The effect of Baduanjin exercises on the functional capacity, respiratory function, and quality of life of patients with idiopathic pulmonary fibrosis: a randomized controlled trial

From Anti-inflammatory Drugs Through Antifibrotic Agents to Lung Transplantation: A Long Road of Research, Clinical Attempts, and Failures in the Treatment of Idiopathic Pulmonary Fibrosis

Familial pulmonary fibrosis is the strongest risk factor for idiopathic pulmonary fibrosis

Background: The entity of idiopathic pulmonary upper lobe fibrosis (IPUF) was first described in Japan before the disease entity of pleuroparenchymal fibroelastosis (PPFE) was reported［1］. It is now recognized that IPUF and PPFE are similar entities. No reports have compared clinical characteristics of these disease entity and idiopathic pulmonary fibrosis (IPF). Methods: Fifteen patients with IPUF, and 61 patients with idiopathic pulmonary fibrosis (IPF) were retrospectively enrolled. Baseline clinical characteristics, erector spinae muscles assessed by computed tomography (CT)［2］, and prognosis were compared. Furthermore, Cox proportional hazards analyses were performed in both group. Results: Body mass index was significantly lower in IPUF than in IPF (P Conclusions: IPUF have the characteristics of leanness, but may have much muscle mass, compared with IPF. The prognosis of IPF was poorer as compared with that of IPUF. In the IPUF group, lower erector spinae muscles mass by CT was a prognostic factor.

Objective To investigate the effect of a high dose of N-acetylcystine on pulmonary fibrosis and oxidative stress in patients with idiopathic pulmonary fibrosis. Methods 68 patients with idiopathic pulmonary fibrosis treated at our hospital from May, 2016 to May, 2018 were randomly divided into a control group (n=34) and an observation group (n=34). The control group was treated with a low dose of N-acetylcystein, and the observation group a high dose. The indicators of pulmonary fibrosis and oxidative stress, cytokines, and adverse reactions were compared between the two groups. Results After the treatment, the levels of type Ⅲ and Ⅳ collagens, hyaluronic acid, malondialdehyde, transforming growth factor-β, vascular cell adhesion molecule-1, and IL-13 were lower and the levels of superoxide dismutase and glutathione were higher in the observation group than in the control group [(69.17±7.84) ng/ml vs. (84.16±8.38) ng/ml, (89.73±11.67) ng/ml vs. (106.42±10.81) ng/ml, (87.92±22.67) ng/ml vs. (110.07±21.76) ng/ml, (9.75±1.36) nmol/L vs. (13.24±1.79) nmol/L, (102.54±13.51) pg/ml vs. (121.78±14.13) pg/ml, (311.14±12.91) pg/ml vs. (320.16±13.28) pg/ml, (13.91±1.73) ng/L vs. (17.65±1.42) ng/L, (81.97±6.01) IU/L vs. (72.13±5.76) IU/L, and (46.85±6.94) μmol/L vs. (34.63±7.29) μmol/L; all P 0.05). Conclusion A high dose of N-B-cysteine for patients with idiopathic pulmonary fibrosis can effectively improve the pulmonary fibrosis and the level of oxidative stress, reduce the levels of cytokines, and contribute to the prognosis. Key words: Idiopathic pulmonary interstitial fibrosis; N-acetylcysteine; Pulmonary fibrosis; Oxidative stress; Cytokines

Pulmonary rehabilitation for interstitial lung disease.

