Independent Clinical Factors Relevant to Prognosis of Patients with Idiopathic Pulmonary Fibrosis.

BACKGROUND The prognosis of idiopathic pulmonary fibrosis (IPF) is the worst among all interstitial lung diseases, and is related to the disease itself. Comorbidities or complications can worsen IPF. We assessed the effect of comorbidities on the survival of IPF patients. A retrospective review of patients with IPF was completed. MATERIAL AND METHODS Information on demographic features, clinical examination, and comorbidities at baseline were obtained. Then, median, 1-year, and 5-year survival was calculated. A total of 380 patients with IPF admitted to Beijing Chao-Yang Hospital from 1 April 2002 to 31 March 2015 were followed up until December 2016. RESULTS Of these 380 patients, 71.9% died during the study period. Median survival was 2.25 years and overall 5-year survival was 28.5%. Also, 86.3% of patients were males. A total of 248 cases underwent lung function tests, and 178 patients underwent bronchoalveolar lavage (BAL). Multivariate analyses showed that forced expiratory volume in 1 second/forced vital capacity (FVC), diffusing capacity of the lungs for carbon monoxide percent predicted, FVC% predicted, the number of macrophages, neutrophils, and lymphocytes in BAL fluid, pulmonary hypertension, hypoxemia, and hydropower disorder were independent prognostic indicators of IPF, GAP gender (G), age (A), and 2 pulmonary physiological parameters (P) model can help to predict prognosis of IPF. CONCLUSIONS Spirometry, GAP model, and BAL are helpful to forecast the prognosis of IPF. IPF patients also suffering from pulmonary arterial hypertension, hypoxemia, and hydropower disorder have a poor prognosis.

Improved heart hemodynamics after draining large-volume pleural effusion: a prospective cohort study.

BACKGROUND: Pleural effusion (PE) drainage can relieve the symptoms of dyspnea; however, details of the resulting hemodynamic changes remain undefined. METHODS: Subjects older than 12 years with massive PE requiring pleural drainage were included in this study. Hemodynamic parameters were collected using transthoracic echocardiography at pre-drainage, immediately post-drainage, and 24 h after drainage. RESULTS: We enrolled 47subjects in this prospective study from June 9, 2015 to September 18, 2016 in Beijing Chaoyang Hospital and 28 subjects were analyzed finally. Draining large-volume PE led to a progressive increase in left ventricular end-diastolic volume index, left atrial volume index, right ventricular area, right atrial area, left ventricular ejection fraction, stroke volume, and tricuspid annular plane systolic excursion, both immediately (P < 0.05) and 24 h after drainage (P < 0.05). The cardiac diastolic measurement ratios of early-transmitral flow velocity to diastolic mitral annular velocity and myocardial performance index decreased significantly following drainage (P < 0.05). More parameters were influenced by left-sided PE drainage. The correlation between effusion volume and changes in echocardiographic measurements was not statistically significant. CONCLUSIONS: Improved preload, and systolic and diastolic function is pivotal for hemodynamic change after draining large PE volumes. Subjects experienced improved cardiac hemodynamics following PE drainage, underlining the beneficial therapeutic and subjective effects.

Emergent severe acute respiratory distress syndrome caused by adenovirus type 55 in immunocompetent adults in 2013: a prospective observational study.

