Updated severity and prognosis score of pulmonary alveolar proteinosis: A multi-center cohort study in China.

Background: The high-resolution computed tomography (HRCT) score is an important component of the severity and prognosis score of pulmonary alveolar proteinosis (SPSP). However, the HRCT score in SPSP only considers the extent of opacity, which is insufficient. Methods: We retrospectively evaluated HRCT scores for 231 patients with autoimmune pulmonary alveolar proteinosis (APAP) from three centers of the China Alliance for Rare Diseases. The SPSPII was created based on the overall density and extent, incorporating the SPSP. The severity of APAP patients was assessed using disease severity scores (DSS), SPSP, and SPSPII to determine the strengths and weaknesses of the different assessment methods. We then prospectively applied the SPSPII to patients before treatment, and the curative effect was assessed after 3 months. Results: The HRCT overall density and extent scores in our retrospective analysis were higher than the extent scores in all patients and every original extent score severity group, as well as higher related to arterial partial oxygen pressure (PaO2) than extent scores. The mild patients accounted for 61.9% based on DSS 1-2, 20.3% based on SPSP 1-3, and 20.8% based on SPSPII 1-3. Based on SPSP or SPSPII, the number of severe patients deteriorating was higher in the mild and moderate groups. When applied prospectively, arterial PaO2 differed between any two SPSPII severity groups. The alveolar-arterial gradient in PaO2 (P[A-a]O2), % predicted carbon monoxide diffusing capacity of the lung (DLCO), and HRCT score were higher in the severe group than in the mild and moderate groups. After diagnosis, mild patients received symptomatic treatment, moderate patients received pure whole lung lavage (WLL) or granulocyte-macrophage colony-stimulating factor (GM-CSF) therapy, and severe patients received WLL and GM-CSF therapy. Importantly, the SPSPII in mild and severe groups were lower than baseline after 3 months. Conclusion: The HRCT density and extent scores of patients with APAP were better than the extent score. The SPSPII score system based on smoking status, symptoms, PaO2, predicted DLCO, and overall HRCT score was better than DSS and SPSP for assessing the severity and efficacy and predicting the prognosis. Trial registration: ClinicalTrial.gov, identifier: NCT04516577.

Cushing's disease with pulmonary Cryptococcus neoformans infection in a single center in Beijing, China: A retrospective study and literature review.

BACKGROUND: Patients with Cushing's disease (CD) with hypercortisolism have an increased risk of opportunistic infection. However, most CD patients exposed to infections are diagnostic latency, leading to a poor prognosis. METHODS: Six patients in our hospital and an additional six patients in the literature were included in this study. Clinical information of CD patients with pulmonary Cryptococcus neoformans are reviewed. RESULTS: The average baseline total cortisol and ACTH in serum at 8 am of all the patients was 44.85 µg/dL (normal range 4.0-22.3 µg/dL) and 200.3 pg/mL (normal range 0-46 pg/mL), respectively. Lymphopenia was found in 2 out of 6 patients in our hospital. The pulmonary radiologic findings included nodules (4/12), masses with or without a cavity (5/12), infiltration (5/12), and consolidation (4/12). The diagnosis of C.neoformans was established by lung pathology results (7/12), microorganism culture (3/12), and serum cryptococcal polysaccharide antigen (4/12). Lung lobectomy was performed in two patients who had a nodule in one lung lobe. Antifungal drugs were administered, including amphotericin-B (7/12), fluconazole (4/12), flucytosine (2/12) and liposomal amphotericin (1/12). Additional therapies for CD included trans-sphenoidal pituitary adenoma surgery (9/12), adrenalectomy (1/12) and ketoconazole (2/12). Seven patients survived, and five patients died. CONCLUSION: Pulmonary C.neoformans is an uncommon but fatal opportunistic infection in CD patients. Pulmonary nodules or masses should be aggressively investigated to exclude the C.neoformans among CD patients. The infiltration lesions in chest CT scan and lymphopenia are associated with poor prognosis.

Diagnosis and treatment of community-acquired pneumonia in adults: 2016 clinical practice guidelines by the Chinese Thoracic Society, Chinese Medical Association.

Community-acquired pneumonia (CAP) in adults is an infectious disease with high morbidity in China and the rest of the world. With the changing pattern in the etiological profile of CAP and advances in medical techniques in diagnosis and treatment over time, Chinese Thoracic Society of Chinese Medical Association updated its CAP guideline in 2016 to address the standard management of CAP in Chinese adults. Extensive and comprehensive literature search was made to collect the data and evidence for experts to review and evaluate the level of evidence. Corresponding recommendations are provided appropriately based on the level of evidence. This updated guideline covers comprehensive topics on CAP, including aetiology, antimicrobial resistance profile, diagnosis, empirical and targeted treatments, adjunctive and supportive therapies, as well as prophylaxis. The recommendations may help clinicians manage CAP patients more effectively and efficiently. CAP in pediatric patients and immunocompromised adults is beyond the scope of this guideline. This guideline is only applicable for the immunocompetent CAP patients aged 18 years and older. The recommendations on selection of antimicrobial agents and the dosing regimens are not mandatory. The clinicians are recommended to prescribe and adjust antimicrobial therapies primarily based on their local etiological profile and results of susceptibility testing, with reference to this guideline.

Characteristics of serum lipid metabolism in patients with autoimmune pulmonary alveolar proteinosis.