Introduction: Idiopathic interstitial pulmonary fibrosis is an important debilitating disease among the interstitial lung diseases, frequently diagnosed with the help of HRCT among the symptomatics. Materials and Methods: We have studied a total of 48 cases of IPF over a span of two and half years. Among a total of 104 ILDs attending our tertiary care centre IPF constituted 48 cases accounting for 46%. IPF cases are diagnosed on the basis of clinical history and hRCT findings. Results: There is a female preponderance of 1.4:1. Mean age of IPF patients in our study is 57.9 years. Mean age in females is 60.71 and 53.9 in males.Among our 48 cases of radiologically diagnosed IPF forty patients had definite UIP Pattern and 8 patients have possible UIP pattern as defined by the radiologist. The frequency of difinite IPF cases increased with severity of symptoms as measured by MMRC scale and the association is statistically significant. More than 90% of patients expressed tiresomeness and breathlessness. Cough was seen in 60% and pain chest was found in 45% of IPF patients. There is a statistically significant correlation of disease severity measured by MMRC scale with decrease in DLCO. BAL fluid lavage showed predominant neutrophils in >90% of IPF patients. Severity of IPF measured by MMRC correlated with decrease in 6 minute walk distance(p Summary and Conclusion: Idiopathic interstitial pulmonary fibrosis is a debilitating disease. Constitutes significant proportion of ILDs. Females outnumbered males. Tiresomeness and breathlessness are predominat symptoms. Disease occurred at a much earlier age in males. Active and passive smoking possibly contributes to the severity of IPF. MMRC grade correlates significantly with DLCO, 6 minute walk distance and definite UIP. There is no statistically significant correlation of MMRC grade with severity of PAH and FVC values. Keywords: Interstitial Lung Disease (ILD); Idiopathic Pulmonary Fibrosis (IPF); MMRC Grade, Desaturation; Br

Keywords: interstitial lung disease; lung function; paediatric lung disease; pulmonary vascular disease; sleep

Objectives: The 6-minute walk test (6MWT) is generally used to evaluate endurance in interstitial lung disease (ILD). In ILD, treatment efficacy differs between patients with idiopathic pulmonary fibrosis (IPF) and those with non-idiopathic pulmonary fibrosis (non-IPF), and the clinical profile varies according to disease severity. This study compared 6MWT parameters by severity of illness in patients with IPF and non-IPF. Methods: The participants were hospitalized patients with ILD and either IPF (n = 20) or non-IPF (n = 25). To compare IPF and non-IPF by severity, patients were classified using the gender–age–physiology (GAP) index as having GAP stage I (i.e., mild) or GAP stage II/III (i.e., moderate to severe). The evaluation parameters were the 6-minute walk distance (6MWD), minimum percutaneous oxygen saturation (SpO2), heart rate recovery at 1 minute (HRR1) after the 6MWT, and modified Borg scale rating of perceived exertion. Results: The minimum SpO2 value during exertion was significantly lower in the IPF group than in the non-IPF group (p < 0.05). Furthermore, when comparing by severity, patients with IPF and GAP stage I had a significantly lower 6MWD, HRR1, and SpO2 value. In patients with GAP stage II/III, there were no significant differences in 6MWD, SpO2 values, or dyspnea. Conclusions: Patients with mild IPF were more prone to hypoxemia, while in moderate-to-severe cases, we observed no significant hypoxemia- or endurance-related differences between IPF and non-IPF patients. This study highlights the importance of severity-based evaluation, particularly in guiding individualized rehabilitation and risk management for patients with IPF.

Medicine and Me: a breath of fresh air for IPF

Effects of Lecithinized Superoxide Dismutase and/or Pirfenidone Against Bleomycin-Induced Pulmonary Fibrosis

Background/Objectives: Idiopathic pulmonary fibrosis (IPF) is the most common form of pulmonary fibrosis (PF) and serves as a key reference for disease severity. Progressive pulmonary fibrosis (PPF), a distinct yet heterogeneous entity arising from various interstitial lung diseases (ILDs), shares similar pathogenetic mechanisms and clinical courses driven by self-perpetuating fibrosis. Antifibrotic therapy, notably nintedanib, can slow disease progression. However, real-world data on antifibrotic therapy’s impact on survival, especially in PPF, are limited. This study aims to compare IPF and PPF regarding phenotype, radiological patterns, comorbidities, prognostic factors, and response to nintedanib, focusing on identifying the patient subsets most likely to benefit. Outcomes assessed include safety, survival, and disease progression over one year, considering various prognostic factors. Methods: This retrospective observational study evaluated patients with fibrosing ILD, affected by either IPF or PPF, and treated with nintedanib. Data collected encompassed clinical, radiological, functional, and treatment-related information. Assessments included chest CT, pulmonary function tests, comorbidities, and survival analysis, utilizing standardized methods and statistical tools to interpret outcomes and tolerability. Results: The study population was composed of 97 patients: 64 were diagnosed with IPF and 33 with PPF. The analysis showed that in PPF patients, ongoing antifibrotic treatment resulted in higher survival (71.1 months vs. 27.4 months, p < 0.001), while no statistically significant differences were found in the IPF group (67.4 months vs. 52.5 months, p = 0.216). Nintedanib was generally well tolerated. Gastrointestinal side effects, predominantly diarrhea, were reported in 61% of patients with IPF and 50% of those with PPF. Dose reduction occurred in 43.75% of IPF patients and 36% of PPF patients, while treatment discontinuation was required in 21.87% of IPF and 21% of PPF patients. Conclusions: This study highlights that in PPF patients, antifibrotic therapy with nintedanib can improve survival. This statement underlines that the primary outcome of antifibrotic treatment should focus on improving patients’ survival.

