high blood pressure in the arteries from heart to lungs Pulmonary arterial hypertension
Conditions
Interventions
None listed
Sponsors
Medisch Universitair Ziekenhuis Maastricht
Eligibility
Age
18 Years to 99 Years
Inclusion criteria
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) =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) =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)
Exclusion criteria
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
Design outcomes
Primary
| Measure | Time frame |
|---|---|
| Determining unique inflammatory VOC profiles in four different groups of patients: PAH-CTD patients, CTEPH patients, IPAH patients and SSc/SLE patients without PAH. | — |
Secondary
| Measure | Time frame |
|---|---|
| Unique inflammatory VOCs will be studied in relation to well-established biomarkers of immune activation and inflammation in PAH-CTD and idiopathic PAH. To this end, the selective inflammatory VOCs will be compared with baseline VOC profiles after 3, 6 and12 months in all patients treated with PAH medication and/or immunosuppressive therapy. | — |
Countries
Netherlands
Outcome results
None listed