[Psychometric Evaluation of the Union Physio-Psycho-Social Assessment Questionnaire].

Objective To validate the Union Physio-Psycho-Social Assessment Questionnaire(UPPSAQ-70)and test its validity and reliability.Methods From April,2013 to July,2018,patients were asked to finish the computer evaluation of UPPSAQ-70 and Symptom Checklist 90(SCL-90)in Peking Union Medical College Hospital(PUMCH).Confirmatory factor analysis(CFA)was conducted on the SPSS 17.0,and the number of fixed factors was 8 factors and 3 factors.Amos 23.0 was used to verify the original 8-factor model,8-factor revision model,3-factor model,3-factor revision model,and single-factor model.Each factor of SCL-90 was used as the calibration standard to calculate the correlation coefficient between factors.The retest reliability was tested by the outpatients in PUMCH in July,2018.Results Exploratory factor analysis indicated that the 8-factor revised model included:depression,anxiety and fatigue,sleep,physical discomfort,sexual function,happiness and satisfaction,hypochondria,and social anxiety.The 3 factors revised model included that:psychological,physiological and social dimension.Confirmatory factor analysis indicated that the 8-factor modified model was superior to the 3-factor model and the single-factor model: χ 2=10 410.4,df=1862,RMSEA=0.07,CFI=0.753,and NFI=0.715.With SCL-90 as the standard criteria,except the low correlation coefficient between emotional scale and depression(r=0.600)and anxiety(r=0.520),the correlation coefficients of other symptoms were below 0.5.The chronbach's α between each factor and total score of UPPSAQ-70 was between 0.823 and 0.904,and the Chronbach's α coefficient of the whole scale was between 0.954 and 0.956 after each item was deleted.The retest reliability of the scale of 32 participants Chronbach's α was 0.847.Each item of the scale measured between one week was significantly correlated(P<0.05). Conclusion UPPSAQ-70 is a good scale for evaluating overall health status and is especially feasible in general hospitals.

[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 analysis of 123 cases of chylous effusion].

OBJECTIVE: To analyze the clinical characteristics of chylous effusion and boost its diagnostic and therapeutic level. METHODS: A retrospective analysis was conducted for 123 cases of chylous effusion at our hospital between January 1990 and December 2009. RESULTS: The main clinical manifestations of chylous effusion included dyspnea (55.3%), edema (26.8%), abdominal distention (22.8%) and loss of weight (17.1%). Hypoalbuminemia was common (45.5%) and it was even more so in patients with idiopathic lymphopathies or of unknown causes (95.2%). The positive rates of identifying chylous effusion by Sudan III test, high triglyceride levels (> 1.25 mmol/L), lymphangiography or lymphangioscintigraphy were 89.1%, 80.6%, 70.6% and 89.5% respectively. Its common etiologies included injury (14.6%), malignancy (9.0%), infections (7.3%), lymphatic disorders or idiopathic lymphopathies (40%), drug-associated (2.4%) or associated with underlying disorders (16.3%). Efficacy was achieved in 63.6% of the patients and 21.8% died. CONCLUSIONS: Chylous effusion is a special type of serous effusion with multiple causes. Its clinical manifestations are often nonspecific. But malnutrition is common. Its causes are identified after a systematic evaluation. Treatment modalities include dietary modification, management of underlying causes and surgical approaches.

[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.

The St. George's Respiratory Questionnaire in lymphangioleiomyomatosis.

OBJECTIVE: To examine the correlation between the health-related quality of life measured by the St. George's Respiratory Questionnaire (SGRQ) and the commonly used physiological measures in lymphangioleiomyomatosis (LAM). METHODS: This study retrospectively analyzed the SGRQ scores and other measures (the Borg scale of breathlessness at rest, 6-minute walking distance, blood oxygen levels, and pulmonary function) of patients diagnosed and confirmed with LAM. Altogether 38 patients between June 2007 and November 2009 were included. RESULTS: The mean values of the SGRQ three components (symptoms, activity, and impacts) and total scores in the LAM patients were 46.95 +/- 28.90, 58.47 +/- 25.41, 47.89 +/- 29.66, and 51.11 +/- 26.35, respectively. The SGRQ total or component scores were correlated well with the Borg scale of breathlessness, 6-minute walking distance, partial pressure of oxygen in arterial blood, spirometry and diffusion capacity of lung. There were poor correlations between SGRQ score and residual volume or total lung capacity. In our preliminary observation, sirolimus improved the SGRQ total and three component scores and the Borg scale of breathlessness significantly after 101-200 days of treatment (n = 6). CONCLUSIONS: The SGRQ score in LAM is correlated well with physiological measures (Borg scale of breathlessness, 6-minute walking distance, blood oxygen levels, and pulmonary function tests). The SGRQ could therefore be recommended in baseline and follow-up evaluation of patients with LAM. Treatment with sirolimus, an inhibitor of mammalian target of rapamycin, may improve the quality of life and patient's perception of breathlessness in LAM.

