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.
Since the beginning of the coronavirus disease 2019 (COVID-19) pandemic at the end of December 2019, more than 85% of the population in China has been infected. Severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) mainly affects the respiratory system, especially the lungs. The mortality rate of patients with severe infection is high. A percentage of 6% to 10% of patients will eventually develop into COVID-related acute respiratory distress syndrome (CARDS), which requires mechanical ventilation and extracorporeal membrane oxygenation (ECMO) support. Some patients who survive acute lung injury will subsequently develop post COVID-19 pulmonary fibrosis (PCPF). Both fully treated CARDS and severe PCPF are suitable candidates for lung transplantation. Due to the special course, evaluation strategies are different from those used in patients with common end-stage lung disease. After lung transplantation in COVID-19 patients, special treatment is required, including standardized nucleic acid testing for the novel coronavirus, adjustment strategy of immunosuppressive drugs, and rational use of antiviral drugs, which is a big challenge for the postoperative management of lung transplantation. This consensus was evidence-based written and was reached by experts after multiple rounds of discussions, providing reference for assessment and postoperative management of patients with interstitial pneumonia after COVID-19 infection.
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 explore the feasibility of medical ozone in treatment of pulmonary fibrosis. MethodsForty Wistar rats were randomly divided into an experimental group and a control group, with 20 rats in each group.All rats were intratreacheally instilled with bleomycin to induce pulmonary fibrosis.Then the rats were intraperitoneally injected with physiological saline every other day in the control group, and with medical ozone every other day in the experimental group.After 28 days, 10 rats in each group were sacrificed after lung function test.Right lung tissues were sampled for pathological examination, and left lung tissues were sampled for measurement of superoxide dismutase (SOD) and hydroxyproline.The remaining 10 rats in each group continued to be normally fed and intraperitoneally injected for observation of the survival time. ResultsThe lung function of the control group significantly decreased compared with the experimental group.The degree of lung fibrosis in the control group was more severe than that in the experimental group (lung fibrosis score: 1.9±0.5 vs.1.2±0.4, P < 0.05). The level of SOD in lung tissue was significantly higher and the level of hydroxyproline was significantly lower in the experimental group compared with the control group [(208.48±29.37)U·mg-1·pro-1 vs.(163.34±21.42) U·mg-1·pro-1, (2.25±0.28) mg/g vs.(2.68±0.37) mg/g, P < 0.05].The rats in the experimental group had longer survival time compared with the control group (79 d vs.59 d, P < 0.05). ConclusionMedical ozone can delay the progress of pulmonary fibrosis in rats.
Objective To study the pathology and possible mechanism of experimental hydrochloric acid（HCl） inhalation-indued pulmonary fibrosis in rats.Methods 120 male SD rats were randomly divided into a nomal control group，a bleomycin group，a high dose HCl group，a middle dose HCl group and a low dose HCl group.The bleomycin group was intratracheally injected with bleomycin once to induce pulmonary fibrosis.The three HCl groups were intratracheally injected with HCl once per week.The control group was given saline by the same way.Six rats of each group were randomly sacrificed on day 7，14，28 and 42 respectively.The histological changes of lung tissue were studied by HE and Masson’s trichrome staining.Hydroxyproline level in lung tissue was measured by digestion method.Protein and mRNA expression of transforming growth factor-β1（TGF-β1） were assayed by immunohistochemistry and RT-PCR respectively.Results Alveolitis in three HCl groups was significantl compared to control group，most severe at the second week，then remained at a high level which was equivalent to or exceeded the level of the bleomysin group after 28 days.Pulmonary fibrosis in three HCl groups was also significantly more severe than that in the control group，but milder than that in the bleomysin group.The high-dose and middle-dose HCl groups were not significantly different from the bleomysin group on day 42.There was no difference between three HCl groups in the earlier period，but the high-dose HCl group has a significantly difference from low-dose group on day 42.The content of hydroxyproline in high-dose and middle-dose HCl groups was also significantly higher than that in the control group.On day 42 hydroxyproline content in high-dose HCl dose rather middle –or low dose group was similiar with the level of bleomysin group.Content of TGF-β1 mRNA in three HCl groups was comparable to the level of bleomysin group on day 28 and exceeded on day 42.The expression of TGF-β1 in three HCl groups was not significantly different from the bleomysin group on day 42.Conclusion Experimental acid aspiration might contribute to pulmonary fibrosis in rats.Acid induced alveolar epithelial cell damage，abnormal proliferation and repair and fibrosis could be involved..
