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Connective Tissue Diseases and Lung Manifestations (Colipris)

The safety and scientific validity of this study is the responsibility of the study sponsor and investigators. Listing a study does not mean it has been evaluated by the U.S. Federal Government. Know the risks and potential benefits of clinical studies and talk to your health care provider before participating. Read our disclaimer for details. Identifier: NCT04095351
Recruitment Status : Recruiting
First Posted : September 19, 2019
Last Update Posted : May 27, 2020
Boehringer Ingelheim
Information provided by (Responsible Party):
Medical University Innsbruck

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.

Condition or disease Intervention/treatment
Connective Tissue Diseases Interstitial Lung Disease Systemic Sclerosis 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.

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Study Type : Observational [Patient Registry]
Estimated Enrollment : 120 participants
Observational Model: Other
Time Perspective: Prospective
Target Follow-Up Duration: 10 Years
Official Title: Connective Tissue Diseases and Lung Manifestations Prospective Trial With Focus on Systemic Sclerosis (Colipris)
Actual Study Start Date : December 9, 2019
Estimated Primary Completion Date : March 1, 2021
Estimated Study Completion Date : October 1, 2034

Intervention Details:
  • Diagnostic Test: Pulmonary function test
    Spirometry or plethysmography, measurement of diffusion capacity
  • Diagnostic Test: Imaging
    Thoracic ultrasound, HRCT as scheduled within routine clinical examinations, echocardiography
  • Biological: Blood sampling
    Standard laboratory test for CTDs as part of routine clinical examination

Primary Outcome Measures :
  1. Decline in forced vital capacity [ Time Frame: 1 year ]
    Absolute decline from baseline in forced vital capacity percent predicted. Lung function tests are performed by a trained professional.

  2. Increase in the modified Rodnan Skin Score [ Time Frame: 1 year ]
    Change in the modified Rodnan Skin Score from baseline of >5 Points assessed by a trained professional

  3. Decline in Diffusion capacity for carbon monoxide [ Time Frame: 1 year ]
    Absolute decline from baseline in diffusion capacity for carbon monoxide in percent measured. Lung function tests are performed by a trained professional.

Biospecimen Retention:   Samples With DNA
From each patient with proven systemic sclerosis one serum vial of 10 ml and 2 EDTA vials à 10 ml will be taken. Peripheral blood mononuclear cells are isolated from the EDTA blood samples, an stored at -80°C.The serum samples will be centrifuged and the resulting serum is collected in cryovials and stored at -80 °C until measurement. Samples and data will be stored and archived central according to the new standards of the biobank facility of the medical university of Innsbruck.

Information from the National Library of Medicine

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Ages Eligible for Study:   18 Years to 80 Years   (Adult, Older Adult)
Sexes Eligible for Study:   All
Accepts Healthy Volunteers:   No
Sampling Method:   Non-Probability Sample
Study Population
Real-life cohort of CTD patients

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.

Information from the National Library of Medicine

To learn more about this study, you or your doctor may contact the study research staff using the contact information provided by the sponsor.

Please refer to this study by its identifier (NCT number): NCT04095351

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Contact: Magdalena Aichner, MD +43 512-504-83765
Contact: Graziella Plank +43 512-504-81884

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Medical University Innsbruck, Department of Internal Medicine II Recruiting
Innsbruck, Austria, 6020
Contact: Graziella Plank         
Sponsors and Collaborators
Medical University Innsbruck
Boehringer Ingelheim
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Principal Investigator: Judith Löffler-Ragg, Prof. MD Medical University Innsbruck, Department Internal Medicine
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Responsible Party: Medical University Innsbruck Identifier: NCT04095351    
Other Study ID Numbers: 20190506-2003
First Posted: September 19, 2019    Key Record Dates
Last Update Posted: May 27, 2020
Last Verified: September 2019

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Studies a U.S. FDA-regulated Drug Product: No
Studies a U.S. FDA-regulated Device Product: No
Additional relevant MeSH terms:
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Lung Diseases
Lung Diseases, Interstitial
Scleroderma, Systemic
Scleroderma, Diffuse
Connective Tissue Diseases
Pathologic Processes
Respiratory Tract Diseases
Skin Diseases