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.
【Abstract】 Objective To evaluate the relationship between multiple tumor biomarkers and idiopathic pulmonary fibrosis ( IPF) , and analyze the prognostic value of these biomarkers in IPF. Methods Clinical data of 43 confirmed IPF patients with no evidence of malignant disaeses, admitted in Peking Union Medical College Hospital between January 2000 and June 2010, were retrospectively analyzed. All IPF patients had detected serum alpha fetoprotein ( AFP) , cancer antigen 50 ( CA50) , cancer antigen 24-2( CA24-2) , carcinoembryonic antigen ( CEA) , carbohydrate antigen 19-9 ( CA19-9) , cancer antigen 125( CA125) , cancer antigen 15-3 ( CA15-3) , tissue polypeptide antigen ( TPA) , neuron specific enolase( NSE) , and cytokeratin-19-fragment ( Cyfra211) . Results The serum levels of CEA, CA19-9, CA125,CA15-3, and TPA were obviously higher than normal range, while the serum levels of AFP, CA50, CA24-2,NSE, and Cyfra211 were within normal range. Neither tumor biomarkers had correlation with 6-minute walk distance, FVC% pred, TLC% pred, DLCO/VA, PaO2 , PaO2 /FiO2 , P( A-a) O2 , BALF cell differentiation counting,or CD4 /CD8. The patients with increased CA19-9 level had shorter survival time than those with normal CA19-9 level ( P lt; 0. 05) . There was no significant difference in survival time between the patients with increased CEA/TPA levels and those with normal CEA/TPA levels( P gt;0. 05) , neither between the patients with glucocorticoid treatment and those with non-glucocorticoid treatment ( P gt; 0. 05) . Conclusions Multiple tumor biomarkers, especially CA19-9, increase in IPF patients. The degrees of those increases arenot associated with the severity of disease, but closely relate to prognosis, and may also indicate the progression. The increases of multiple tumor biomarkers may be a sign of poor prognosis of IPF with no evidence of malignant disaeses.
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.
ObjectiveTo understand the genetic polymorphisms of MUC5B and TOLLIP in Chinese patients with idiopathic pulmonary fibrosis (IPF), and to explore whether gene polymorphism variation in Chinese IPF patients can be used as a genetic biomarker for accurate treatment and prognosis judgment.MethodsA total of one hundred and twenty-six patients with IPF were enrolled in this study. The baseline characteristics, total lung capacity (TLC), forced vital capacity (FVC), carbon monoxide diffusion function (DLCO), imaging changes of the patients were followed up. The levels of serum sputum glycosylated antigen-6 (Krebs Von den Lungen-6, KL-6) and B lymphocyte chemotactic factor C-X-C motif chemokine 13 (CXCL13) were detected by chemiluminescent enzyme immunoassay and enzyme-linked immunosorbent assay. The gene MUC5B rs35705950 and TOLLIP rs5743890, rs5743894 single nucleotide polymorphism (SNP) were determined by polymerase chain reaction.ResultsOne hundred and twenty-six patients with IPF were found with AA type by TOLLIP rs5743890 and rs5743894 SNP, accounting for 100.0%; MUC5B rs35705950 SNP was expressed as 116 patients (92.1%) with GG type, and 10 patients (7.9%) with GT type, no TT patients were detected. There was no significant difference in clinical characteristics between the two groups in age and non-smokers (P>0.05). Compared with group G, annual decrease of lung function (FVC, DLCO, and TLC), serum biomarkers (KL-6 and CXCL13), annual increase of reticular and honeycombing lesions, and mortality were significantly lower in group T (P<0.05). The median survival time of IPF patients carrying the MUC5B SNP rs35705950 minor allele (gene phenotype GT) heterozygous was significantly higher than that of homozygous IPF patients with a genetic phenotype of GG.ConclusionsThere are genetic polymorphisms in Chinese patients with IPF. MUC5B rs35705950 and TOLLIP rs5743890, rs5743894 gene subtypes have low mutation rates in the cohort. Compared with homozygous patients of MUC5B SNP rs35705950, heterozygous patients have smaller changes in lung function and radiological image, lower levels of serum KL-6 and CXCL13, and better prognosis.
