Connective Tissue Diseases and Lung Manifestations

NCT ID: NCT04095351

Last Updated: 2020-05-27

Basic Information

• Recruitment Status: RECRUITING
• Total Enrollment: 120 participants
• Study Classification: OBSERVATIONAL
• Study Start Date: 2019-12-09
• Study Completion Date: 2034-10-01

Brief Summary

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.

Detailed Description

Specifically, this registry will collect demographic data, disease-related clinical data, routine laboratory values (including antibody-profile and iron status), diagnostic procedures, significant comorbidities, therapeutic managements (e.g. thoracic ultrasound, HRCT), and disease outcomes over 10 years.

In addition, blood biobank samples for translational research will be collected in a subgroup of patients with systemic sclerosis over serial time points to study the systemic inflammatory and profibrotic phenotype of patients. In summary, this registry will monitor the disease course of pulmonary manifestations of patients with CTD and may be hypothesis-generating and provide new insights in underlying inflammatory/pro-fibrotic patterns.

Conditions

Connective Tissue Diseases; Interstitial Lung Disease; Systemic Sclerosis

Study Design

• Observational Model Type: OTHER
• Study Time Perspective: PROSPECTIVE

Interventions

Pulmonary function test

Spirometry or plethysmography, measurement of diffusion capacity

Intervention Type: DIAGNOSTIC_TEST

Imaging

Thoracic ultrasound, HRCT as scheduled within routine clinical examinations, echocardiography

Intervention Type: DIAGNOSTIC_TEST

Blood sampling

Standard laboratory test for CTDs as part of routine clinical examination

Intervention Type: BIOLOGICAL

Eligibility Criteria

Inclusion Criteria

Inclusion Criteria:

• The inclusion criteria are adult patients (≥18 years) diagnosed with CTD with signed informed consent.

Exclusion Criteria:

• Exclusion criteria are patients with a serious co-morbidity interfering with the course of interstitial lung disease or pulmonary hypertension.

• Minimum Eligible Age: 18 Years
• Maximum Eligible Age: 80 Years
• Eligible Sex: ALL
• Accepts Healthy Volunteers: No

Sponsors

Boehringer Ingelheim

INDUSTRY

Sponsor Role: collaborator

Medical University Innsbruck

OTHER

Sponsor Role: lead

Responsible Party

Responsibility Role: SPONSOR

Principal Investigators

Judith Löffler-Ragg, Prof. MD

Role: PRINCIPAL_INVESTIGATOR

Medical University Innsbruck, Department Internal Medicine

Locations

Medical University Innsbruck, Department of Internal Medicine II

Innsbruck, , Austria

Site Status: RECRUITING

Countries

Austria

Central Contacts

Magdalena Aichner, MD

Role: CONTACT

magdalena.aichner@i-med.ac.at

+43 512-504-83765

Graziella Plank

Role: CONTACT

graziella.plank@i-med.ac.at

+43 512-504-81884

Facility Contacts

Graziella Plank

Role: primary

Other Identifiers

20190506-2003

Identifier Type: -

Identifier Source: org_study_id