INTRODUCTION: Since 2008, severe cases of emerging human adenovirus type 55 (HAdV-55) in immunocompetent adults have been reported sporadically in China. The clinical features and outcomes of the most critically ill patients with severe acute respiratory distress syndrome (ARDS) caused by HAdV-55 requiring invasive mechanical ventilation (IMV) and/or extracorporeal membrane oxygenation (ECMO) are lacking. METHODS: We conducted a prospective, single-center observational study of pneumonia with ARDS in immunocompetent adults admitted to our respiratory ICU. We prospectively collected and analyzed clinical, laboratory, radiological characteristics, sequential tests of viral load in respiratory tract and blood, treatments and outcomes. RESULTS: The results for a total of five consecutive patients with severe ARDS with confirmed HAdV-55 infection were included. All five patients were immunocompetent young men with a median age of 32 years. The mean time from onset to dyspnea was 5 days. Arterial blood gas analysis at ICU admission revealed profound hypoxia. Mean partial oxygen pressure/fraction of inspired oxygen was 58.1. Mean durations from onset to a single-lobe consolidation shown on chest X-rays (CXRs) and, from the first positive CXR to bilateral multilobar lung infiltrates, were 2 days and 4.8 days, respectively. The viral load was higher than 1 × 108 copies in three patients and was 1 × 104 in one patient. It was negative in the only patient who survived. The mean duration for noninvasive positive pressure ventilation (NPPV) failure and IMV failure were 30.8 hours and 6.2 days, respectively. Four patients received venovenous ECMO. Four (80%) of the five patients died despite receiving appropriate respiratory support. CONCLUSIONS: HAdV-55 may cause severe ARDS in immunocompetent young men. Persistent high fever, dyspnea and rapid progression to respiratory failure within 2 weeks, together with bilateral consolidations and infiltrates, are the most frequent clinical manifestations of HAdV-55-induced severe ARDS. Viral load monitoring may help predict disease severity and outcome. The NPPV and IMV failure rates were very high, but ECMO may still be the respiratory support therapy of choice. TRIAL REGISTRATION: Clinicaltrials.gov NCT01585922. Registered 20 April 2012.

Increased lung cancer risk in patients with interstitial lung disease and elevated CEA and CA125 serum tumour markers.

BACKGROUND AND OBJECTIVE: The aetiology and pathogenesis of interstitial lung disease (ILD) and ILD combined with lung cancer (ILD-CA) are unclear. We aim to investigate serum tumour marker (STM) levels and to explore their predictive and diagnostic value of cancer in ILD. METHODS: Fifty-eight patients with ILD-CA, 632 with ILD only and 628 with acute respiratory illness were studied. Serum levels of carcinoembryonic antigen (CEA), carbohydrate antigen 19-9 (CA19-9), CA125 and neuron-specific enolase (NSE) were measured. RESULTS: All STM levels were elevated in ILD-CA compared with ILD group (P < 0.01). CEA and CA125 levels were significantly higher in ILD than in controls (P < 0.01). After adjustment for gender, age and smoking, ILD-CA risk in the CEA and CA125 fourth quartiles was increased compared with the first quartiles (CEA: odds ratio (OR) = 8.7, 95% confidence interval (CI) = 2.0-37.6; CA125: OR = 9.8, 95% CI = 2.3-42.7). Receiver operating characteristic (ROC) curve analysis in patients with ILD-CA showed a cut-off points of 3.99 ng/mL for CEA and 35.00 U/mL for CA125 with sensitivities of 74.6% and 71.9%, specificities 70.4% and 66.1%, and the areas under the curve 0.76 (95% CI = 0.69-0.82) and 0.75 (95% CI = 0.69-0.81), respectively. CONCLUSIONS: Serum CEA and CA125 levels are often elevated in ILD patients. The risk of cancer in ILD is increased with an elevation of serum CEA and CA125 levels. Serum CEA and CA125 levels may be a marker of cancer in ILD patients.

A new species of Neohydatothrips (Thysanoptera: Sericothripinae) from China.

Neohydatothrips stachyurus sp. n. is described from Guizhou, China. Morphologically, this new Sericothripinae species is characterized by the shape of blotch on pronotum and the distribution of microtrichia on abdominal segments. The distribution of Neohydatothrips species from China also is discussed.

Inhibiting virus replication and excessive inflammatory response: Mechanism of combined prescription of Ma-Xing-Shi-Gan decoction and Xiao-Chai-Hu decoction against influenza virus.

