Connective Tissue Diseases, Interstitial Lung Disease, Systemic Sclerosis
Conditions
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.
Interventions
DIAGNOSTIC_TESTPulmonary function test
Spirometry or plethysmography, measurement of diffusion capacity
DIAGNOSTIC_TESTImaging
Thoracic ultrasound, HRCT as scheduled within routine clinical examinations, echocardiography
BIOLOGICALBlood sampling
Standard laboratory test for CTDs as part of routine clinical examination
Sponsors
Boehringer Ingelheim
Medical University Innsbruck
Study design
Observational model
OTHER
Time perspective
PROSPECTIVE
Eligibility
Sex/Gender
ALL
Age
18 Years to 80 Years
Healthy volunteers
No
Inclusion criteria
* The inclusion criteria are adult patients (≥18 years) diagnosed with CTD with signed informed consent.
Exclusion criteria
*
Design outcomes
Primary
| Measure | Time frame | Description |
|---|---|---|
| Decline in Diffusion capacity for carbon monoxide | 1 year | Absolute decline from baseline in diffusion capacity for carbon monoxide in percent measured. Lung function tests are performed by a trained professional. |
| Increase in the modified Rodnan Skin Score | 1 year | Change in the modified Rodnan Skin Score from baseline of \>5 Points assessed by a trained professional |
| Decline in forced vital capacity | 1 year | Absolute decline from baseline in forced vital capacity percent predicted. Lung function tests are performed by a trained professional. |
Countries
Austria
Contacts
Primary ContactMagdalena Aichner, MD
Backup ContactGraziella Plank
Outcome results
None listed