Objective To investigate the prevalence of obstructive sleep apnea hypopnea syndrome ( OSAHS) in patients with idiopathic pulmonary fibrosis ( IPF) and its clinical significance. Methods Sleep quality and breathing disorders were measured by polysomnography and the relationship with lung function was analyzed in 20 IPF patients. Results Thirteen of 20 subjects ( 65% ) had OSAHS as defined by an AHI ≥5 events per hour. Three subjects ( 15% ) had mild OSAHS ( AHI,5 to 20 events per hour) , and 10 subjects ( 50% ) had moderate-to-severe OSAHS ( AHI≥20 events per hour) . The sleep architecture in these patients showed a reduction in sleep efficiency, rapid eye movement ( REM) sleep and slow wave sleep, and a marked sleep fragmentation due to an increased arousal index. The AHI was negatively correlated with FVC% pred ( r =-0.672, P=0.001) and FEV1% pred ( r =-0.659, P=0.002) , and positively correlated with body mass index ( BMI) ( r=0.791, Plt;0.0001) . Conclusions OSAHS is a common comorbidity in IPF. Early treatment of OSAHS may improve quality of life and the prognosis of patients with IPF.
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.
Objective To systematically evaluate the effectiveness of N-acetylcysteine (NAC) combined with low-dose glucocorticoid for patients with idiopathic pulmonary fibrosis (IPF). Methods Such databases as The Cochrane Library (Issue 12, 2012), EMbase (January 1974 to July 2012), PubMed (January 1966 to July 2012), CHEST (January 1995 to July 2012), CNKI (January 1994 to July 2012), CBM (January 1978 to July 2012), VIP (January 1989 to July 2012) and WanFang Data (January 1995 to July 2012) were searched to collect the randomized controlled trials (RCTs) about NAC combined with low-dose glucocorticoid versus glucocorticoid alone for IPF patients. Two reviewers independently screened the literature according to the inclusion and exclusion criteria, extracted the data, and assessed the quality, and then the meta-analysis was performed using RevMan 5.1 software. Results A total of seven RCTs including 264 IPF patients were included. The results of meta-analysis demonstrated that, compared with the glucocorticoid used alone, a) NAC combined with low-dose glucocorticoid could significantly improve PaO2 (SMD=0.82 mmHg, 95%CI 0.30 to 1.35, P=0.002) and DLco (SMD=0.59 mmHg, 95%CI 0.16 to 1.03, P=0.008) with a significant difference. b) NAC combined with low-dose glucocorticoid could significantly improve all clinical symptoms (RR=1.56, 95%CI 1.26 to 1.92, Plt;0.000 1). Conclusion NAC combined with low-dose glucocorticoid for IPF patients can significantly improve PaO2, DLco, and the clinical symptoms such as cough, difficulty breathing after activities, cyanosis, and Velcro rales. Due to the quantity and quality limitation of included studies, this conclusion still needs to be further proved by more high quality and double blind RCTs.
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.
ObjectiveTo detect the levels of Krebs von den lungen 6 (KL-6) in bronchoalveolar lavage fluid (BALF) and serum of patients with idiopathic pulmonary fibrosis (IPF),and explore its clinical significance.
MethodsThirty-four patients with IPF and 10 patients with sarcoidosis in Ⅰ period were recruited in the study. ELISA was used to detect the level of KL-6 in BALF and serum.
ResultsIn the IPF group,the forced vital capacity as percentage of predicted value (FVC% pred) and diffusion capacity for carbon monoxide as percentage of predicted value (DLCO %pred) were both significantly lower than those of the sarcoidosis group[(69.51±13.65)% vs. (82.06±5.84)%,(48.58±12.73)% vs. (81.47±6.39)%,P<0.01]. In the BALF of IPF group,the percentage of neutrophils was higher[(8.91±6.79)% vs. (5.50±3.60)%,P<0.05],and the percentages of lymphocytes and CD4/CD8 ratio were lower than those of the sarcoidosis group[(11.71±6.64)% vs. (23.30±12.68)%,(1.46±0.83) vs. (4.01±5.10),P<0.05]. In the IPF group,the level of KL-6 in the BALF and serum was higher than that of the arcoidosis group[(437.43±251.70) U/mL vs. (221.59±127.41) U/mL,(857.81±515.53) U/mL vs. (338.67±168.13) U/mL,P<0.001]. There was obvious correlation between the level of serum KL-6 with FVC%pred and DLCO%pred in the IPF group (r=-0.46,r=-0.58,P<0.05).
ConclusionsThe level of KL-6 in BALF and serum is elevated in patients with IPF. There is obvious correlation between the level of serum KL-6 with FVC%pred and DLCO%pred in IPF patients. KL-6 may be an indicator of IPF in clinical diagnose.
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.
ObjectiveTo systematically review the efficacy and safety of N-acetylcysteine (NAC) for patients with idiopathic pulmonary fibrosis (IPF).
MethodsWe electronically searched PubMed, EMbase, The Cochrane Library (Issue 2, 2014), CNKI, WanFang Data and VIP databases from the date of establishment to February 2014 for all randomized controlled trials (RCTs) on the use of NAC in patients with IPF. Manual search in relevant journals were also performed. The data extraction and quality assessment of included RCTs were conducted by two reviewers independently. Then, meta-analysis was conducted using RevMan 5.1 software.
ResultsA total of 13 RCTs involving 713 patients were included. The results of meta-analysis indicated that the NAC group was better than the control group in clinical effectiveness (RR=1.34, 95%CI 1.19 to 1.51, P < 0.000 1). After treatment, the lung function was also improved in the NAC group than in the control group in the following index:PaO2 (MD=6.06, 95%CI 3.79 to 8.32, P < 0.000 01), vital capacity (VC) (%) (MD=4.79, 95%CI 0.35 to 9.24, P=0.03) and diffusing capacity of carbon monoxide (Dlco) (%) (MD=5.74, 95%CI 2.67 to 8.81, P=0.000 2). However, no significant difference was found between groups in total lung capacity (TLC) (%) (MD=5.56, 95%CI-1.73 to 12.86, P=0.14). No serious or frequently-happened adverse effect was reported in the NAC group.
ConclusionThe current evidence suggests that NAC in long term use could improve clinical conditions, PaO2 and lung function of IPF patients, with less adverse effects.
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.
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.