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.

[Diagnostic specificity of dynamic assessment and monitoring of spirometry in asthma].

OBJECTIVE: To evaluate the diagnostic specificity of dynamic assessment and monitoring using a portable spirometer in diagnosis and differential diagnosis of asthma. METHODS: We retrospectively reviewed the results of dynamic monitoring of spirometry in 145 symptomatic patients with physician-diagnosed asthma. Flow-volume curve and simultaneous symptoms and mood were measured in a fixed-time thrice-daily assessment schedule for 2 weeks. Patients were allowed to make additional measurements in case of symptom exacerbations. RESULTS: The clinical data of 51 males and 94 females with a mean age of (39.1 +/- 13.0) years (ranged from 10 to 65 years) were analyzed. Duration of asthma before study was (6.7 +/- 9.9) years. Of 145 patients with physician-diagnosed asthma, 126 (87%) could be conclusively confirmed for a diagnosis of asthma. Asthma was misdiagnosed in 14 patients (9.7%). Overdiagnosis of asthma was observed in 5 patients (3.4%). CONCLUSION: Dynamic assessment and monitoring using a portable spirometer by revealing variability and reversibility of airway obstruction may provide an additional tool for diagnosis and differential diagnosis of asthma.

[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 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.

Fearful imagery induces hyperventilation and dyspnea in medically unexplained dyspnea.

BACKGROUND: Medically unexplained dyspnea refers to a condition characterized by a sensation of dyspnea and is typically applied to patients presenting with anxiety and hyperventilation without underlying cardiopulmonary pathology. We were interested to know how anxiety triggers hyperventilation and elicits subjective symptoms in those patients. Using an imagery paradigm, we investigated the role of fearful imagery in provoking hyperventilation and in eliciting symptoms, specifically dyspnea. METHODS: Forty patients with medically unexplained dyspnea and 40 normal subjects matched for age and gender were exposed to scripts and asked to imagine both fearful and restful scenarios, while end-tidal PCO(2) (PetCO(2)) and breathing frequency were recorded and subjective symptoms evaluated. The subject who had PetCO(2) falling more than 5 mmHg from baseline and persisting at this low level for more than 15 seconds in the imagination was regarded as a hyperventilation responder. RESULTS: In patients with medically unexplained dyspnea, imagination of fearful scenarios, being blocked in an elevator in particular, induced anxious feelings, and provoked a significant fall in PetCO(2) (P < 0.05). Breathing frequency tended to increase. Eighteen out of 40 patients were identified as hyperventilation responders compared to 5 out of 40 normal subjects (P < 0.01). The patients reported symptoms of dyspnea, palpitation or fast heart beat in the same fearful script imagery. Additionally, PetCO(2) fall was significantly correlated with the intensity of dyspnea and palpitation experienced during the mental imagery on one hand, and with anxiety symptoms on the other. CONCLUSIONS: Fearful imagery provokes hyperventilation and induces subjective symptoms of dyspnea and palpitation in patients with medically unexplained dyspnea.

[Clinicopathologic study of Churg-Strauss syndrome].

OBJECTIVE: To study the clinical and pathologic features of Churg-Strauss syndrome (CCS). METHODS: Three cases of Churg-Strauss syndrome, including 1 autopsy case and 2 cases with open thoracoscopic lung biopsy, were retrospectively reviewed. All the tissue samples were formalin-fixed, paraffin-embedded and stained with hematoxylin and eosin. RESULTS: The first patient was a 68-year-old man who had history of asthma for 4 years, with recent exacerbation and chest pain for 2 weeks. Patient died 1 day after admission due to myocarditis and myocardial infarction. He did not have peripheral eosinophilia, skin or paranasal sinus pathology. CSS represented an incidental autopsy finding and he had never been treated with corticosteroid before. The other 2 patients were a 58-year-old male and a 12-year-old female, respectively. Both had history of asthma, peripheral eosinophilia and lung consolidations on computed tomographic examination. Pathologically, all cases showed vasculitis, perivascular allergic-type granulomas, eosinophilic pneumonia and asthmatic bronchitis. CONCLUSIONS: Thorough understanding of the clinical and pathologic criteria is essential for arriving at a correct diagnosis of CSS. Although some patients may present with atypical symptoms, lung biopsies often reveal the classic histologic findings which include vasculitis and perivascular allergic granuloma formation.

