Objective To improve the awareness of acute exacerbation of idiopathic pulmonary fibrosis ( AEIPF) and discuss its clinical characteristics, diagnosis, treatment and outcome. Methods The clinical data of patients with AEIPF from June 2006 to June 2011 in 11 hospitals in Jiangsu were collected and analyzed. Resluts There were 18 males and 3 females in the AEIPF patients with mean age of ( 67.4 ± 8.1) years. The duration from IPF diagnosis was ( 7.4 ±8.2) months. The duration of acute symptom before admission was ( 7.0 ±5.3) days. The distribution pattern of new groud-glass opacity was peripheral in 3 patients,multifocal in 5 patients, and diffuse in13 patients. All patients were treated with corticosteroid pulse therapy. Nine patients survived and 12 patients died. The mortality rate was 57.1% . Conclusions AEIPF progresses quickly and the mortality rate is very high. Corticosteroid pulse therapy is the mainstay of therapy in AEIPF patients.
ObjectiveTo determine the diagnostic value of serum KL-6 level in patients with interstitial lung diseases (ILD). MethodsAll the ILD patients enrolled were hospitalized from April 2013 to April 2014. Patients with other pulmonary diseases and healthy subjects were chosen as control groups simultaneously. Serum KL-6 concentrations were measured by chemiluminescent enzyme immunoassay. The association with serum KL-6 level and pulmonary function was analyzed. ResultsThere were 149 ILD patients, 155 patients with other pulmonary diseases, and 64 healthy subjects. The average serum levels of KL-6 were (1 801.86±2 831.36) U/mL, (267.00±124.41) U/mL, (201.28±81.18) U/mL in the patients with ILD, the patients with other pulmonary diseases and the healthy controls, respectively. The sensitivity and the specificity of the serum KL-6 for the diagnosis of ILD was 83.89% and 92.24% respectively when the cut-off level was set at 500 U/mL. The Kappa value was 0.767 (P < 0.001). The best cut-off value of KL-6 was 469.5 U/mL. Serum KL-6 levels in the patients with ILD were significantly higher compared with the patients with chronic obstructive pulmonary disease, pneumonia, tuberculosis, bronchiectasis and the healthy controls, respectively (all P < 0.001). The KL-6 levels in the pulmonary alveolar proteinosis patients were significantly higher compared with the patients with cryptogenic organizing pneumonia (COP), the patients with idiopathic pulmonary fibrosis (IPF) and the patients with connective tissue disease (CTD-ILD) (all P < 0.001). While the KL-6 concentration in IPF and CTD-ILD were significantly higher than that in COP (P=0.003 and P=0.008, respectively). Significant negative correlations were found between the levels of serum KL-6 and vital capacity as a percentage of the predicted value, forced vital capacity as a percentage of the predicted value, forced expiratory volume in one second as a percentage of the predicted value and carbon monoxide diffusing capacity as a percentage of the predicted value (all P < 0.001). Follow-up study showed the levels of serum KL-6 were consistent with clinical efficacy. ConclusionSerum KL-6 level is a reliable serum marker for ILD, and is related with the severity of disease and clinical efficacy.
Objective To analyze the clinical presentations and radiological characteristics of acute exacerbation of idiopathic pulmonary fibrosis ( IPF) . Methods Clinical and radiological data of 2 patients with acute exacerbation of IPF from April 2006 to July 2008 were retrospectively analyzed and literatures were reviewed. Results Both patients were senior male patients over 60 years old. Dyspnea, cough and inspiratory crackles were the major symptoms and signs. Two patients were experiencing an exacerbation of dyspnea for one week and half of month, respectively. PaO2 /FiO2 of both patients was less than225 mm Hg. In both patients, high-resolution computed tomography ( HRCT) scans at the exacerbation showed typical signs of IPF including peripheral predominant, basal predominant reticular abnormality, with honeycombing and traction bronchiectasis and bronchiolectasis, and newly developing alveolar opacity. HRCT scan showed peripheral area of ground-glass attenuation adjacent to subpleural honeycombing in one patient, and diffusely distributed ground-glass opacity in another patient. Two patients had received corticosteroid treatment. For one patient, the symptoms improved, and ground-glass attenuation adjacent to subpleural honeycombing had almostly resolved. The other patient died of respiratory failure. Conclusions Some acute exacerbation in idiopatic pulmonary fibrosis can be idiopathic. The clinical presentations mainly include the worsening of dyspnea within short time. HRCT generally demonstrates new bilateral ground-glass abnormality with or without areas of consolidation, superimposed on typical changes of IPF.
After pirfenidone and nintedanib showed efficacy, drug treatment for idiopathic pulmonary fibrosis began to focused on anti-fibrosis. Current research on idiopathic pulmonary fibrosis mainly focus on the pathogenesis and therapeutic targets, and more targeted drugs are gradually entering clinical trials. This article summarizes the results of recent studies on the treatment of idiopathic pulmonary fibrosis with pirfenidone and nintedanib alone or in combination by searching the literature, and reviews the mechanism and test results of the new target anti-fibrosis drugs based on molecular biology that are currently undergoing clinical research in various phases, and aims to provide a basis for how to choose drugs to treat idiopathic pulmonary fibrosis.
