Volatile Organic Compounds (VOCs) as a Biomarker in Immune-mediated Pulmonary Arterial Hypertension (PAH)

NCT ID: NCT03819777

Last Updated: 2021-09-08

Study Results

Results pending

The study team has not published outcome measurements, participant flow, or safety data for this trial yet. Check back later for updates.

Basic Information

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Recruitment Status

UNKNOWN

Total Enrollment

150 participants

Study Classification

OBSERVATIONAL

Study Start Date

2018-03-01

Study Completion Date

2023-01-31

Brief Summary

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Aim: to investigate the role of inflammation and auto-immunity in pulmonary arterial hypertension by using the profile of volatile organic compounds.

Hypothesis: first, the investigators hypothesize that at time of diagnosis the VOC profiles will discriminate patients with PAH-CTD and idiopathic PAH (IPAH) from patients with systemic sclerosis or systemic lupus erythematosus (CTD) without PAH, supporting the contention that there is a overlapping inflammatory and auto-immune pathway in PAH. During follow-up, the investigators will measure the VOC profiles of patients in all three groups who will be treated according standard clinical care. The hypothesis is that VOC profiles are affected by therapy.

Detailed Description

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Conditions

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Pulmonary Arterial Hypertension Systemic Sclerosis Systemic Lupus

Study Design

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Observational Model Type

OTHER

Study Time Perspective

PROSPECTIVE

Study Groups

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Idiopathic PAH

No interventions assigned to this group

CTD-PAH

No interventions assigned to this group

CTD without PAH

No interventions assigned to this group

Eligibility Criteria

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Inclusion Criteria

* classification as definite systemic sclerosis or systemic lupus erythematosus according to respectively the ACR-EULAR criteria (16) and SLICC criteria (17)
* minimal age of 18 year
* diagnosis of pulmonary arterial hypertension: mean pulmonary artery pressure (mPAP) of ≥25 mmHg, pulmonary capillary wedge pressure (PCWP) ≤15 mmHg, and a pulmonary vascular resistance (PVR) ≥240 dynes.s.cm-5 measured by right heart catherization.

* diagnosis of pulmonary arterial hypertension: mean pulmonary artery pressure (mPAP) of ≥25 mmHg, pulmonary capillary wedge pressure (PCWP) ≤15 mmHg, and a pulmonary vascular resistance (PVR) ≥240 dynes.s.cm-5 measured by right heart catherization.
* no family history of PAH
* triggering factor is excluded: connective tissue disease, drugs or toxins, human immunodeficiency virus, congenital heart disease, portal hypertension, schistosomiasis (2)
* minimal age of 18 year

* classification as definite systemic sclerosis or systemic lupus erythematosus according to respectively the ACR-EULAR criteria (16) and SLICC criteria (17)
* minimal age of 18 year
* no signs of PAH at screening visit

Exclusion Criteria

* active or treated malignancy
* tuberculosis or hepatitis B/C infection in case of start immunosuppressive therapy
* need to start immediately with therapy
* already use of immune suppression
Minimum Eligible Age

18 Years

Eligible Sex

ALL

Accepts Healthy Volunteers

No

Sponsors

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Maastricht University Medical Center

OTHER

Sponsor Role lead

Responsible Party

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Responsibility Role SPONSOR

Principal Investigators

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Pieter van Paassen, PhD

Role: PRINCIPAL_INVESTIGATOR

Maastricht University Medical Center

Locations

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Maastricht University Medical Center

Maastricht, , Netherlands

Site Status RECRUITING

Countries

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Netherlands

Central Contacts

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Judith Potjewijd, MD

Role: CONTACT

+31 (0) 433871198

Facility Contacts

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Judith Potjewijd, MD

Role: primary

+31-(0)43-3871198

Other Identifiers

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NL57351.068.17

Identifier Type: -

Identifier Source: org_study_id

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