BackgroundInterstitial lung diseases (ILDs) significantly impair health-related quality of life (HRQL). While HRQL in idiopathic pulmonary fibrosis (IPF) is well studied, data on hypersensitivity pneumonitis (HP) remain limited despite comparable clinical impacts. This study evaluates HRQL in patients with IPF and HP using the King’s Brief Interstitial Lung Disease Questionnaire (K-BILD) and EuroQoL 5-Dimension 5-Level Questionnaire (EQ-5D-5L) and examines factors associated with reduced HRQL.MethodsThis longitudinal, observational study included 153 patients [48 IPF, 105 HP, including 72 fibrotic HP (fHP), of whom 32 had usual interstitial pneumonia (UIP) pattern (fHP + UIP)]. Demographic, anamnestic and clinical data, HRQL, respiratory function, echocardiography parameters, dyspnea [modified Medical Research Council (mMRC) dyspnea scale], cough [visual analog scale (VAS scale), Leicester Cough Questionnaire (LCQ)], six-minute walk test (6MWT) results, and high-resolution computed tomography (HRCT) changes were assessed at baseline and after 12 months. Statistical analyses included non-parametric tests, correlation, regression, and receiver operator characteristic (ROC)-curve evaluation to identify factors associated with reduced HRQL.ResultsIPF patients demonstrated more severe dyspnea and reduced exercise capacity compared to HP, while overall lung function parameters and cough-related measures showed comparable results between the groups. Within the HP cohort, fibrotic forms were associated with greater symptom burden, more advanced physiological impairment, longer diagnostic delay. A statistically significant relationship between the K-BILD and the EQ-5D-5L was found. HRQL assessed by the K-BILD questionnaire did not differ significantly between patients with IPF and those with HP. In contrast, EQ-5D-5L scores were significantly lower in IPF compared to HP, although no significant differences were observed between IPF and fHP. Both questionnaires demonstrated significant differences in HRQL between fHP and nfHP, while no differences were found between fHP patients with and without UIP pattern. Additionally, HRQL measured by K-BILD was associated with gender-age-physiology (GAP) stage in both IPF and HP cohorts. HRQL according to the K-BILD was mainly associated with cough severity (LCQ), 6-minute walk distance (6MWD), dyspnea intensity (mMRC) and lung function parameters [forced vital capacity (FVC)]. For the EQ-5D-5L, the main predictors of lower HRQL were reduced exercise capacity (6MWD) and higher cough burden (LCQ). According to the results of ROC analysis, the predictors of HRQL score less than 50 points (K-BILD): cough score <13 points (LCQ) and dyspnea score of ≥3 (mMRC).ConclusionsPatients with fHP, especially with UIP pattern, exhibit similar clinical features and functional decline as seen in IPF patients. Cough severity, dyspnea, and exercise capacity were the strongest predictors of reduced HRQL, highlighting the need for comprehensive symptom assessment and individualized management in both IPF and HP. K-BILD and EQ-5D-5L are reliable tools for assessing HRQL and identifying significant clinical and functional factors.

