Clinical research on treatment of pulmonary arterial hypertension associated with connective tissue disease by Mycophenolate mofetil

• Register: ChiCTR
• Last refreshed on: 18 April 2017
• Main ID: ChiCTR-TRC-14004894
• Date of registration: 2014-06-30
• Prospective Registration: Yes
• Primary sponsor: Beijing Chaoyang Hospital affiliated to Capital Medical University
• Public title: Clinical research on treatment of pulmonary arterial hypertension associated with connective tissue disease by Mycophenolate mofetil
• Scientific title: Clinical research on treatment of pulmonary arterial hypertension associated with connective tissue
• Date of first enrolment: 2014-07-01
• Target sample size: A:8;B:8;
• Recruitment status: Recruiting
• URL: http://www.chictr.org.cn/showproj.aspx?proj=4679
• Study type: Interventional study
• Study design: Randomized parallel controlled trial
• Phase: Other

Contacts

• Name: Zheng Yi

• Name: Wen Xiaohong
• Affiliation: Beijing Chaoyang Hospital affiliated to Capital Me

Key inclusion & exclusion criteria

Inclusion criteria: 1. The subjects aged 18 to 80 years old patients; 2. The patients with connective tissue disease are in line with the corresponding classification standard (China Medical Association rheumatism branch to make the disease diagnosis and treatment guidelines); 3. Using color Doppler ultrasound cardiogram indirect estimation of pulmonary artery systolic pressure, it is determined for pulmonary arterial hypertension by greater than 40mmHg (according to American College of Cardiology Foundation and American Heart Association standard); or critical pulmonary hypertension associated with connective tissue (reference 2009 edition of the European Society of Cardiology diagnosis and treatment of pulmonary artery hypertension guidelines) when three tricuspid back flow velocity less than 2.8 m/s or pulmonary artery systolic pressure less than 36 mmHg, in combination with other ultrasound cardiogram index (such as the right ventricular hypertrophy, right ventricular enlargement, pulmonary regurgitation rate etc.) determine the presence of pulmonary arterial hypertension; 4. The naive patients (with pulmonary artery hypertension specific drug use or not); 5. Patients or spouse must be willing and able to take safe, effective contraception; 6. The nonspecific treatment allows for the treatment of pulmonary arterial hypertension, for example: oral anticoagulation, diuretics, digitalis, calcium channel blockers or oxygen. But the anticoagulation therapy (if indicated) must be in before entering the group for at least 30 days already, diuretic therapy must be at least 30 days before entering the group has stable dose. Require long-term oxygen therapy patients, oxygen supplement and complement method into the group of at least 90 days before has been stable, can be put into a group; 7. The screen without using immunosuppressant (including mycophenolate mofetil, cyclophosphamide, methotrexate, leflunomide, azathioprine, cyclosporine, tacrolimus) or stop drug at least 4 weeks; 8. The screen without using glucocorticoid or oral corticosteroid dose stability (the equivalent of prednisone acetate) less than 15mg/d; 9. The signed informed consent voluntarily.
Exclusion criteria: 1. At the same time participating in any other clinical trial or participated in any clinical studies in 30 days before entering the group;
2. The pregnancy (such as human chorionic gonadotropin test positive or other pregnancy signs) or lactating women, contraception and fertility in women without the use of safety;
3. According to the researchers, patients, medical abnormal physical condition or history may affect their ability to participate in or complete the study;
- 180 days before the visit 1, substance abuse (such as alcohol or drug abuse) patients.
- In patients with underlying diseases, the expected survival time of less than 2 years (for example: malignant tumor, local and / or metastatic tumor mass)
- Patients who are hypersensitive to the study drugs or non active group.
- Patients who are in urgent, chronic infection during or prior history of active tuberculosis without regular anti tuberculosis treatment.
- Patient can't complete the 6MWD test (such as: severe peripheral arterial occlusive disease). Note: the need for walking assist patients, if researchers think the walking distance is not affected can be selected.
- A history of mental illness or other reasons cannot cooperate with the treatment of inspection.
4. The other causes of pulmonary arterial hypertension:
- Familial pulmonary arterial hypertension;
- Pulmonary arterial hypertension associated with congenital heart diseases (i.e., ASD, VSD, patent ductus arteriosus continues), whether or not to correct operation;
- Pulmonary arterial hypertension caused by portal hypertension because of hepatic cirrhosis;
- Anorexigen (appetite suppressing drugs) or amphetamine related pulmonary hypertension.
5. The lung disease associated with one of the following circumstances:
- Moderate to severe obstructive pulmonary disease (FEV1 less than the expected value of 60%);
- Severe restrictive lung disease (less than 70% of the expected value of total lung capacity);
- Severe lung, thoracic and mediastinal congenital anomaly.
6. The cardiovascular disorders associated with one of the following circumstances:
- Systolic blood pressure more than 180mmHg and / or more than 110mmHg diastolic blood pressure or arterial hypotension; history of uncontrolled and / or systolic blood pressure of less than 95mmHg;
- Resting heart rate less than 50 / minute or more than 105 / min.;
- Atrial fibrillation or flutter history in 90 days;
- There have been left heart failure, ejection fraction less than 40%, in 90 days;
- Hypertrophic obstructive cardiomyopathy;
- Severe coronary heart disease suspected or confirmed (Canadian Cardiovascular Society classification of angina pectoris severity is 2-4, and/or nitrate therapy, and/or in the group within 90 days prior to myocardial infarction);
- Clinical evidence of atherosclerotic disease (stroke such as: walking distance to reduce the peripheral artery disease, persistent neurological deficits)
- In addition to the three cusp of pulmonary hypertension caused by the insufficiency, the clinical significance of congenital or acquired valvular or myocardial disease;
7. Liver function and clinical significance of incomplete:
- Bilirubin more than 2 times the upper limit of normal and/or ALT or AST more than 3 times the upper limit of normal;
- Serum creatinine more than 1.5 times the upper limit of normal;
The leukocyte less than 3 time 10*9/L or hemoglobin less than 80g/L or platelet less than 50 times 10*9/L
8. inc

Age minimum: 18
Age maximum: 80
Gender: Male

Health Condition(s) or Problem(s) studied

pulmonary arterial hypertension associated with connective tissue disease

Intervention(s)

A:Glucocorticoid plus sildenafil;B:Glucocorticoid plus sildenafil;

Primary Outcome(s)

6-min walk distance, 6MW;

Secondary Outcome(s)

Estimation of pulmonary artery pressure by UCG;visual analog scale, VAS;The modified Borg dyspnea score;The WHO function classificatio;

Source(s) of Monetary Support

Beijing City financial technology funding