Clinical trial NCT04095351: Connective Tissue Diseases and Lung Manifestations
Official title: Connective Tissue Diseases and Lung Manifestations Prospective Trial With Focus on Systemic Sclerosis (Colipris)
Study type: OBSERVATIONAL
Overall status: RECRUITING
Conditions: Connective Tissue Diseases
Interstitial Lung Disease
Systemic Sclerosis
Lead sponsor: Medical University Innsbruck
Sponsor class: OTHER
Collaborators: name: Boehringer Ingelheim
First posted: 2019-09-19
Enrollment: 120
Sex: ALL
Minimum age (years): 18
Maximum age (years): 80
Standard ages: ADULT
OLDER_ADULT
Accepts healthy volunteers: no
Observational model: OTHER
Time perspective: PROSPECTIVE
Sampling method: NON_PROBABILITY_SAMPLE
Study population: Real-life cohort of CTD patients

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.

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.

Primary outcomes:
measure: Decline in forced vital capacity
timeFrame: 1 year
measure: Increase in the modified Rodnan Skin Score
timeFrame: 1 year
measure: Decline in Diffusion capacity for carbon monoxide
timeFrame: 1 year
Secondary outcomes:
Other outcomes:
Locations:
city: Innsbruck
country: Austria
facility: Medical University Innsbruck, Department of Internal Medicine II
Study officials:
affiliation: Medical University Innsbruck, Department Internal Medicine
name: Judith Löffler-Ragg, Prof. MD
role: PRINCIPAL_INVESTIGATOR
References: