Pulmonary arterial hypertension associated with connective tissue disease
Conditions
Interventions
Weekly rituximab (375mg/m2) infusion for 4 weeks
Sponsors
The University of Tokyo Hospital
Eligibility
Sex/Gender
All
Inclusion criteria
Inclusion criteria: 1)Patients with mean pulmonary arterial pressure over 25mmHg and pulmonary arterial wedge pressure under 15mmHg by right heart catheterization 2)Patients with connective tissue disease clinically suspected of pulmonary arterial hypertension associated with connective tissue disease 3)Patients with pulmonary arterial hypertension resistant to methylprednisolone pulse therapy (1 g/day for 3 days) associated with high-dose prednisolone, or high-dose oral prednisolone (over 0.8mg/kg) and intravenous cyclophosphamide therapy. Or patients with disease flare after the above therapy
Exclusion criteria
Exclusion criteria: 1)Patients with pulmonary hypertension associated with left heart disease, chronic thromboembolic pulmonary hypertension, respiratory disease, and hypoxia 2)Patients with vasodilators (endothelin receptor antagonists, prostacyclin analogues and phosphodiesterase 5 inhibitors) started, or the dosage of the vasodilators changed within 4 weeks before the trial 3)Patients with malignancies or malignancy history within 5 years 4)Patients with sever liver diseases (AST(GOT) or ALT(GPT) over 100 U/L) 5)Patients with sever kidney diseases (serum creatinine over 3.0 mg/dL) 6)Patients with sever heart diseases 7)Patients with apparent active infectious diseases 8)Patients with hepatitis B virus carrier (patients with positive HBs antibody or HBc antibody should be monitored on their HBV-DNA monthly.(Exclude patients with history of HBV vaccination)) 9)Patients with history of serious infectious diseases
Design outcomes
Primary
| Measure | Time frame |
|---|---|
| Efficacy: Estimated right ventricular systolic pressure (echocardiography) and mean pulmonary arterial pressure (right heart catheterization) Safety: Adverse events and laboratory tests | — |
Countries
Japan
Contacts
Public ContactYukiko Iwasaki
The University of Tokyo Hospital Allergy and Rheumatology
Outcome results
None listed