ETHNOPHARMACOLOGICAL RELEVANCE: The combined prescription of two classical decoctions (Ma-Xing-Shi-Gan decoction with Xiao-Chai-Hu decoction), named as San-Yang-He-Zhi (SYHZ) decoction, has been widely used for the treatment of influenza virus (IFV) infections for decades. AIM OF THE STUDY: This study aimed to evaluate the anti-influenza effect of SYHZ decoction and explore the underlying mechanism. MATERIALS AND METHODS: The ingredients of SYHZ decoction were analyzed by mass spectrometry. An animal model of IFV infection was established by challenging C57BL/6J mice with PR8 virus. Three groups of mice were infected with lethal or non-lethal doses of IFV, then followed by oral administration of phosphate-buffered saline (PBS), or SYHZ, or oseltamir; blank control mice (without IFV infection) were treated with PBS. Survival rate, Lung index, colon length, body weight loss and IFV viral load were measured 7 days post infection; histology and electron-microscopy examinations of lung tissue were performed; cytokine and chemokine levels in lung and serum were measured; and the intestinal metagenome, the cecum metabolome, and the lung transcriptome were analyzed. RESULTS: SYHZ treatment significantly improved survival rate compared with PBS (40% vs 0%); improved lung index, colon length, and body weight loss; and alleviated lung histological damage and viral load. SYHZ-treated mice had significantly lower levels of IL-1ß, TNF-α, IL-6, CCL2, CXCL10 in lung and serum, and increased levels of multiple bioactive components in cecum. Pro-inflammatory cytokines, Toll- and NOD-like receptors, pro-apoptosis molecules, and lung-injury-related proteins were downregulated in SYHZ mice, whereas surfactant protein and mucin were upregulated. The NOD-like receptor pathway, Toll-like receptor pathway, and NF-κB pathway were downregulated by SYHZ treatment. CONCLUSIONS: SYHZ decoction alleviated IFV infection in a mouse model. Multiple bioactive ingredients of SYHZ may inhibit replication of IFV and suppress excessive immune response.

Double tracheal stents reduce side effects of progression of malignant tracheoesophageal fistula treated with immunotherapy: A case report.

BACKGROUND: The protective effect of tracheal stents is reported to relieve airway obstruction and reduce side effects of rapid progression of malignant tracheoesophageal fistula (MTEF) after immunotherapy in this case with 10 mo follow-up. CASE SUMMARY: Two kinds of silicone stents were placed in the main airway of a 58-year-old male to relieve the airway obstruction caused by advanced esophageal carcinoma. The patient then received four doses of toripalimab. Subsequently, rapid, progressive deterioration of the original fistula was found. Although the fistula enlarged rapidly after immunotherapy, it remained covered completely, and likely because of this, his condition remained stable. Therefore, immunotherapy could be continued to treat the primary tumor. Despite these efforts, the patient died of the advancement of his esophageal cancer. CONCLUSION: Appropriately-sized tracheal stent placement combined with immune checkpoint inhibitors may improve the quality of life and survival of patients with MTEF.

Qingfeiyin Decoction Inhibits H1N1 Virus Infection via Modulation of Gut Microbiota and Inflammatory Pathways in a Murine Model.

Influenza virus-caused lung infection and its pandemic outbreaks are a persistent public health challenge. The H1N1 subtype is the most common type of influenza infection observed in humans. Maxingshigantang decoction, a classic formula of Chinese herbal medicine, has been used for the prevention and treatment of respiratory infection for many centuries. Qingfeiyin decoction, based on Maxingshigantang, has been used in the clinic for decades. To explore the underlying mechanisms, according to the traditional Chinese medicine theory "the lung and the large intestine are interior-exterior," which can be translated to the "gut-lung axis" in a contemporary term, the composition of gut microbiota was determined using 16S rRNA and the transcriptome of the colon was determined by RNA sequencing. The results showed that Qingfeiyin decoction decreased the viral load, alleviated the lung injury, increased the survival rate, partly restored the shortening of the colon caused by the H1N1 virus, and downregulated inflammatory pathways including MAPK, TNFα, and JAK-STAT signaling pathways. Qingfeiyin decoction increased the relative abundance of the genera of Coprococcus , Ruminococcus, Lactobacillus, and Prevotella and prevented the H1N1 virus-induced decrease in the abundance of the genera of Escherichia, Parabacteroides, Butyricimonas, and Anacrotruncus. These results will help better understand the mechanisms for Qingfeiyin decoction's protective effect against influenza virus infection.