Introduction: Idiopathic pulmonary fibrosis (IPF) is a chronic, devastating, interstitial lung disease, with few therapeutic options. IPF is characterized by pulmonary restriction, dyspnea, hypoxemia, exercise intolerance and poor quality of life (QOL). Aims and objective: We aimed to examine the effect of exercise training (ET) on clinical outcomes and on quality of life and symptoms in IPF patients. Methods: Subjects who met current criteria for IPF were randomized to a 3-month pulmonary rehabilitation program (n _ 14) or to a control group (n _ 10). The rehabilitation group participated in twiceweekly, 90-min exercise sessions (24 total sessions). The control group maintained its preceding normal physical activity. 6-min walking distance (6MWD) test, 30-second chair-stand test, pulmonary function tests, dyspnea and QOL were assessed at baseline and at the end of the 12-week intervention. The SGRQ-I and a 5-point self-assessment of health were completed at baseline, after 3 months of intervention or observation. Results: Subjects in the rehabilitation group maintained significantly higher levels of physical activity throughout the 3-month rehabilitation program. SGRQ-I symptom domain scores improved considerably by _9 _ 22 in the rehabilitation group, whereas in the control group they worsened Significant differences were observed between the ET and the control groups in raw mean: Δ6MWD, 94 m, p Conclusion: ET improves exercise tolerance, functional capacity, pulmonary function, dyspnea and QOL in patients with IPF and should be considered the standard care for IPF.

Idiopathic pulmonary fibrosis (IPF) is a progressive, fatal interstitial lung disease (ILD) with an unpredictable clinical course. Although IPF is rare, healthcare professionals should consider IPF as a potential cause of unexplained chronic dyspnea and/or cough in middle-aged/elderly patients and refer patients to a pulmonologist for evaluation. Making a diagnosis of IPF requires specialist expertise. Multidisciplinary discussion, involving at minimum a pulmonologist and a radiologist with expertise in the differential diagnosis of ILDs, is required to ensure the most accurate diagnosis. Prompt diagnosis of IPF is important to enable patients to receive appropriate care from an early stage. Optimal management of IPF involves the use of antifibrotic drugs, as well as the provision of supportive care to alleviate symptoms and preserve patients’ quality of life. Antifibrotic drugs have been shown to slow lung function decline seen in patients with IPF. Patients’ symptoms and functional capacity can be improved through participation in pulmonary rehabilitation programs and the use of supplemental oxygen. Patient education is essential to help patients understand and manage their disease. The identification and management of comorbidities, such as obstructive sleep apnea, pulmonary hypertension, and emphysema, is also an important element of the overall care of patients with IPF. Patients with IPF should be evaluated for lung transplantation at an early stage to maximize their chances of meeting eligibility criteria. In this review, we describe the clinical course and impact of IPF and best practice in its management, highlighting the importance of taking a patient-centered approach.

BackgroundFollowing COVID-19 infection, some patients acquired lung injury and fibrosis. Idiopathic pulmonary fibrosis is characterized by lung fibrosis. Both post-COVID lung injury and idiopathic pulmonary fibrosis cause loss of respiratory function and involvement of the lung parenchyma. We aimed to compare respiratory related functional characteristics and radiological involvement between post-COVID lung injury and idiopathic pulmonary fibrosis.MethodsA single center, cross-sectional study was applied. Patients with post-COVID lung injury and idiopathic pulmonary fibrosis included in the study. All patients underwent the 6-minute walk test, as well as the Borg and MRC scales. Radiological images were evaluated and scored for lung parenchymal involvement. The impact of post-COVID lung injury and idiopathic pulmonary fibrosis on respiratory functions of were compared. The relationship of functional status and radiological involvement, as well as the effect of potential confounding factors were investigated.ResultsA total of 71 patients were included in the study. Forty-eight (67.6%) of the patients were male and the mean age was 65.4 ± 10.3 years. Patients with post-COVID lung injury had greater 6-minute walk test distance and duration, as well as higher oxygen saturations. The MRC and Borg dyspnea scores were comparable. At radiologic evaluation, ground glass opacity scores were higher in patients with post-COVID lung injury, whereas pulmonary fibrosis scores were higher in patients with idiopathic pulmonary fibrosis. However, the total severity scores were similar. While pulmonary fibrosis score was found to have a negative correlation with 6-minute walk test distance, test duration, and pre- and post-test oxygen saturation levels, there was a positive correlation with oxygen saturation recovery time and MRC score. There was no relationship between ground glass opacity and the functional parameters.ConclusionsDespite having equal degrees of radiological involvement and dyspnea symptom severity, PCLI patients exhibited higher levels of functional status. This might be due to different pathophysiological mechanisms and radiological involvement patterns of both diseases.