Connective Tissue Diseases and Lung Manifestations (Colipris)

Connective Tissue Diseases and Lung Manifestations Prospective Trial With Focus on Systemic Sclerosis (Colipris)

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.

Study Overview

• Status: Recruiting
• Conditions: Connective Tissue Diseases; Systemic Sclerosis; Interstitial Lung Disease
• Intervention / Treatment: Diagnostic test: Pulmonary function test; Diagnostic test: Imaging; Biological: Blood sampling

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.

• Study Type: Observational
• Enrollment (Anticipated): 120

Contacts and Locations

• Study Contact: Name: Magdalena Aichner, MD; Phone Number: +43 512-504-83765; Email: magdalena.aichner@i-med.ac.at
• Study Contact Backup: Name: Graziella Plank; Phone Number: +43 512-504-81884; Email: graziella.plank@i-med.ac.at

Study Locations

• Austria
  • Innsbruck, Austria, 6020
    • Recruiting
    • Medical University Innsbruck, Department of Internal Medicine II
    • Contact: Graziella Plank

Participation Criteria

Eligibility Criteria

• Ages Eligible for Study: 18 years to 80 years (ADULT, OLDER_ADULT)
• Accepts Healthy Volunteers: No
• Genders Eligible for Study: All

• Sampling Method: Non-Probability Sample

Study Population

Real-life cohort of CTD patients

Description

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.

Study Plan

How is the study designed?

What is the study measuring?

Primary Outcome Measures

Outcome Measure	Measure Description	Time Frame
Decline in forced vital capacity	Absolute decline from baseline in forced vital capacity percent predicted. Lung function tests are performed by a trained professional.	1 year
Increase in the modified Rodnan Skin Score	Change in the modified Rodnan Skin Score from baseline of >5 Points assessed by a trained professional	1 year
Decline in Diffusion capacity for carbon monoxide	Absolute decline from baseline in diffusion capacity for carbon monoxide in percent measured. Lung function tests are performed by a trained professional.	1 year

Collaborators and Investigators

• Sponsor: Medical University Innsbruck
• Collaborators: Boehringer Ingelheim
• Investigators: Principal Investigator: Judith Löffler-Ragg, Prof. MD, Medical University Innsbruck, Department Internal Medicine

Study record dates

Study Major Dates

• Study Start (ACTUAL): December 9, 2019
• Primary Completion (ANTICIPATED): March 1, 2021
• Study Completion (ANTICIPATED): October 1, 2034

Study Registration Dates

• First Submitted: September 13, 2019
• First Submitted That Met QC Criteria: September 18, 2019
• First Posted (ACTUAL): September 19, 2019

Study Record Updates

• Last Update Posted (ACTUAL): May 27, 2020
• Last Update Submitted That Met QC Criteria: May 25, 2020
• Last Verified: September 1, 2019

More Information

Terms related to this study

• Additional Relevant MeSH Terms: Pathologic Processes; Skin Diseases; Respiratory Tract Diseases; Sclerosis; Lung Diseases; Scleroderma, Systemic; Scleroderma, Diffuse; Connective Tissue Diseases; Lung Diseases, Interstitial
• Other Study ID Numbers: 20190506-2003

Drug and device information, study documents

• Studies a U.S. FDA-regulated drug product: No
• Studies a U.S. FDA-regulated device product: No