View clinical trials related to Idiopathic Pulmonary Fibrosis.
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By mean of registry of newly diagnosed Chinese IPF patients from more than 15 sites, this study aims to build IPF prospective cohort, set up normative clinical database and a biological specimen bank, and examine the clinical characteristics of newly diagnosed Chinese IPF patients, as well as the nature history, prognosis, comorbidities and complications of IPF patients in China, the current treatment pattern, burden of illness, and quality of life of Chinese IPF patients.
Idiopathic pulmonary fibrosis (IPF) is a fibroproliferative, irreversible disease of unknown cause, occurring mainly in patients older than 50. IPF is a rare but fatal lung disease, with an estimated prevalence of 14 to 28/100000 and a median survival time of 3 years. Acute exacerbation of IPF (AE-IPF) is a major event of IPF, as it is responsible for the death of 30-50 % of IPF patients; its annual incidence varies between 5 and 10%. The current literature indicates that IPF is associated with the development of an auto-immunity process targeting epithelial and endothelial lung cells. Autoantibodies have been associated with a poorer prognosis. A study by DONAHOE et al. (Plos One, 2015) indicates that the combination of corticosteroids, plasma exchanges, rituximab and immunoglobulins may improve the prognosis of the most severe forms of AE-IPF. In that study, the observed survival rate in patients receiving this combination of treatment was 70% as compared with 20% in historical controls. This therapeutic combination approach is designed both to eliminate and inhibit the production of circulating antibodies targeting the lungs. Considering the high mortality rate of an AE-IPF episode and the potential benefit of such an original approach, a well-conducted randomized controlled trial is critical.
Introduction Idiopathic Pulmonary Fibrosis (IPF) is a progressive fibrotic lung disease of unknown cause with a median survival of 3-5 years. As the disease progresses, worsening of lung function and gas exchange impairment cause hypoxemia during physical activity leading to a downward spiral; dyspnea and fatigue lead to a reduction in daily physical activities, exercise tolerance, muscle strength and quality of life. Problems reported by IPF patients are social isolation, increased level of dependency and immobility. There is no curative treatment for IPF, but pulmonary rehabilitation (PR) is recommended by expert opinion for the majority of IPF patients in order to improve quality of life and exercise tolerance. There are, however, no official PR programs for IPF patients and they therefore either participate in PR programs patients with chronic obstructive lung diseases (COPD) or train by themselves in fitness centers without guidance. PR for COPD is mostly offered in specialized clinics with an average duration of 8 weeks. Not all patients with IPF are fit for these programs or want to participate in an extensive external program due to, among other reasons, distance to the clinic. Tele-rehabilitation may offer these patients an alternative. New technologies in healthcare that can treat patients from a distance are implemented in these years. Tele-rehabilitation has been shown to be feasible in patients with lymphedema, COPD, orthopedic diseases (lower back, knee and shoulder). To ensure that all IPF patients are offered the possibility to participate in IPF specific rehabilitation programs, even though they live far away from expert ILD centers, tele-rehabilitation might be an alternative to participation in COPD rehabilitation programs. There have so far been no studies on the feasibility effect of tele-rehabilitation in IPF. Aim To assess the feasibility and effect of tele-rehabilitation with a new and innovative TR platform (NITRP) compared to standard treatment with respect to exercise capacity, quality of life and activities of daily living in patients with IPF. Method & material The study is a prospective randomized controlled trial comparing the effects of tele-rehabilitation and standard treatment in patients with IPF. 24 patients with IPF will be randomized in two groups, and the intervention group trained by tele-rehabilitation for 12 weeks with follow-up after training at 3 and 6 months. The control group will follow the usual control program for IPF patients that only involves outpatient visits approximately every 3rd month. The intervention group will recieve tele-rehabilitation.in the form of video consultations- and chat sessions with a real physiotherapist and workout sessions with a virtual physiotherapist agent. They will also train with virtual reality glasses or tablets that show the actual exercises in the training program. Patients will be tested with pulmonary function parameters, 6 minute walk test, a pedometer registering steps walked in 7 days, King's brief ILD questionnaire (a disease specific quality of life questionnaire), the General Anxiety Disorder Score (GAD-7) (measures the presence and severity of general anxiety disorder) and the SGRQ-IPF, a disease specific, self-administered questionnaire for IPF, all at baseline, after 12 weeks of intervention, 3 and 6 months after cessation of the program
The goal of this study is to investigate the safety of [68Ga]CBP8 and its efficacy to detect collagen deposition in pulmonary fibrosis.
The purpose of this study is to determine whether a lung transplantation prior to bone marrow transplantation (BMT) would allow for restoration of pulmonary function prior to BMT, allowing to proceed to BMT, to restore hematologic function.
This study seeks to screen first degree family members of people with Idiopathic Pulmonary Fibrosis (IPF) for the earliest signs of lung fibrosis.
This a prospective, longitudinal study of first-degree family members of patients diagnosed with familial interstitial pneumonia (FIP). FIP is the familial form of idiopathic pulmonary fibrosis (IPF), which is defined as 2 or more bloodline relatives which have a diagnosis of idiopathic interstitial pneumonia (IIP). The most common form of idiopathic interstitial pneumonia in FIP families is IPF (approximately 70%). The inheritance pattern in FIP is consistent with autosomal dominant inheritance with incomplete penetrance. Therefore, individuals in this study have approximately 50% risk of carrying a disease-associated allele. The causative gene is currently only known approximately 20% of families. The main goal of this longitudinal study is to better establish the natural history of FIP and to identify risk factors for later development of symptomatic disease. The investigators' plan is to follow these at-risk individuals with yearly questionnaires and planned in person 2 year follow-ups through age 75 or until they develop symptomatic FIP.
Idiopathic pulmonary fibrosis (IPF) is a chronic and potentially fatal lung disease. As IPF progresses, patients become increasingly breathless with reduced exercise capacity and quality of life. A cardiopulmonary exercise test (CPET) is a gold standard way of assessing patients with IPF. An incremental shuttle walk test is simpler, cheaper, more widely available and anecdotally preferable to CPET. The investigators will compare the measurements made in an ISWT and a CPET in patients with IPF . We aim to determine whether sufficient information can be gathered in an ISWT to negate the need to undertake CPET.
Acute exacerbations (AE) are a dreaded manifestation of idiopathic pulmonary fibrosis (IPF) that presents with rapidly worsening respiratory function over days to weeks. AE account for about 1/2 the deaths in IPF patients, and are refractory to all medical therapies attempted to date. Considerable preliminary data shows pathological B-cell abnormalities and autoantibodies are present in AE-IPF and associated with disease severity. The experimental therapy here (therapeutic plasma exchange plus rituximab plus intravenous immunoglobulin) is mechanistically targeted to ameliorate autoantibody-mediated pulmonary injury. Anecdotal pilot studies indicate these treatments have significant benefit for a disease syndrome that has, until now, been almost invariably inexorable. This clinical trial has the potential to profoundly affect current paradigms and treatment approaches to patients with AE-IPF.