagnosed through transbronchial lung biopsy or surgical lung biopsy.

[Ten cases analysis of small vessel vasculitis with myocardial involvement].

OBJECTIVE: This study was designed to investigate the clinical characteristics of systemic vasculitis with cardiac involvement. METHODS: Clinical information of 10 patients with systemic vasculitis complicated by myocardial vasculitis, selected from 181 small vessel vasculitis patients and 114 systemic vasculitis patients were analyzed. RESULTS: The clinical manifestations were varied significantly dependent on the etiology of small vessel vasculitis. It is usually difficult to make the diagnosis because of the insidious onset, varied etiology and the undifferentiated manifestations of heart involvement. Echocardiography is commonly used in detecting and monitoring cardiac involvement. Glucocorticoid therapy can improve left ventricular systolic function dramatically when used properly. CONCLUSIONS: The cardiac involvement of systemic vasculitis is quite rare. Dyspnea of various degrees and left ventricular systolic dysfunction are the most common clinical findings. The earlier the establishment of diagnosis and institution of appropriate treatment, the better the prognosis.