[Clinicopathologic study of primary marginal zone B-cell lymphoma of MALT type and lymphoid hyperplasia of lung].

OBJECTIVE: To study the clinicopathologic features, immunohistochemical findings and immunoglobulin heavy chain (IgH) gene rearrangement results of primary pulmonary mucosa-associated lymphoid tissue lymphoma (MALToma) and reactive lymphoid hyperplasia. METHODS: Twenty cases, included 13 cases of pulmonary MALToma and 7 cases of pulmonary lymphoid hyperplasia, encountered during the period from 1989 to 2007, were retrospectively analyzed. The samples were paraffin-embedded and stained with hematoxylin and eosin. Immunohistochemical study and semi-nested polymerase chain reaction for IgH gene rearrangement were performed. RESULTS: The 13 cases of primary pulmonary MALToma were composed of a spectrum of lymphoid cells, including lymphocyte-like cells, centrocyte-like cells and mononuclear B cells with plasmacytoid differentiation. They often had diffuse or marginal zone growth patterns. Lymphoid follicles with neoplastic colonization were apparent. The lymphoma cells spread along alveolar septa and bronchovascular bundles. Vascular invasion was noted in 9 cases, pleura involvement in 6 cases and nodal involvement in 2 cases. Lymphoepithelial lesions (LEL) were identified in 9 cases of pulmonary MALToma. Immunohistochemically, the lymphocytes in LEL were CD20-positive and CD3-negative. On the other hand, LEL was also present in 2 of the 7 cases of lymphoid hyperplasia studied, with a mixture of CD20-positive B cells and CD3-negative T cells. Eight of the 9 cases of primary pulmonary MALToma were positive for IgH gene rearrangement, while all of the 7 cases of lymphoid hyperplasia were negative. CONCLUSIONS: Histologically, the cell population of primary pulmonary MALToma is similar to that of extranodal MALToma occurring in other organs. LEL, though commonly observed in pulmonary MALToma, are not specific and can also be seen in cases of reactive lymphoid hyperplasia. The immunophenotype of intraepithelial lymphocytes in pulmonary MALToma and reactive lymphoid hyperplasia is different. The presence of a monotonous population of CD20-positive intraepithelial lymphocytes supports a diagnosis of MALToma. IgH gene rearrangement study is also useful in differentiating both entities.

[Primary pulmonary lymphoma: analysis of 18 cases].

OBJECTIVE: To study the clinical characteristics, pathology, diagnosis and treatment of primary pulmonary lymphoma. METHODS: Eighteen cases of primary pulmonary lymphoma diagnosed from Jan 1989 to Feb 2007 were retrospectively analyzed. RESULTS: There were 6 males and 12 females, with a median age of 47.5 years (17-71 years). Fifteen cases were diagnosed by surgical lung biopsy; 1 by percutaneous needle lung biopsy (1/6), 1 by percutaneous needle lung biopsy and bronchoscopic examination at the same time, the other 1 by bronchoscopic examination (1/10). Histological diagnosis showed that 2 cases were Hodgkin lymphoma, 9 mucosa-associated lymphoid tissue lymphoma, 1 follicular lymphoma, 2 diffuse large B cell lymphoma 2 anaplastic large cell lymphoma, 2 non-Hodgkin lymphoma whichcould not be classified because the slides were from other hospitals. The most common symptoms were cough (9/18) and fever (6/18). ESR elevation was common (10/12). CT features included solitary or multiple nodules (14/18), patchy opacities (11/18), consolidations (5/18), pleural effusions (5/18), atelectasis (5/18), and cavities (1/18). Misdiagnosis was found in 11 patients. Treatment modalities included surgical resection, radiotherapy and chemotherapy. Median follow-up time was 11 months (10 d to 205 mon). Thirteen patients were still alive, 4 patients were lost, and 1 patient died. The prognosis was associated with the level of [25.1 x 10(9)/L(18.1 - 39. -1) x 10(9)/L in poor prognosis group, 6.7 x 10(9)/L (5.48 - 8.41) x 10(9)/L in good prognosis group, u = 0.000, P <0.05] leukocytosis (3/3 vs 1/10, P <0.05). CONCLUSIONS: The clinical manifestations of primary pulmonary lymphoma are nonspecific. Misdiagnosis is common. Surgical lung biopsy is necessary for early diagnosis.

