Connective tissue disease
Conditions
Interventions
Case series:immunosuppressive and PAH target therapy
Sponsors
Guangdong Provincial People's Hospital
Eligibility
Sex/Gender
All
Age
18 Years to 75 Years
Inclusion criteria
Inclusion criteria: 1. Aged 18 to 75 years; 2. Meet the criteria of classification and diagnosis of connective tissue diseases; 3. In the past three months, the patient was first diagnosed as pulmonary hypertension (PAH) in the World Health Organization (who) updated clinical classification of pulmonary hypertension (PH) class 1; 4. Right heart catheterization will be conducted within 3 months before screening, and fulfill the following hemodynamic criteria: (1) Mean pulmonary artery pressure >=25mmhg; (2) Pulmonary vascular resistance >=240dyn/s/cm5; (3) Pulmonary capillary wedge pressure <=15mmhg; 5. Will Sign written informed consent.
Exclusion criteria
Exclusion criteria: 1. Patients who are diagnosed as type 2, 3, 4 and 5 of pulmonary hypertension 2. Serum alanine aminotransferase (ALT) was more than 2 times of ULN; 3. With severe renal insufficiency(eGFR =2mg/kg) in the past 3 months; 5. Received biologic agent in the past 6 months; 6. With serious hypotension (diastolic pressure < 50 mmHg or systolic pressure < 80 mmHg); 7. Having aortic or mitral valve disease (any degree of valve stenosis, moderate or severe valve regurgitation), pericardial stenosis, restrictive or dilated cardiomyopathy, fatal arrhythmia, left ventricular ejection fraction < 40%, left ventricular outflow tract obstruction, symptomatic coronary heart disease; 8. Had malignant tumor history in the past 5 years; 9. Pregnant and lactating women; 10. Subjects who are participating in other clinical trials and whose protocol conflicts with this protocol; 11. Patients who is considered unsuitable for this study by researchers.
Design outcomes
Primary
| Measure | Time frame |
|---|---|
| withdraw rate of PAH target therapy; | — |
Secondary
| Measure | Time frame |
|---|---|
| rate of reaching low-risk category;time between baseline and drug withdrawal;time of drug withdraw;Drug withAdverse events and serious adverse events; | — |
Countries
China
Contacts
Public ContactXiao Zhang
Guangdong Provincial People's Hospital
Outcome results
None listed