Efficacy and safety of Jin-shui Huan-xian granule for idiopathic pulmonary fibrosis: study protocol for a multicenter, randomized, double-blind, placebo-controlled trial.

BACKGROUND AND RATIONALE: Idiopathic pulmonary fibrosis is a critical disease with a poor prognosis. Although different studies have been conducted for the treatment of idiopathic pulmonary fibrosis, limited treatments are available. Jin-shui Huan-xian granule (JHG), which is a Chinese medicine herbal compound, has shown promising efficacy in reducing frequencies of acute exacerbations, improving exercise capacity the quality of life of patients with idiopathic pulmonary fibrosis. This study is to evaluate the efficacy and safety of JHG for IPF. SUBJECTS AND METHODS: This is a multicenter, randomized, double-blind, placebo-controlled clinical trial. A total of 312 idiopathic pulmonary fibrosis patients will be enrolled and randomly allocated to one of the two groups with 1:1. After a 2-week washout period, 52-week treatment will also be performed for all the patients. Patients in the experimental group and the control group will be given JHG and JHG placebo, respectively. Outcome measures including acute exacerbations, pulmonary function, dyspnea, exercise capacity, and quality of life will be evaluated in this study. DISCUSSION: Based on our previous study, it is hypothesized that JHG will reduce acute exacerbations; improve exercise capacity, pulmonary function, and quality of life; and delay the disease progression-free. High-level evidence-based support for TCM in IPF will also be obtained in this study. TRIAL REGISTRATION: ClinicalTrials.gov NCT04187690. Register on December 11, 2019.

[Chest high resolution CT features of extrinsic allergic alveolitis and its diagnostic value].

OBJECTIVE: To summarize the chest high-resolution CT (HRCT) features of the patients with extrinsic allergic alveolitis (EAA). METHODS: We analyzed the images of chest HRCT of 34 patients diagnosed as EAA at our hospital from February 2001 to August 2009. RESULTS: All patients had a history of environmental exposure. The duration of intermittent or continuous antigen exposure was from 3 months to 13 years. Two patients showed acute clinical manifestations. There were 22 sub-acute and 10 chronic cases. Acute EAA was characterized by ground-glass opacities, air trapping and/or mosaic sign on HRCT. The HRCT features of subacute EAA included patchy ground-glass opacities with mosaic sign (n = 11, 50.0%) and diffusely distributed centrilobular nodules (n = 7, 31.8%) with mosaic sign (n = 4, 18.2%). All patients with chronic EAA had reticular and honeycombing lesions on HRCT. There were 3 cases with ground-glass opacities, 3 with mosaic sign, and 3 with centrilobular nodules. CONCLUSION: The typical findings of chest HRCT are helpful for making a diagnosis and differential diagnosis of EAA.

[Causes of respiratory failure complicating interstitial lung disease and application value of mechanical ventilation].