[Predictive value of single and combined indexes in the diagnosis of sarcoidosis].

OBJECTIVE: To evaluate the predictive value of single and combined indexes in the diagnosis of sarcoidosis. METHODS: Eighty-eight patients suspected of having sarcoidosis were retrospectively investigated. The diagnostic predictive value of lymphocyte percentage and CD4/CD8 ratio in bronchoalveolar lavage (BAL), angiotensin converting enzyme (ACE) and their combination was evaluated by ROC curve method and Bayes' rule. RESULTS: Final diagnosis of sarcoidosis by biopsy was available in 59 patients (67%), 16 were males (27%), 43 were females (73%), with a mean age of (48 +/- 10) years. The diagnosis of non-sarcoidosis was available in 29 patients (33%), 12 were males (41%), 17 were females (59%), with a mean age of (49 +/- 13) years. The ROC curve area of lymphocyte percentage, CD4/CD8 ratio and ACE were 0.64, 0.74 and 0.69 respectively; CD4/CD8 ratio had the best diagnostic efficiency. The optimized cut-offs of the three single indexes, lymphocyte percentage > or = 30%, CD4/CD8 ratio > or = 4.0 and ACE > or = 40 U/L, were determined by the tangential points and coordinates of the ROC curve. The positive predictive values were 76.7%, 80.4% and 76.8% respectively; CD4/CD8 ratio had the best predictive value. The ROC curve area of the combined CD4/CD8 ratio and ACE was 0.81, and the combination of lymphocyte percentage, CD4/CD8 ratio and ACE was 0.78. They were both higher than those of the respective single indexes. The combination of CD4/CD8 ratio and ACE had the best diagnostic efficiency. On the other hand, it had the best positive predictive value of 90.5% in all the diagnostic indexes. CONCLUSION: CD4/CD8 ratio in BAL is still a useful index in the auxiliary diagnosis of sarcoidosis. The combination of CD4/CD8 ratio and ACE can improve the diagnostic efficiency and predictive value of sarcoidosis.

[The diagnostic value of transbronchial lung biopsy in diffuse parenchymal lung diseases].

OBJECTIVE: This study was to evaluate the efficacy and limitations of transbronchial lung biopsy (TBLB) in the diagnosis of diffuse parenchymal lung diseases (DPLD). METHODS: TBLB was performed in 416 patients with diffuse lung diseases from January 2001 to October 2006 in Peking Union Medical College Hospital. The results of clinical data and pathologic diagnosis were retrospectively analyzed. RESULTS: Confirmed diagnosis by TBLB was obtained in 124 patients, the total positive diagnostic rate was 29.8%. The diseases included pulmonary sarcoidosis (52/124, 41.9%), bronchiolitis obliterans organizing pneumonia/organizing pneumonia (BOOP/OP) (28/124, 22.6%), pulmonary alveolar proteinosis (19/124, 15.3%), lung cancer (12/124, 9.7%), pulmonary vasculitis (5/124, 4.0%), pulmonary tuberculosis (3/124, 2.4%), and pneumocystis carinii pneumonia, lung fungal infection, lymphangiomyomatosis, and pulmonary amyloidosis (each 1/124, 0.8%). Open lung or thoracoscopic biopsy was performed in 104 cases in whom the diagnosis was undetermined by TBLB. Confirmed diagnosis was obtained in 109 patients, including nonspecific interstitial pneumonia (37/104, 37.7%), usual interstitial pneumonia (18/104, 18.4%), pulmonary sarcoidosis (11/104, 11.2%), BOOP/OP (6/104, 6.1%), and lung cancer (5/104, 5.1%). CONCLUSIONS: Pathologic diagnosis can be obtained by TBLB in about 30% of the cases with DPLD, and therefore it should be considered to be a routine diagnostic procedure before open lung or thoracoscopic biopsy.

[Clinico-pathological manifestations in interstitial lung diseases associated with polymyositis-dermatomyositis].

