The Pulmonary Fibrosis Foundation Patient Registry will collect data on at least 2,000 patients with interstitial lung disease (ILD) at approximately 40 clinical sites in the US. The Registry is targeting enrollment of approximately 60% of the 2,000 ILD participants to have idiopathic pulmonary fibrosis (IPF). The aim of the Registry is to create a cohort of well-characterized patients with interstitial lung disease (ILD) for participation in retrospective and prospective research
This is an interventional double-blind randomized controlled trial, to investigate the short and long-term effects of a supervised exercise training program in patients with IPF, depending on alternate patterns of oxygen supplementation during PR.
The objective of the current research project is, by using high quality Swedish registry data, to evaluate use, tolerance and effect of anti-fibrotic drugs in IPF-patients. Secondary study objectives are to determine the clinical profile, determinants of treatment adherence, long-term safety and to describe the patient journey from the first sign of disease to end of treatment.
Respiratory muscle strength, dyspnea perception, physical activity and quality of life measurements will be performed and groups will be compared in two groups consisting of patients with idiopathic pulmonary fibrosis referred to pulmonary rehabilitation clinic and healthy volunteers in similar age range.
The overall goal of this study is to define the phenotype of Interstitial Lung Disease (ILD) in patients with rheumatoid arthritis (RA). The investigators hypothesize that there are common elements between other forms of ILD such as idiopathic pulmonary fibrosis (IPF) and sub-clinical RA-ILD that places individuals at risk for the development of lung disease. This is not a treatment study. It is a protocol designed to enroll individuals affected by RA and explore associated lung disease so that the investigators can better understand the clinical phenotype and genetic and molecular endotypes of this disease.
Mechanistic study to assess whether dual B-cell immunotherapy by co-administration of rituximab and belimumab will result in improvements in biological endpoints, functional outcomes and clinical status compared to rituximab with placebo.
Phase III, comparative, multicenter, randomized, controlled, double-blind and superiority research, comparing rituximab-based regimen with conventional therapeutic strategy for the induction of remission in patients with eosinophilic granulomatosis with polyangiitis (EGPA). Patients with newly diagnosed or relapsing EGPA will be randomized in a 1:1 ratio to receive: - Experimental therapeutic strategy based on the use of rituximab (experimental group) - Conventional therapeutic strategy based on Five-Factor Score (FFS)-assessed disease severity (comparative group)
Interstitial lung disease (ILD) is characterised by inflammation and scarring of the lung and is the leading cause of death in patients with systemic sclerosis, and contributes significantly to morbidity and mortality in many other connective tissue diseases (CTDs) such as polymyositis/dermatomyositis and mixed connective tissue disease. When ILD is extensive and/or progressive, immunosuppressive medication is often required to stabilize lung disease and alleviate symptoms. Current standard care for CTD associated ILD is extrapolated from studies performed in individuals with systemic sclerosis and comprises low dose corticosteroids and...
The purpose of this study is to investigate inherited genetic factors that play a role in the development of familial pulmonary fibrosis and to identify a group of genes that predispose individuals to develop pulmonary fibrosis. Finding the genes that cause pulmonary fibrosis is the first step at developing better methods for early diagnosis and improved treatment for pulmonary fibrosis. The overall hypothesis is that inherited genetic factors predispose individuals to develop pulmonary fibrosis.
This is a multicenter, randomized (1:1 inhaled treprostinil: placebo), double-blinded, placebo-controlled trial to evaluate the safety and efficacy of inhaled treprostinil in subjects with pre-capillary pulmonary hypertension (PH) associated with interstitial lung disease (ILD) including combined pulmonary fibrosis and emphysema (CPFE). The study will include about 314 patients at approximately 120 clinical trial centers. The treatment phase of the study will last approximately 16 weeks. Patients who complete all required assessments will also be eligible to enter an open-label, extension study (RIN-PH-202).