OBJECTIVE: To investigate the potential causes of respiratory failure in patients with interstitial lung disease (ILD) and evaluate the application value of mechanical ventilation (MV) in its treatment. METHODS: This study included the clinical data of 47 ILD patients (29 males and 18 females) complicating respiratory failure and admitted to respiratory care unit (RICU) for receiving MV at Beijing Chaoyang Hospital from January 1, 1998 to June 30, 2008. The median age was 67 years old. And a retrospective analysis was conducted for clinical characteristics, potential causes of respiratory failure, RICU treatment, prognosis and causes of death, etc. RESULTS: The causes of respiratory failure were as follows: respiratory infections (n = 18, 38.3%), respiratory infection superimposed aggravated ILD (n = 15, 31.9%), pulmonary embolism (n = 8, 17.0%), ILD with acute exacerbation (n = 5, 10.6%) and heart failure (n = 1, 2.1%). And the mortality rates of RICU were 55.6% (10/18), 73.3% (11/15), 5/8, 4/5 and 1/1 respectively. Upon initial admission into RICU, 31 cases received noninvasive ventilation and 16 cases tracheal intubation. The mortality rates were 54.8% and 87.5% respectively. And there was statistically significant difference between the groups (chi(2) = 5.014, P = 0.025). In all patients, the RICU mortality rate was 66.0% (31/47) and the hospital mortality rate 70.2% (33/47). CONCLUSIONS: Respiratory infection, pulmonary embolism and acute exacerbation of ILD are the main common causes of respiratory failure in ILD patients. Noninvasive ventilation therapy is clinically preferable to invasive ventilation for ILD patients with respiratory failure.

Adult-onset Still's disease successfully treated with Chinese herbal medicine: A case report with 15-month follow-up.

Adult-onset Still's disease (AOSD) is a rare but clinically well-known, polygenic, and systemic autoinflammatory disease, which is characterized by spiking fever, evanescent skin rash, arthralgia, and sore throat. The application of non-steroidal anti-inflammatory drugs and glucocorticoids, which are first-line therapies of AOSD, is limited due to their side effects such as liver injury or disorder of blood glucose. Therefore, patients who suffer from systemic diseases in China prefer to seek help from Chinese herbal medicine (CHM), which is an important part of complementary and alternative medicine. In this case, we report a 28-year-old male badminton coach presenting with a 15-day history of fever and skin rash, accompanied by sore throat, fatigue, myalgia and chills. Additionally, hepatosplenomegaly, multiple lymphadenopathies, aminotransferase abnormality, and elevated inflammatory factor levels were observed during hospitalization. Infectious diseases, solid tumors, hematological diseases, and common autoimmune diseases were excluded. Not benefitting from antibiotic therapy, the patient was finally diagnosed with AOSD, after a careful examination, then showed rapid remission after a six-week treatment with CHM granules based on Xiaochaihu Decoction and Yinqiao Powder. After stopping the treatment, there was no relapse within a 15-month follow-up period. To the best of our knowledge, this is the first well-documented case of this successful treatment. The present case report suggests that CHM is a reliable choice for complementary and alternative therapy for AOSD, but confirming the utility of CHM for AOSD requires further support from prospective studies.

Idiopathic Pulmonary Fibrosis Registry China study (PORTRAY): protocol for a prospective, multicentre registry study.

INTRODUCTION: Idiopathic pulmonary fibrosis (IPF) is a progressive and lethal lung disease characterised by a fibrotic histological pattern found in usual interstitial pneumonia. Its causes, pathogenesis, clinical phenotype and molecular mechanisms are poorly defined. Large-scale, multicentre studies are warranted to better understand IPF as a disease in China, its associated risk factors, clinical characteristics, diagnosis, disease progression and treatment. METHODS AND ANALYSIS: The Idiopathic Pulmonary Fibrosis Registry China Study (PORTRAY) is a prospective, multicentre registry study of patients with IPF in China. Eight hundred patients will be enrolled over a 36-month period and followed for at least 3 years to generate a comprehensive database on baseline characteristics and various follow-up parameters including patient-reported outcomes. Biological specimens will also be collected from patients to develop a library of blood, bronchoalveolar lavage fluid and lung biopsy samples, to support future research. As of 15 December 2019, 204 patients from 19 large medical centres with relatively high IPF diagnosis and treatment rates had been enrolled. Patient characteristics will be presented using descriptive statistics. The Kaplan-Meier method will be used for survival analyses. Repeated measures will be used to compare longitudinal changes in lung function, imaging and laboratory tests. Results following analysis have been projected to be available by July 2025. ETHICS AND DISSEMINATION: The study protocol was reviewed and approved by the Institutional Review Board from all the study sites currently recruiting patients. Study results will be published in peer-reviewed journals. TRIAL REGISTRATION NUMBER: NCT03666234.

