Connective Tissue Diseases and Lung Manifestations

Despite a number of prospective studies already initiated in the past years, the current epidemiology and course of interstitial lung disease (ILD) and pulmonary hypertension (PH) in patients with connective tissue disease (CTD) is still not well defined, particularly regarding its prevalence, incidence and the management of a broad spectrum of disease presentations. Major challenges include the identification of patients with progressive disease, the appropriate time point of therapeutic intervention and the underlying driver of disease (inflammatory or pro-fibrotic stimulus or both?). To address these issues in Western Austria, a progressive registry of patients with CTD exploring routine clinical and pathophysiological characteristics of ILD and PH will be conducted. This multidisciplinary, prospective and observational registry aims to collect comprehensive clinical data on incidence, prevalence and course of disease regarding all PH and ILD presentations in a real-world setting.

NCT Number	NCT04095351
Lead Sponsor	Medical University Innsbruck Collaborators: Boehringer Ingelheim
Conditions	Connective Tissue Diseases, Interstitial Lung Disease, Systemic Sclerosis
Enrollment	120 participants
Start Date	2019-12-09
Primary Completion	2021-03-01 (estimated)
Study Completion	2034-10-01 (estimated)
Updated on ClinicalTrials.gov	2020-05-27