Life In Idiopathic Pulmonary Fibrosis Patients Research Articles

Effect of Chest Physiotherapy on Quality of Life, Exercise Capacity and Pulmonary Function in Patients with Idiopathic Pulmonary Fibrosis: A Systematic Review and Meta-Analysis.

Idiopathic pulmonary fibrosis (IPF) is one of the most deleterious diseases of the pulmonary fibrosis spectrum. Its clinical presentation includes irreversible loss of lung function, and increasing cough, dyspnea and impaired quality of life. Chest physiotherapy can improve ventilation capacity, gas exchange, dyspnea, exercise capacity and quality of life. The aim of this study was to review the evidence about chest physiotherapy in IPF, specifically meta-analyzing quality of life, exercise capacity and pulmonary function. A wide search was conducted in PubMed, Embase, Cochrane and Web of Science for articles included until October 2023. PROSPERO Identifier: CRD42022333496. The Downs and Black scale and the Cochrane tool were employed to evaluate quality assessments and to assess the risk of bias. Data were pooled, and a meta-analysis was conducted. We selected 10 studies in which a chest physiotherapy program was performed with a total of 340 patients; of these, three articles were meta-analyzed. Significant effects in favor of chest physiotherapy were found for quality of life (MD = -8.60, 95% CI = -11.30, -5.90; p < 0.00001; I2 = 24%), exercise capacity (MD = 37.62, 95% CI = 15.10, 60.13; p = 0.001; I2 = 65%) and pulmonary function (MD = 7.86, 95% CI = 2.54, 13.17; p = 0.004; I2 = 80%). The systematic review showed significant results for the application of chest physiotherapy regarding pulmonary capacity, diffusion of gases and quality of life in IPF patients. The meta-analysis showed a significant improvement associated with applying chest physiotherapy in pulmonary function, exercise capacity and quality of life.

Pulmonary Rehabilitation for Exercise Tolerance and Quality of Life in IPF Patients: A Systematic Review and Meta-Analysis.

Objective The aim of this study is to evaluate the efficacy and safety of pulmonary rehabilitation (PR) in patients with idiopathic pulmonary fibrosis (IPF). Methods. Embase, PubMed, Cochrane Library, China National Knowledge Infrastructure (CNKI), Chongqing VIP (CQVIP), Wanfang Data, and Chinese Biomedical Literature Database (SinoMed) were comprehensively searched. Randomized controlled trials (RCTs) that investigated the effects of PR for IPF patients were included. Literature selection and data extraction were conducted by two review authors independently. The Cochrane Collaboration's Risk of Bias tool and RevMan software (version 5.3) were used to evaluate the quality of studies and conduct statistical analysis, respectively. Results. Seven studies (190 participants) were included. PR had a significant effect on six-minute walk distance (6MWD) (MD:48.60; 95%CI: 29.03 to 68.18; Z=4.87, P<0.00001), and 6MWD was improved more in subgroup analysis including studies conducted in Asia (MD: 53.62; 95%CI: 30.48 to 76.66; Z=4.54, P<0.00001) and Europe (MD:54.10; 95% CI: 26.65 to 101.56; Z=2.23, P=0.03). Forced vital capacity (FVC%) was higher (MD: 3.69; 95%CI: 0.16 to 7.23; Z=2.05, P=0.04). St. George's Respiratory Questionnaire (SGRQ)/IPF-specific SGRQ (SGRQ-I) total score was lower (MD: -7.87; 95% CI: -11.44 to -4.30; Z=4.32, P<0.0001). No significant effects were found for lung diffusing capacity determined by the single-breath technique (DLCO%) (MD: 3.02; 95%CI: -0.38 to 6.42; Z=1.74, P=0.08). Conclusions This study suggests that PR may enhance exercise capacity and improve quality of life in IPF patients. Besides, PR may also delay the decline of lung function of patients with IPF. However, further research should more fully assess the efficacy and safety of PR for IPF.

Mitochondria in the spotlight of aging and idiopathic pulmonary fibrosis.

Idiopathic pulmonary fibrosis (IPF) is a chronic age-related lung disease with high mortality that is characterized by abnormal scarring of the lung parenchyma. There has been a recent attempt to define the age-associated changes predisposing individuals to develop IPF. Age-related perturbations that are increasingly found in epithelial cells and fibroblasts from IPF lungs compared with age-matched cells from normal lungs include defective autophagy, telomere attrition, altered proteostasis, and cell senescence. These divergent processes seem to converge in mitochondrial dysfunction and metabolic distress, which potentiate maladaptation to stress and susceptibility to age-related diseases such as IPF. Therapeutic approaches that target aging processes may be beneficial for halting the progression of disease and improving quality of life in IPF patients.