[Association of ENA-78, IP-10 and VEGF gene polymorphism with idiopathic pulmonary fibrosis].

OBJECTIVE: To examine whether there was an association between epithelial neutrophil activating peptide 78 (ENA-78), interferon-inducible protein 10 (IP-10), vascular endothelial growth factor (VEGF) polymorphism and Chinese patients with idiopathic pulmonary fibrosis (IPF). METHODS: Polymerase chain reaction (PCR) and restriction fragment length polymorphism (RFLP) were performed to analyze the gene polymorphisms of ENA-78 (-156G/C), IP-10 (-1596C/T) and VEGF (+405G/C) in 60 IPF patients and 60 controls with trauma or bone fracture matched in age, gender and smoking status. RESULTS: The distribution of carrying GC + CC genotype frequency (20.0%) and C allele frequency (12.7%) for ENA-78 in IPF patients was significantly higher than that in healthy controls [6.7% (P = 0.032) and 3.3% (P = 0.008), respectively], the relative risk of suffering from IPF of -156C allele gene carrier significantly increased (OR = 4.23, 95%CI: 1.35-13.20). The distribution of carrying CT + TT genotype frequency (10.0%) and T allele frequency (5.8%) for IP-10 in IPF patients was significantly lower than that in healthy controls [26.7% (P = 0.018) and 14.2% (P = 0.031), respectively], the relative risk of suffering from IPF of -1596T allele gene carrier decreased (OR = 0.38, 95%CI: 0.15-0.95). No association was found between VEGF (+405G/C) polymorphism and IPF. CONCLUSIONS: -156C allele for ENA-78 may be a risk factor of IPF and -1596T allele for IP-10 a beneficial factor of IPF. The VEGF (+405G/C) gene polymorphism has no effect upon the predisposition to IPF.

Clinical features and prognosis of microscopic polyangiitis with usual interstitial pneumonia compared with idiopathic pulmonary fibrosis.

INTRODUCTION: Pulmonary involvement in microscopic polyangiitis (MPA) is common, little is known about the clinical features of MPA with interstitial pneumonia (MPA-IP). OBJECTIVES: This study aimed to explore the prevalence of microscopic polyangiitis associated usual interstitial pneumonia (UIP)(MPA-UIP) and compare its clinical features and prognosis with those of MPA-non-UIP and idiopathic pulmonary fibrosis (IPF). METHODS: A total of 73 patients with MPA-IP were identified and divided into MPA-UIP patients and MPA-non-UIP patients. The clinical characteristics and survival of MPA-UIP patients were analysed and compared with those of MPA-non-UIP patients and 68 patients with IPF. RESULTS: The results showed that 34/73 (47%) MPA-IP patients were classified as MPA-UIP patients. Compared with MPA-non-UIP patients, MPA-UIP patients tend to have longer duration of symptoms prior to diagnosis and usually have pulmonary involvement as the initial presentation. However, they were less likely to have proteinuria and/or hematuria. Compared with IPF patients, MPA-UIP patients usually had multisystem damage, positive anti-neutrophil cytoplasmic autoantibodies and elevated levels of nonspecific inflammatory markers. MPA-UIP death was concentrated mainly in the first 3 months after diagnosis and resulted in a higher early mortality compared with IPF. CONCLUSION: UIP is the most frequent type of MPA-IP. These patients tend to have longer duration of symptoms prior to diagnosis and usually have pulmonary involvement as the first presentation. However, they are less likely to have proteinuria and/or hematuria. MPA patients with UIP can be differentiated from IPF patients through comprehensive analysis of clinical and laboratory findings.