OBJECTIVE: Polymyositis-dermatomyositis has been associated with various histological patterns of interstitial lung disease (ILD). This study was to investigate the clinico-pathologic features of interstitial lung diseases associated with polymyositis-dermatomyositis. METHODS: We retrospectively identified 26 patients with polymyositis-dermatomyositis associated ILD who underwent lung biopsies (6 autopsies, 5 surgical lung biopsies and 15 percutaneous lung biopsies) at our hospital during a 27-year period from January 1980 through October 2007. Histopathologic findings were analyzed and correlated with radiological features and outcome. RESULTS: Median age was 48 years (range, 19 to 65 years), and 16 patients were women. Chest X-ray imaging revealed bilateral infiltrates including ground-glass attenuation, patchy infiltration, and reticular opacities. The major histopathologic patterns included diffuse alveolar damage (DAD) (n = 5), lymphocytic interstitial pneumonia (n = 2), nonspecific interstitial pneumonia (NSIP) (cellular pattern, n = 6; mixed pattern, n = 8), organizing pneumonia (n = 4), usual interstitial pneumonia (UIP) (n = 1). Treatment commonly included prednisone with immunosuppressive agent. During the follow-up period (median, 15 month,range from 6 to 108 month), 18 patients improved or remained stable. Eight patients died, including 5 deaths from DAD, 2 from NSIP mixed pattern, 1 from UIP. CONCLUSIONS: A variety of histological patterns can be seen in patients with polymyositis-dermatomyositis-associated ILD. Those with DAD tended to have poor prognosis.

[Acute exacerbation of usual interstitial pneumonia and nonspecific interstitial pneumonia: analysis of 6 cases].

OBJECTIVE: Acute exacerbation of diffuse parenchymal lung disease (DPLD) is a condition in which patients with usual interstitial pneumonia (UIP), and other forms of interstitial lung disease, develops rapid respiratory failure, accompanied by extensive radiological infiltrates, and had no evidence of infection. The pathologic features of this condition are usually diffuse alveolar damage (DAD) and the outcome is poor. Our study was to define the clinicopathologic features and outcome of acute exacerbation in 3 patients with UIP and 3 with nonspecific interstitial pneumonia (NSIP). METHOD: The clinical data of the 6 patients from April 1999 to Jun 2007 were analyzed retrospectively. RESULTS: In the 6 patients, 2 were males. The median age was 51 yrs (29 -57 yrs). Three case had UIP [1 UIP/idiopathic pulmonary fibrosis (IPF), 1 UIP/dermatomyositis (DM), 1 UIP/UCTD], 2 had NSIP (1 idiopathic NSIP, 1 NSIP/DM), and 1 was diagnosed as DAD (the basic pathology was NSIP/DM) by autopsy. Four of the patients underwent video-assisted thoracoscopic surgery (VATS) for diagnosis, and 1 underwent CT-guided transthoracic needle biopsy. Two of them underwent surgical lung biopsy 1 week before acute exacerbation. Five cases had fever. Computed tomography data were available in all cases and showed the presence of extensive bilateral ground-glass opacities (5/6), sometimes accompanied by focal consolidation (2/6), superimposed on underlying fibrosis. The median oxygenation index was 200 (quartile range: 158-237) mm Hg (1 mm Hg = 0.133 kPa). Five patients were treated with corticosteroids, and in some combined with cyclophosphamide or intravenous immunoglobulin. Two patients survived the acute episode and were discharged from hospital. All 3 patients using mechanical ventilation died. CONCLUSIONS: Both UIP and NSIP can develop acute exacerbations. The trigger of acute exacerbation was unclear, but in some cases it maybe related to VATS. The clinical features include rapid respiratory failure, accompanied by extensive radiological infiltrates. The fatality is high. Corticosteroids or intravenous immunoglobulin may be helpful in the treatment of the condition.

[Clinicopathological manifestations of drug-induced lung injury].

OBJECTIVE: To investigate the clinicopathological features of drug-induced lung injury. METHOD: The clinical, radiological and pathological presentations of drug-induced lung injury diagnosed from January 2003 to October 2005 in Peking Union Medical College Hospital were reviewed. RESULTS: There were 7 cases of drug-induced lung injury, 3 caused by amiodarone, and the other 4 caused by interferon-alpha, atorvastatin, carbamazepine, and propylthiouracil respectively. The clinical manifestations included cough, fever, worsening dyspnea and inspiratory crackles. Patchy infiltration, ground-glass attenuation with thickening of bronchovascular markings were the main findings in computed tomography. The most common pathological manifestation was cellular interstitial pneumonia associated with intra-alveolar macrophages with fine vacuolation in the cytoplasm, and type II pneumocyte hyperplasia. The organizing pneumonia pattern was observed in 2 patients. Diffuse alveolar hemorrhage was present in 2 cases. CONCLUSIONS: Drug-induced pulmonary toxicity can be difficult to diagnose. The diagnosis of pulmonary drug toxicity should be considered in patients with a history of drug therapy and new onset or progressive respiratory complaints.