Connective Tissue Diseases and Lung Manifestations
For patients and families
In plain language
An automatic summary of structured registry data. It is an orientation aid, not a substitute for the official protocol or a physician assessment.
- What is being studied
- The protocol lists: Pulmonary function test, Imaging, Blood sampling.
- Who it may be relevant to
- Registry conditions: Connective Tissue Diseases, Interstitial Lung Disease, Systemic Sclerosis. Basic parameters: 18 years — 80 years · All.
- What needs checking
- Age, condition and sex are only basic indicators. Prior treatment, laboratory values and other mandatory requirements appear in the eligibility criteria below.
- Where it takes place
- Austria
- Next step
- Save the trial, show it to the treating physician, and confirm current recruitment with the study center. Costs, documents and travel →
Unsure about the terms? Read our patient guide →
Official title
Connective Tissue Diseases and Lung Manifestations Prospective Trial With Focus on Systemic Sclerosis (Colipris)
Overview
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.
Interventions
- 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
- Decline in forced vital capacity [Time frame: 1 year]
- Increase in the modified Rodnan Skin Score [Time frame: 1 year]
- Decline in Diffusion capacity for carbon monoxide [Time frame: 1 year]
Eligibility 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.
Criteria are shown verbatim from the registry (in English). Final eligibility is always assessed by the study center.
Healthy volunteers: No
Study design
- Observational model
- Other
Study locations
Austria · 1 center
- Medical University Innsbruck, Department of Internal Medicine II — Innsbruck
Identifiers
NCT: NCT04095351 · 20190506-2003