Connective Tissue Diseases and Lung Manifestations

Recruiting

• Conditions: Connective Tissue Diseases; Systemic Sclerosis; Interstitial Lung Disease

• Registration Number: NCT04095351
• Lead Sponsor: 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.

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 Timeline

• Status Verified: 2019-09
• Study First Submitted: 2019-09-13
• Study First Submitted QC: 2019-09-18
• Study First Posted: 2019-09-19
• Study Start: 2019-12-09
• Last Update Submitted: 2020-05-25
• Last Update Posted: 2020-05-27
• Primary Completion: 2021-03-01
• Study Completion: 2034-10-01

Recruitment & Eligibility

• Status: RECRUITING
• Sex: All
• Target Recruitment: 120

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 & Design

• Study Type: OBSERVATIONAL
• Study Design: Not specified

Primary Outcome Measures

Name	Time	Method
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 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.
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.

Trial Locations

Locations (1)

Medical University Innsbruck, Department of Internal Medicine II; 🇦🇹 Innsbruck, Austria