Disease-specific Severity Measures and Health-related Quality of Life in Idiopathic Pulmonary Fibrosis

Objective: To elucidate the correlation between two disease-specific severity measures (CPI, composite physiologic index; GAP, gender, age, lung physiology variables) and health-related quality of life in patients with idiopathic pulmonary fibrosis (IPF). Methods: We used data from a previously reported observational cohort study using the Medical Outcome Study Short Form 36 (SF-36) for measuring health-related quality of life. Of the 44 patients with IPF who participated in the initial cross-sectional study, 32 patients participated in the follow-up study. The CPI and the GAP index were calculated at baseline and follow-up. Results: In the cross-sectional study, the CPI only correlated with one SF-36 domain and the GAP index did not correlate with any of the SF-36 domains. In the current longitudinal study (the median follow-up; 14 months), there was a significant increase in both indices: ΔCPI = 11.5 (95% confidence interval; 6.8, 16.1) and ΔGAP index = 0.59 (95% confidence interval; 0.25, 0.93). Within-subject changes in the CPI and the GAP index were significantly correlated with those of 5 and 3 subscales of the SF-36, respectively. Declines in 4 subscales of the SF-36 were significantly more severe in subjects whose CPI increased by ≥ 5 than in subjects whose CPI did not. Similarly, declines in 3 subscales were significantly more severe in subjects whose GAP stage increased than in subjects whose GAP stage did not. Conclusion: Serial changes in the CPI and the GAP index may be useful to predict changes in the healthrelated quality of life of IPF patients.

Idiopathic pulmonary fibrosis: diagnostic pitfalls and therapeutic challenges

Idiopathic pulmonary fibrosis (IPF), the most common of the idiopathic interstitial pneumonias, is a devastating condition that carries a prognosis worse than that of many cancers. As such, it represents one of the most challenging diseases for chest physicians. The diagnostic process is complex and relies on the clinician integrating clinical, laboratory, radiologic, and/or pathologic data. Therefore, a close collaboration between chest physicians, radiologists, and pathologists experienced in the diagnosis of interstitial lung diseases (ILDs) is necessary in order to minimize diagnostic uncertainty. Similarly, the management of IPF continues to pose major difficulties. However, while there are no proven effective therapies for IPF beyond lung transplantation, recent trials of novel agents suggest that pharmacological treatment may retard the progression of the disease. In this regard, enrolment of patients into clinical trials is considered the “best current practice”by the most recent guidelines as it offers IPF patients the chance to receive new agents that may be more effective than current therapies. A more recent trend focusing on improving quality of life in IPF patients has also been gaining ground. The diagnosis and management of IPF remains a constant challenge for even the most experienced of clinicians. However, a multidisciplinary approach to this complex disease is steadily improving diagnostic accuracy, while recent advances in the pharmacological therapy offer the genuine promise of future treatments for this devastating disease.

Idiopathic pulmonary fibrosis: diagnostic pitfalls and therapeutic challenges

Idiopathic pulmonary fibrosis (IPF), the most common of the idiopathic interstitial pneumonias, is a devastating condition that carries a prognosis worse than that of many cancers. As such, it represents one of the most challenging diseases for chest physicians. The diagnostic process is complex and relies on the clinician integrating clinical, laboratory, radiologic, and/or pathologic data. Therefore, a close collaboration between chest physicians, radiologists, and pathologists experienced in the diagnosis of interstitial lung diseases (ILDs) is necessary in order to minimize diagnostic uncertainty. Similarly, the management of IPF continues to pose major difficulties. However, while there are no proven effective therapies for IPF beyond lung transplantation, recent trials of novel agents suggest that pharmacological treatment may retard the progression of the disease. In this regard, enrolment of patients into clinical trials is considered the “best current practice”by the most recent guidelines as it offers IPF patients the chance to receive new agents that may be more effective than current therapies. A more recent trend focusing on improving quality of life in IPF patients has also been gaining ground.The diagnosis and management of IPF remains a constant challenge for even the most experienced of clinicians. However, a multidisciplinary approach to this complex disease is steadily improving diagnostic accuracy, while recent advances in the pharmacological therapy offer the genuine promise of future treatments for this devastating disease.

Non-pharmacological treatment of idiopathic pulmonary fibrosis

Idiopathic pulmonary fibrosis (IPF) is a chronic progressive fatal disorder that remains difficult to treat. In this review, we examine non-pharmacological treatment modalities, including lung transplantation, pulmonary rehabilitation and palliation. Lung transplantation, the only therapeutic intervention that offers survival benefit, should be considered in all IPF patients with progressive disease who meet the International Society for Heart and Lung transplantation guidelines. Pulmonary rehabilitation improves exercise capacity, reduces dyspnoea and improves quality of life in IPF patients, and should be made available to patients. For those patients with advanced disease, palliative services offer symptom management, improved quality of life and psychological support for patients and their caregivers.

Treatment of idiopathic pulmonary fibrosis: Is there anything new?

Idiopathic pulmonary fibrosis (IPF) is a specific form of chronic fibrosing interstitial pneumonia of unknown aetiology and is associated with the histological picture of usual interstitial pneumonia. Treatment in most cases is unsatisfactory and the prognosis remains poor. There is insufficient evidence to suggest that any treatment, apart from lung transplantation, improves survival or halts disease progression for IPF patients. Data on treatment response are limited by the paucity of clinical trails, the lack of homogenous clinical features, the small number of patients, and the absence of histological and radiological documentation in many cases. Anti-inflammatory medications such as corticosteroids, azathioprine and cyclophosphamide remain the commonly used medications. More recently, it has been proposed that IPF is a primary fibrotic disease rather than an inflammatory condition. Antifibrotic agents such as colchicine, pirfenidone and interferon-gamma (IFN-gamma) have been tried. However, a recent placebo-controlled trial has failed to demonstrate a significant effect of IFN-gamma on disease progression, lung function or quality of life in IPF patients, though a clinically significant survival benefit of the drug could not be ruled out.