美國胸科協會(ATS)和歐洲呼吸學會(ERS)聯合發表的共識中，將特發性肺纖維化(IPF)定義為原因不明并以普通型間質性肺炎(UIP)為特征性病理改變的一種慢性纖維化性間質性肺疾病。在2000年ATS／ERS的IPF共識意見 及2003年中華醫學會呼吸病學分會IPF的診斷和治療指南(草案) 中均推薦的治療方案為糖皮質激素，或與細胞毒制劑(環磷酰胺及硫唑嘌呤)聯合使用。但目前尚缺乏循證醫學證據支持該治療方案能夠提高IPF患者生活質量或生存率 。近年來隨著對IPF的發病機制認識的深入，越來越多的臨床醫師和研究者對IPF患者是否需要用糖皮質激素等藥物的治療提出了質疑。
Objective To investigate the characteristics on chest high-resolution computed tomography (HRCT) of patients with interstitial pneumonia with positive myeloperoxidase antineutrophil cytoplasmic antibody (MPO-IP). Methods The extent of fibrosis and subtypes of emphysema on HRCT of MPO-IP patients were retrospectively analyzed and compared with idiopathic pulmonary fibrosis (IPF) cases admitted in the same period. Results From July 2014 to March 2016, 10 patients was diagnosed with IPF and 10 patients was diagnosed with MPO-IP. Emphysema was not different between two groups. Among the MPO-IP patients, 8 patients presented with a usual interstitial pneumonia (UIP) pattern. There existed statistical difference in the bronchial bifurcation level, the fibrosis score of lungs in the MPO-IP patients presented with UIP was lower than that in the IPF patients. Conclusions UIP is the predominant radiologic type of MPO-IP patients. Fibrosis in IPF is more serious than that in MPO-IP with UIP. Paraseptal and centrilobular emphysema are main forms in MPO-IP patients.
Objective To explore the imaging features of acute exacerbation of idiopathic pulmonary fibrosis ( IPF) under high-resolution computed tomography ( HRCT) . Methods The HRCT imaging features of six patients who met the criteria for acute exacerbation of IPF were analyzed retrospectively. Results The manifestations of IPF on HRCT scan were various in forms and distribution, as multifocal, ground-glass opacity, reticular shadow, honeycombing densities, capillary bronchiectasis,subpleural lines, traction bronchiolectasis and emphysema. The characteristic lesions were newly diffuse bilateral ground-glass opacity at the time of acute exacerbation, superimposed on subpleural reticular and honeycombing densities. Conclusions Chest HRCT findings in acute exacerbation of IPF are characteristic.HRCT is accurate and superior in diagnosis of IPF and in determining acute exacerbation of IPF.
ObjectiveTo evaluate the clinical efficacy and safety of pirfenidone in Chinese patients with idiopathic pulmonary fibrosis (IPF). MethodsIn a multicenter,randomized,double-blind,comparative clinical trial,87 patients with IPF were randomly divided into two groups. Group A (43 patients) were treated with pirfenidone (1 200 mg per day) for 48 weeks,while Group B (44 patients) were treated with placebo. Clinical features were compared between two groups including efficacy indicators (pulmonary function,6MWT,and quality of life scores) and safety indicators (incidence of adverse events). ResultsForced vital capacity (FVC) was increased by (90±410)mL in Group A and decreased by (70±310)mL in Group B (P<0.05);In Group A,forced expiratory volume in 1 second was raised by (100±330)mL and (110±240)mL following 12 and 24 weeks after treatment,significantly different from group B (P<0.05). There were significant differences in 6MWT between two groups 36 and 48 weeks after treatment respectively(both P<0.05). Quality of life scores,including the St. George's score (excluding symptoms) and dyspnea score,were significantly higher in Group A than Group B (both P<0.05). There was no significant difference in the incidence of adverse events between Groups A and B (83.72% vs. 72.73%,P>0.05). ConclusionDomestic pirfenidone is clinically effective and safe for the treatment of IPF in Chinese patients.
2000 年美國胸科學會/ 歐洲呼吸學會( ATS/ERS) 發表了特發性肺纖維化( idiopathic pulmonary fibrosis, IPF) 診斷和治療的共識。歷經11 年, IPF 的臨床和基礎研究均取得了許多重要進展。2011 年來自美國胸科學會( American Thoracic Society,ATS) 、歐洲呼吸學會( European Respiratory Society, ERS) 、日本呼吸學會( Japanese Respiratory Society,JRS) 和拉丁美洲胸科學會( Latin American Thoracic Association, ALAT) 的間質性肺疾病( ILD) 、特發性間質性肺炎( IIP) 和IPF領域的著名專家, 系統回顧了2010 年5 月前有關IPF的文獻, 共同制定了第一部以循證為基礎的IPF診斷和治療指南( 簡稱2011 指南) , 于2011 年3 月正式頒布 。2011 指南涵蓋當今對IPF 的最新知識, 囊括IPF 定義、流行病學、危險因素、診斷、自然病史、分期與預后、治療和疾病過程監控、將來的研究方向等多方面的內容。限于篇幅, 本文對2011 指南中的主要內容作一簡介, 以饗國內同道。
Idiopathic pulmonary fibrosis (IPF) is a chronic fibrotic and interstitial lung disease of unknown cause. It has a serious impact on people's health. Traditional Chinese medicine (TCM) has certain advantages in diagnosing and treating on IPF, which have been widely used in clinic. In order to improve the diagnostic and treatment level for IPF with TCM. The Internal Medicine Committee of World Federation of Chinese Medicine Societies organized and established a multidisciplinary background working group. The document was formulated by referring to the formulation method and process of clinical practice guidelines, which are based on the best evidence and the opinions of clinical physicians and patients. Physicians can use this guideline to make clinical decisions.