OBJECTIVE: To study the serum lipid panels in consecutive autoimmune pulmonary alveolar proteinosis(APAP)patients and analyze their relationship with anti-granulocyte macrophage-colony stimulating factor(GM-CSF)antibody and other markers. METHODS: Thirty-two non-diabetic APAP patients were enrolled in the study. Serum lipids of these patients and 100 healthy volunteers were tested after an overnight fasting. Anti-GM-CSF antibody levels were measured with enzyme-linked immunosorbent assay. The correlation of serum lipids with lactate dehydrogenase,carcinoembryonic antigen,pulmonary function,and artery blood gas parameters were analyzed. RESULTS: Total cholesterol and low-density lipoprotein cholesterol levels [(5.54±0.99)and(3.73±0.83)mmol/L respectively] were significantly higher in APAP patients than in healthy volunteers [(5.05±0.97)and(3.17±0.89)mmol/L respectively](all P<0.05). High-density lipoprotein cholesterol(HDL-C)level of the APAP group [(1.10±0.18)mmol/L ]was significantly lower than that of the healthy group(P<0.05). Low-density lipoprotein/HDL and total cholesterol/HDL ratios in the APAP group(3.47±0.90 and 5.14±1.12 respectively)were significantly higher than those in the healthy group[(2.63±0.87)and(4.18±1.12)](all P<0.05). There was no significant difference in triglyceride level between the two groups(P>0.05). HDL-C level was negatively correlated with alveolar-arterial oxygen pressure difference(r=-0.436,P<0.05)and positively correlated with arterial oxygen saturation(r=0.459,P<0.05). None of the lipid markers correlated with serum anti-GM-CSF antibody levels(all P>0.05). CONCLUSIONS: APAP patients were likely to suffer from disturbed lipid metabolism,which was correlated with disease severity to some degree. Lipid markers deserved more attention in the management of APAP patients.

[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 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.

[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.

[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.

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.

[Combined pulmonary fibrosis and emphysema syndrome].

OBJECTIVE: To retrospectively analyze clinical presentations, pulmonary function parameters and radiological appearance in patients with combined pulmonary fibrosis and emphysema (CPFE) syndrome. METHODS: We reviewed the clinical features, imaging, and lung function indices from 8 patients diagnosed with CPFE according to the findings by high resolution computerized tomography (HRCT) since 2006 to 2009 at Peking Union Medical College Hospital. RESULTS: All patients were male, aged 65 (59 - 75) years, and 7 of them were smokers. Dyspnea on exertion was presented in 7 patients. Basal crackles were heard in 6 patients and finger clubbing was observed in 4 patients. Pulmonary function demonstrated that forced expiratory volume in one second/forced vital capacity (FEV1/FVC) was slightly lower with a median of 76% (range 60% to 86%), forced vital capacity (FVC) was 73% (51% - 92%), and total lung capacity (TLC) was 80% (59% - 114%). However, carbon monoxide diffusion capacity (D(L)CO) was significantly impaired (44%, 16% - 65%). HRCT findings included emphysema predominantly at the upper zone, while reticular opacities, honeycombing and traction bronchiectasis were in the lower lobes. Pulmonary hypertension was seen in 6 patients. CONCLUSIONS: The co-existence of lower lung fibrosis and upper lung emphysema was found in some smokers. Patients with CPFE syndrome can present with a normal or nearly normal lung volume but a remarkable impairment in gas exchange. A high prevalence of pulmonary hypertension is seen in CPFE syndrome. Further studies to elucidate the pathogenesis and to explore the treatment and prognosis are warranted.

[Clinical analysis of four cases of primary ciliary dyskinesia and literature review].

OBJECTIVE: To study the clinical characteristics and diagnosis of primary ciliary dyskinesia (PCD). METHODS: Four cases diagnosed as PCD by cilia electron microscopy examination from Jan. 2007 to Aug. 2009 in this hospital were retrospectively analyzed, and the related literature was reviewed. RESULTS: In the 4 patients, there were 1 male and 3 females. The age at disease onset was 0 - 10 years, and the age at diagnosis was 15 - 53 years. The most common symptoms were productive cough (4/4), sinusitis (3/4), and shortness of breath (3/4). Other manifestations included situs inversus (2/4), infertility (1/4), and tympanitis (1/4). Hypoxemia was found in 3 cases. Obstructive ventilatory impairment accompanied with diffusion dysfunction was observed in 2 cases, while the pulmonary function tests were normal in the other 2 cases. All 4 cases received chest CT scan, and bronchiectasis was present in all of them. Bilateral diffuse micronodules and patchy infiltrates were found in 3 and 2 cases respectively. Electron microscopic examination of the endobronchial biopsy specimen showed lack of dynein arms in 4, lack of muco-cilia in 2, and abnormal arrangement of microtubules in 2 cases. CONCLUSIONS: The Kartagener syndrome is relatively easy to be diagnosed, because it is characterized by the triad of sinusitis, bronchiectasis and situs inversus. However, PCD without situs inversus often goes unrecognized. PCD should be considered in patients with childhood onset disease, bronchiectasis, centrilobular micronodules or tree-in-bud signs in CT scan. Examination of the ciliary ultrastructure is essential to the confirmation of the diagnosis.

[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.

[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.

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 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.

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.

[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.

[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.