Acute exacerbation of idiopathic pulmonary fibrosis (AE-IPF) is defined as an acute and clinically significant respiratory deterioration characterized by evidence of new, widespread alveolar abnormality. In the past, AE-IPF was considered to be idiopathic, which was hard to be prevented and its prognosis was hard to be obviously improved; the latest researches have shown that AE-IPF can be triggered by known causes, including pulmonary infection, aspiration, etc. This review summarizes the etiology or risk factors, treatment and prevention of AE-IPF according to the latest researches.
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 systematically evaluate the prognostic prediction models for Idiopathic Pulmonary Fibrosis (IPF). MethodsA computer-based search was conducted in the PubMed, Embase, Web of Science, and Cochrane Library databases for literature relevant to the research objective, with the search period ranging from database inception to Jun 2025. Two researchers independently screened the articles. Data were extracted according to the key assessment and data extraction checklist for systematic reviews of prediction models (CHARMS). The risk of bias and applicability of the models were assessed using the PROBAST (Prediction model Risk of Bias Assessment Tool). The quality of model reporting was evaluated using the TRIPOD (Transparent Reporting of a Multivariable Prediction Model for Individual Prognosis or Diagnosis) checklist. ResultsA total of 49 studies were included, of which 26 (53.06%) reported both model development and validation. The most common predictors included gender, age, diffusing capacity for carbon monoxide, forced vital capacity (FVC), and FVC percentage of predicted value. In terms of bias risk, 32 studies (65.31%) were classified as high risk of bias, mainly due to factors related to study subjects and predictors. Regarding applicability, 26 studies (53.06%) were rated as high risk, 11 studies (22.45%) were rated as unclear, and only 12 studies (24.49%) were rated as low risk, suggesting limited clinical applicability of the models. As for reporting quality, existing models showed generally insufficient adherence to the TRIPOD statement, especially in key areas such as research methods and result reporting, where normative issues were prominent. Of the 22 signaling questions in the TRIPOD checklist, most studies achieved only moderate reporting quality, with 8 signaling questions (1, 5c, 6b, 7b, 8, 11e, 13a, 14a) showing key information omissions or vague descriptions. ConclusionExisting prognostic prediction models for IPF generally exhibit high methodological bias risk and reporting deficiencies. Future studies should control for modeling biases based on the PROBAST framework, adhere to the TRIPOD guidelines for transparent reporting, and optimize clinical applicability through external validation.
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.
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.
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 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.
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.