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 investigate the impact and mechanisms of periostin (POSTN), Krebs von den Lungen-6 (KL-6), pulmonary surfactant protein A (SP-A), and pulmonary surfactant protein D (SP-D) on the diagnosis and disease assessment of idiopathic pulmonary fibrosis (IPF), and conduct a comparative analysis. Methods From October 2022 to October 2023, a total of 55 patients diagnosed with IPF and treated at the Third Affiliated Hospital of Anhui Medical University were enrolled as an IPF group. Additionally, 30 patients with bacterial pneumonia and 30 healthy individuals undergoing concurrent health examinations during the same period were selected as a pneumonia control group and a healthy control group, respectively. All participants underwent enzyme-linked immunosorbent assay to measure serum levels of POSTN, KL-6, SP-A, and SP-D, along with pulmonary function tests. The IPF patients also underwent high-resolution computed tomography (HRCT) and echocardiography to quantify HRCT scores and pulmonary artery systolic pressure (PASP). Receiver operating characteristic (ROC) curves were plotted to analyze the significance of serum POSTN, KL-6, SP-A, and SP-D levels in IPF diagnosis. Pearson and Spearman correlation tests were used to analyze the relationships between these biomarkers and pulmonary function, PASP, and HRCT scores. Results Serum concentrations of POSTN, KL-6, SP-A, and SP-D were significantly elevated in the IPF group compared with the pneumonia group and the healthy controls (P<0.05), while serum levels of SP-A and SP-D were notably higher in the pneumonia group compared with the healthy control group (P<0.05). Within the IPF group, serum POSTN levels were negatively correlated with forced expiratory volume in the first second as a percentage of predicted value (FEV1%pred) and diffusion capacity of the lung for carbon monoxide as a percentage of predicted value (DLCO%pred) (P<0.05); KL-6 and SP-D levels were also negatively correlated with FEV1%pred, forced vital capacity as a percentage of predicted value (FVC%pred), and DLCO%pred (P<0.05); and the concentration of SP-A was negatively correlated with DLCO%pred and positively correlated with PASP (P<0.05). Additionally, serum levels of POSTN, KL-6, and SP-A in the IPF group showed significant positive associations with HRCT scores (P<0.01). Conclusions POSTN is a valuable serum biomarker for IPF, exhibiting the highest sensitivity and specificity among the four serum markers, with diagnostic performance superior to KL-6, SP-A, and SP-D. POSTN, KL-6, SP-A, and SP-D can all be used for the diagnosis and assessment of IPF.
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.
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.
美国胸科协会(ATS)和欧洲呼吸学会(ERS)联合发表的共识中，将特发性肺纤维化(IPF)定义为原因不明并以普通型间质性肺炎(UIP)为特征性病理改变的一种慢性纤维化性间质性肺疾病。在2000年ATS／ERS的IPF共识意见 及2003年中华医学会呼吸病学分会IPF的诊断和治疗指南(草案) 中均推荐的治疗方案为糖皮质激素，或与细胞毒制剂(环磷酰胺及硫唑嘌呤)联合使用。但目前尚缺乏循证医学证据支持该治疗方案能够提高IPF患者生活质量或生存率 。近年来随着对IPF的发病机制认识的深入，越来越多的临床医师和研究者对IPF患者是否需要用糖皮质激素等药物的治疗提出了质疑。
ObjectiveTo evaluate the efficacy and safety of pirfenidone in patients with idiopathic pulmonary fibrosis. MethodsPubMed, Cochrane Library, CNKI, CBM, Wanfang, and VIP databases were searched for randomized controlled trials of pirfenidone as interventions for the treatment of idiopathic pulmonary fibrosis. According to the Cochrane system evaluation method, the methodological quality of included studies was evaluated and the effective data were extracted. The meta-analysis was performed with RevMan 5.2 software. ResultsSix studies were included with 1727 patients in total. Compared with placebo groups, pirfenidon could improve the changing rate of vital capacity at the end of the treatment[WMD=0.06, 95% CI (0.01, 0.12), Z=2.48, P=0.01; heterogeneity inspection χ2=1.03, P=0.31]. Pirfenidon could not improve the changing rate of lowest SpO2 in 6-minute walking test[WMD=0.82, 95% CI (-1.35, 2.98), Z=0.74, P=0.46; heterogeneity inspection χ2=8.90, P=0.003] and could not reduce the mortality[RR=0.62, 95% CI (0.37, 1.03), Z=1.85, P=0.06; heterogeneity inspection χ2=3.05, P=0.55]. The incidences of photosensitivity, dizziness, nausea, abdominal discomfort, joint pain, fatigue in pirfenidone group were more frequent than those in placebo group. ConclusionsBecause of lack of enough eligible studies and defects in design and reporting data in the studies, this meta-analysis can not evaluate pirfenidone's long-term efficacy and safety. Hence, the existed clinical evidences can't support pirfenidone to be the treatment of IPF medication.