Pulmonary hypertensIon in systemic sclerosis Pulmonary hypertensIon in systemic sclerosis
Conditions
Interventions
None listed
Sponsors
Hisada Ryo
Eligibility
Sex/Gender
All
Inclusion criteria
Inclusion criteria: Patients who fall under 1, 2, 3, and 4 below are eligible. 1.Age 18 years or older at the time of inclusion. 2.Fulfilling 2013 ACR/EULAR classification criteria for systemic sclerosis or having scleroderma spectrum disorders (sclerodactyly or systemic sclerosis specific autoantibodies (anti-Scl-70, anti-centromere or anti-RNA polymerase III antibodies)) and fulfilling on of the following criteria;2004 MHLW classification criteria for mixed connective tissue disease,1997 ACR classification criteria for systemic lupus erythematosus, 2012 SLICC classification criteria for systemic lupus erythematosus, Bohan and Peter criteria for the diagnosis of polymyositis and dermatomyositis, or 2017 ACR/EULAR classification criteria for primary Sjogren's syndrome. 3.Having had first right heart catheterization later than 2015 with suspicion of PH by unexplained dyspnea,increased NT-proBNP (more than twice the upper limit of normal), reduced pulmonary diffusing capacity (DLCO 1.6),and elevated tricuspid regurgitant velocity (>2.8m/s). 4.A person who voluntarily agrees to participate in the study or who does not refuse to participate in the study.
Exclusion criteria
Exclusion criteria: 1.A person who is judged by the principal investigator to be unsuitable as a research subject
Design outcomes
Primary
| Measure | Time frame |
|---|---|
| Time between the first right heart catheterization and first hospitalization or death due to worsening pulmonary hypertension. | — |
Secondary
| Measure | Time frame |
|---|---|
| 1. Change in parameters of right heart catheterization including mPAP 2. WHO functional class 3. emPHasis-10 (10 questionnaire for assessing health-related quality of life in pulmonary hypertension) 4. Six-minute walk distance 5. Plasma and serum biochemical markers including , BNP , NT-proBNP , hemoglobin , uric acid , haptoglobin , KL-6 6. Pulmonary function tests including diffusing capacity for carbon monoxide 7. Transthoracic echocardiography including tricuspid regurgitation velocity, left ventricle ejection fraction, transmitral flow velocity pattern(E, E/A ratio and deceleration time), peak velocity of early diastolic mitral annulus motion (septal e' velocity, lateral e' velocity and E/e') and left atrial volume index 8. Modified Rodnan total skin thickness score(mRSS) | — |
Contacts
Public ContactNaoko Okita
Research and Development Division,Clinical Research and Medical Innovation Center,Institute of Health Science Innovation for Medical Care,Hokkaido University Hospital
Outcome results
None listed