Early Diagnosis of Pulmonary Hypertension in Patients With Inflammatory Rheumatic Connective Tissue Diseases

The purposes of this study are (I), to evaluate various screening methods for their ability to predict and to confirm Pulmonary Hypertension (PH) in scleroderma patients, and (II) to evaluate the incidence of PH (i.e. the number of new cases per year) in scleroderma patients.

Study Overview

• Status: Recruiting
• Conditions: Pulmonary Hypertension; Connective Tissue Disease

• Study Type: Observational
• Enrollment (Anticipated): 50

Contacts and Locations

• Study Contact: Name: Ekkehard Grünig, MD, Prof.; Phone Number: +49 6221 396 8053; Email: ekkehard.gruenig@med.uni-heidelberg.de

Study Locations

• Germany
  • Heidelberg, Germany, 69126
    • Recruiting
    • Center for pulmonary Hypertension, Thoraxclinic Heidelberg
    • Contact: Christian Nagel, MD; Phone Number: +49 6221 396 8063; Email: christian.nagel@thoraxklinik-heidelberg.de
    • Contact: Ekkehard Grünig, Professor; Phone Number: +49 6221 396 8053; Email: ekkehard.gruenig@thoraxklinik-heidelberg.de
    • Principal Investigator: Christian Nagel, MD

Participation Criteria

Eligibility Criteria

• Ages Eligible for Study: 18 years and older (Adult, Older Adult)
• Accepts Healthy Volunteers: No
• Genders Eligible for Study: All

• Sampling Method: Probability Sample

Study Population

Included are all patients who fulfill the current definition of a collagen vascular disease according to the actual guidelines of the American College of Rheumatology (ACR) and who are 18 years or older.

Description

Inclusion Criteria:

• consent form
• men and women> 18 years
• fulfill the current definition of a collagen vascular disease according to the actual guidelines of the American College of Rheumatology (ACR)

Exclusion Criteria:

• all contraindications for exercise testing
• significant restriction of the left ventricle, unstable coronary artery disease and myocardial infarction in the last 6 months
• patients with other lung diseases (as the pulmonary infestations as part of systemic disease) or related musculoskeletal disorders influencing baseline exercise capacity
• pregnancy

Study Plan

How is the study designed?

Design Details

• Observational Models: Cohort
• Time Perspectives: Prospective

What is the study measuring?

Primary Outcome Measures

Outcome Measure	Measure Description	Time Frame
Evaluation of various screening methods for their ability to predict and to confirm PH in scleroderma patients	Correlate Spiroergometric, lung functional Magnetic Resonance Imaging (MRI) and echocardiographic parameters with invasive hemodynamic findings collected by right heart catheterization: which other non-invasive parameters can be found to detect pulmonary hypertension? Can pulmonary hypertension be detected at rest in the echocardiography? In which patients can only stress-Doppler echocardiography confirm manifest or latent pulmonary hypertension? Is stress-Doppler echocardiography a suitable for early detection of pulmonary hypertension in patients with collagen vascular disease?	3 years

Secondary Outcome Measures

Outcome Measure	Measure Description	Time Frame
Subdivision of patients with connective tissue disease in different degrees of severity	Can spiroergometric findings help to subdivide patients with connective tissue diseases into groups according to degrees of severity, and differ the patients based on spiroergometric findings? To what extent is this distinction comparable with the classifications for the "functional classes" (application for PH according to the New York Heart Association (NYHA) classification)	3 years

Collaborators and Investigators

• Sponsor: Heidelberg University
• Investigators: Study Director: Ekkehard Grünig, Professor, Center for pulmonary Hypertension, Thoraxclinic Heidelberg; Study Director: Christian Nagel, MD, Center for pulmonary Hypertension, Thoraxclinic Heidelberg

Publications and helpful links

General Publications

• Nagel C, Marra AM, Benjamin N, Blank N, Cittadini A, Coghlan G, Distler O, Denton CP, Egenlauf B, Fiehn C, Fischer C, Harutyunova S, Hoeper MM, Lorenz HM, Xanthouli P, Bossone E, Grunig E. Reduced Right Ventricular Output Reserve in Patients With Systemic Sclerosis and Mildly Elevated Pulmonary Artery Pressure. Arthritis Rheumatol. 2019 May;71(5):805-816. doi: 10.1002/art.40814. Epub 2019 Apr 10.
• Nagel C, Henn P, Ehlken N, D'Andrea A, Blank N, Bossone E, Bottger A, Fiehn C, Fischer C, Lorenz HM, Stockl F, Grunig E, Egenlauf B. Stress Doppler echocardiography for early detection of systemic sclerosis-associated pulmonary arterial hypertension. Arthritis Res Ther. 2015 Jun 19;17(1):165. doi: 10.1186/s13075-015-0673-7.

Study record dates

Study Major Dates

• Study Start: January 1, 2010
• Primary Completion (Anticipated): December 1, 2025
• Study Completion (Anticipated): December 1, 2025

Study Registration Dates

• First Submitted: June 29, 2011
• First Submitted That Met QC Criteria: July 1, 2011
• First Posted (Estimate): July 4, 2011

Study Record Updates

• Last Update Posted (Actual): July 14, 2022
• Last Update Submitted That Met QC Criteria: July 12, 2022
• Last Verified: July 1, 2022

More Information

Terms related to this study

• Additional Relevant MeSH Terms: Cardiovascular Diseases; Vascular Diseases; Respiratory Tract Diseases; Lung Diseases; Hypertension; Hypertension, Pulmonary; Connective Tissue Diseases
• Other Study ID Numbers: S-360/2009