Clinical Research Directory

Inclusion Criteria: * Fulfilment of the 2013 American College of Rheumatology/European Alliance of Associations for Rheumatology (ACR/EULAR) SSc classification criteria * Fulfilment of the 2022 hemodynamic definition of PAH (mean pulmonary arterial pressure (mPAP) \>20 mmHg, pulmonary artery wedge pressure (PAWP) ≤15 mmHg, and pulmonary vascular resistance (PVR) \>2 WU) in the absence of other causes of pre-capillary PH (no significant ILD and no clinical suspicion of pulmonary or left sided heart disease as the predominant cause of PH), independent of diagnostic period and previous treatment * Participants must be able to understand and follow trial procedures including completion of questionnaires regarding Patient Reported Outcome measures * Participants must have access to the internet, and experience in using smartphones or other electronic devices with internet access. * Capable of giving signed informed consent Exclusion Criteria: * Severe end organ disease 1. Severe heart failure with EF \< 30% 2. End stage kidney disease with eGFR \< 30 mL/min 3. End stage lung disease with FVC \< 50% or coexisting severe lung diseases (e.g., COPD (including emphysema), GOLD grade 3-4 with FEV1 \<50%) 4. In the opinion of the investigator, other clinically significant pulmonary abnormalities * Active treatment for cancer or non-curable cancer * Contraindications for functional assessment (6MWD and 1MSTS): 1. Uncontrolled systemic hypertension (systolic \>220 mmHg or diastolic \>120 mmHg) or hypotension (systolic \<90 mmHg), resting tachycardia (\>130 beats per minute). 2. Surgery, myocardial infarction/unstable angina, pneumothorax or stroke within the past 8 weeks. 3. Severe musculoskeletal or neurological limitations preventing safe ambulation or any acute illness which might impair performance or safety in the opinion of the investigator. * Unable to speak, write and read Norwegian * Pregnancy or planned pregnancy