Volatile organic compounds as a biomarker in immune-mediated pulmonary arterial hypertensio

Recruitment & Eligibility

Status: Completed

Sex: Not specified

Target Recruitment: 150

Inclusion Criteria

1) PAH-CTD patients, inclusion criteria:
- classification as definite systemic sclerosis or systemic lupus erythematosus
according to respectively the ACR-EULAR criteria and SLICC criteria
- 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. , 2) IPAH patients, inclusion criteria:
- 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
- minimal age of 18 year, 3) SSc and SLE patients without PAH, 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
- no signs of PAH at screening visit
4) CTEPH patients, inclusion criteria:
- diagnosis of pulmonary hypertension: mean pulmonary artery pressure
(mPAP) of >=25 mmHg, pulmonary capillary wedge pressure (PCWP) <=15 mmHg measured
by right heart catherization.
- mismatched perfusion defects on V/Q lung scan

Exclusion Criteria

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

Study & Design

Study Type: Observational invasive

Study Design: Not specified

Primary Outcome Measures

Name	Time	Method
<p>Determining unique inflammatory VOC profiles in four different groups of<br /><br>patients: PAH-CTD patients, CTEPH patients, IPAH patients and SSc/SLE patients<br /><br>without PAH.</p><br>

Secondary Outcome Measures

Name	Time	Method
<p>Unique inflammatory VOCs will be studied in relation to well-established<br /><br>biomarkers of immune activation and inflammation in PAH-CTD and idiopathic PAH.<br /><br>To this end, the selective inflammatory VOCs will be compared with baseline VOC<br /><br>profiles after 3, 6 and12 months in all patients treated with PAH medication<br /><br>and/or immunosuppressive therapy.</p><br>