Trying to provide ultrasonic image-aid measures for quantitative diagnosis and dynamic monitoring of liver fibrosis, we propose two scoring methods for liver fibrosis tissue in vivo, based on ultrasound radio frequency (RF) time series in this paper. Firstly, RF echo signals of human liver were recorded in this study. Then one of the recorded frame RF data was demodulated to be B model image. After that, a region of interest (ROI) in the B model image was selected. For each point in the ROI, its all frame data were acquired so that RF time series were formed. An SMR (size measure relationship) fractal dimension and six spectral features were extracted from RF time series in the ROI. With relative deviation and Fisher's discriminant ratio, seven features were weighted and summed so that the liver tissues' scores were obtained, Score-rd and Score-fisher, respectively. Area under ROC curve (AUC) and a support vector machine (SVM) were used to evaluate whether these scoring methods would be useful in distinguishing normal and cirrhosis tissues. Experimental results are shown as follows: Score-rd's AUC was 0.843, while Score-fisher was 0.816, SVM classification accuracies were both up to 87.5%. This proved that our proposed scoring methods were effective in distinguishing normal and cirrhosis tissues. Score-rd and Score-fisher have potential for clinical applications. They can also provide quantitative references for liver fibrosis diagnosis.
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 explore the antioxidant effects of reduced glutathione on rat pulmonary fibrosis compared with traditional corticosteroid. MethodsOne-hundred and eight healthy SD rats were randomly divided into 6 groups,ie. a control group,a model group,a dexamethasone group,a low-dose glutathione group,a middle-dose glutathione group,and a high-dose glutathione group,with 18 rats in each group. The pulmonary fibrosis model was established by intratrachially instillation of bleomycin in all rats except the control group. The severity of lung fibrosis was evaluated by HE staining and Masson staining of collagen,and measurement of glutathione,hydroxyproline,superoxide dismutase (SOD),glutathion peroxidase (GSH-Px)in lung tissue homogenate by photocolorimetric method. ResultsOn 7th day and 14th day after bleomycin instillation, the severity of alveolitis in the model group,the dexamethasone group,and three glutathione intervention groups was significantly reduced compared with the control group (P<0.05). On 28 day, the severity of lung fibrosis was significantly reduced in the dexamethasone group and three glutathione intervention groups compared with the model group (P<0.05). On 7th day,lung glutathione content was significantly lower in the model group compared with the control group (P<0.05), significantly higher in the dexamethasone group and three glutathione intervention groups compared with the model group (P<0.05), significantly lower in the dexamethasone group and the low-dose glutathione group compared with the control group (P<0.05), and significantly higher in the high-dose glutathione group compared with the dexamethasone group (P<0.05). On 7th,14th,and 28th day,the hydroxyproline content in the dexamethasone group and three glutathione intervention groups decreased significantly compared with the model group (P<0.05). On 14th day,the hydroxyproline content in the middle-dose and high-dose glutathione groups was significantly lower than that in the dexamethasone group (P<0.05). SOD and GSH-Px were significantly reduced in the model group compared with the control group on all time points (P<0.05),but significantly increased after intervention by different doses of glutathione (P<0.05). ConclusionReduced glutathione can significantly reduce the degree of pulmonary fibrosis in rats,but has no obvious advantage over dexamethasone.
Objective To assess the effectiveness and safety of Jiuweirougan granule in the treatment of chronic hepatitis with hepatic fibrosis. Methods A double-blind, double dummy and randomized controlled method was adopted. Forty-six patients were selected by using the inclusion and exclusion criteria and were randomizedly allocated to the treatment group (n=23, Jiuweirougan 10 g, twice a day) and the control group (n=23, treated with Biejiaruangan 4 pills, three times a day). The duration of treatment for both groups lasted for 6 months. Results Two patients in the treatment group dropped out halfway through the study, while one patient in the control group was withdrawal for irregular treatment. Analysis according to intention-to-treat (ITT) and per-protocol (PP) was conducted. In the treatment group, the effective rates of TCM (Traditional Chinese Medicine) on the syndrome were 82.61% (19/23) (ITT) and 90.48% (19/21) (PP), while in the control group, there were 86.96% (20/23) (ITT) and 86.36% (19/20) (PP). There was no significant difference between the two groups (P＞0.05). The improvement rate of serum parameters in the treatment group such as hyaluronic acid (HA), type Ⅲ procollagen (PCⅢ), type Ⅳ collagewn (CⅣ) and laminin (LN) were 28.57% (6/21), 23.81% (5/21), 28.57% (6/21) and 4.76% (1/21), respectively, while in control group, the rates were 36.37% (8/22),13.64% (3/22), 36.37% (8/22) and 9.09% (2/22), respectively. No significant difference was seen between the two groups (P＞0.05). ITT and PP analysis revealed similar results. No obvious adverse effects were noted. Conclusion Jiuweirougan granule may improve chronic hepatitis with fibrosis, and its effect is equal to that of Biejiaruangan. No obvious toxic-adverse effects were seen.