Generation of a common European database and biobank Continous assessment and implementation of guidelines and treatment protocols Establishment of a large observational cohort of chILD patients Determination the value of outcomes used in child Assess treatment variations used, deliver data from defined protocols and linked outcomes
Immunosuppressive therapy of granulomatosis with polyangiitis (GPA, Wegener's) and microscopic polyangiitis (MPA) has transformed the outcome from death to a strong likelihood of disease control and temporary remission. However, most patients have recurrent relapses that lead to damage and require repeated treatment associated with long-term morbidity and death. Rituximab has been shown to be as effective as cyclophosphamide to induce remission and maintenance of remission in severe GPA and MPA patients, with an acceptable safety profile . Although rituximab is becoming the standard of care for maintenance therapy in these patients,...
This is a Phase 3 trial to evaluate the efficacy and safety of 30 mg/kg intravenous (IV) infusions of pamrevlumab administered every 3 weeks as compared to placebo in subjects with Idiopathic Pulmonary Fibrosis
A Phase 2a, multicenter, 3-part, randomized, double-blind, dose-ranging, placebo-controlled study to evaluate the safety, tolerability, and PK of once-daily treatment with PLN-74809 in participants with idiopathic pulmonary fibrosis.
The purpose of the study is to evaluate the efficacy on lung function 6 months after one course of rituximab (2 infusions) and mycophénolate mofétil (MMF) treatment compared to one course of placebo and 6 months of MMF treatment in a broad range of patients with Interstitial Lung Diseases (ILD) non-responders to a first line immunosuppressive treatment.
Current diagnostic tools used in pulmonary disease often do not meet the challenges set by the respective pathophysiology. The investigators therefore aimed to evaluate novel or not widely used diagnostic approaches for the detection and therapeutic monitoring of patients with various pulmonary diseases.
Current diagnostic tools used in interstitial lung disease (ILD) do not meet the challenges set by the complex pathophysiology of this heterogenous group. The investigators therefore aimed to evaluate novel or not widely used diagnostic approaches for the detection and therapeutic monitoring of patients with various ILDs.
Systemic Scleroderma (SCS) is an autoimmune disease characterized by vascular involvement, a dysimmune condition, cutaneous and visceral fibrosis. Interstitial lung disease (ILD) affects 75% of SSc patients and is the leading cause of death in SSc. No diagnostic or prognostic biomarkers of SSc-associated ILD have been validated to date. The search for such a serum biomarker is essential to assess the severity of these patients and to help the therapeutic management. We have shown that soluble fractalkine is elevated in SSc patients, especially in SSc patients with ILD. The fractalkine is both an endothelial adhesion molecule and a...
The study evaluates the rest and exercise hemodynamics of patients presenting either idiopathic fibrotic pulmonary disease or pulmonary fibrosis secondary to connective tissue disease.
The goal of this study is to use the tissues from the explanted lungs in order to better study the cause of pulmonary fibrosis at a cellular level.