Incidence and radiologic-pathological features of lung cancer in idiopathic pulmonary fibrosis.

OBJECTIVE: To investigate the incidence and risk factors of lung cancer in patients with idiopathic pulmonary fibrosis (IPF), and to learn the clinical, imaging and pathological features and of lung cancer in IPF. METHODS: The study population included consecutive 268 IPF patients. Of them, 46 patients had pathologically or cytologically proven lung cancer. The demographic, clinical, HRCT and pathological features in patients with IPF and lung cancer were analysed and compared with the patients with IPF alone. RESULTS: Of 268 IPF patients, 46 patients were diagnosed as IPF with lung cancer, accounted for 17.16%. 45.65% were adenocarcinoma. Lung cancer located mostly in the peripheral area and lower lobes of lungs consistent with IPF affected area. Old age and heavy smoking were risk factors of lung cancer developing in IPF. Chest pain and haemoptysis were more frequent in IPF patients with lung cancer than without lung cancer (P = .000). Nodular or mass shadows were found only in IPF patients with lung cancer (P = .000). The levels of CEA and CA125 in IPF patients were much higher in IPF patients with lung cancer (P ≤ .001). The median survival time was 36.2 ± 22.7 months in IPF patients, longer than 6.9 ± 3.3 months in IPF patients with lung cancer (P < .001). CONCLUSIONS: Lung cancer frequently develops in patients with IPF, which is mainly adenocarcinoma, located in IPF affected area. Chest pain and haemoptysis are potential indicative of lung cancer developing in patients with IPF as atypical nodes or masses located in the peripheral areas and lower lobes on chest HRCT.

Fibrinolytic system related to pulmonary arterial pressure and lung function of patients with idiopathic pulmonary fibrosis.

OBJECTIVES AND AIMS: To investigate urokinase-(uPA) and tissue-type (tPA) plasminogen activator and plasminogen activator inhibitor type-1 (PAI-1) levels in patients with idiopathic pulmonary fibrosis (IPF) and to determine the relationship between fibrinolytic system and pulmonary arterial pressure and pulmonary function. METHODS: Seventy-nine patients with IPF were included. Bronchoalveolar lavage fluid (BALF) and blood samples were collected. The concentrations of tPA, uPA and PAI-1 were measured using enzyme-linked immunosorbent assay. Doppler echocardiography was used to detect tricuspid regurgitation pressure gradient (TRPG) to estimate pulmonary arterial pressure. RESULTS: BALF tPA elevated (P < 0.005), circulatory PAI-1 decreased (P = 0.05) and the ratio of uPA and PAI-1 decreased (P = 0.01) in BALF in IPF patients with pulmonary hypertension (PH) compared to those without PH. Positive linear correlations were found: BALF tPA and TRPG (r = 0.558, P = 0.013); the predicted percentage of diffusion capacity of lung for carbon monoxide adjustments for alveolar volume and BALF uPA (r = 0.319, P = 0.035). Negative linear correlations were as follows: BALF PAI-1 and the predicted percentage of VCmax (r = -0.325, P = 0.020), or total lung capacity (r = -0.312, P = 0.033); circulatory PAI-1 and TRPG (r = -0.697, P = 0.003). CONCLUSIONS: The change of alveolar fibrolytic system in IPF, especially the uPA reduction and the PAI-1elevation, contributes to the deterioration of lung function. During the lung injury initiating fibrosis, tPA and PAI-1 might be leaked out of the pulmonary capillaries into alveoli, resulting in their elevation in alveoli and reduction in circulation, and finally contributing to the development of PH in IPF.