Connective Tissue Diseases and Lung Manifestations

Study Overview
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Brief Summary
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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
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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.

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

Conditions
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Connective Tissue Diseases, Interstitial Lung Disease, Systemic Sclerosis

Intervention / Treatment
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* Diagnostic Test: Pulmonary function test
* Diagnostic Test: Imaging
* Biological: Blood sampling


Participation Criteria
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Eligibility Criteria
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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.

Ages Eligible for Study
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Minimum Age: 18 Years
Maximum Age: 80 Years

Sexes Eligible for Study
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All

Accepts Healthy Volunteers
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No

Study Plan
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How is the study designed?
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Arms and Interventions

| Intervention/Treatment |
| --- |
|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|

What is the study measuring?
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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 |