Study on the Treatment of Nonthrombotic Obstructive Pulmonary Hypertension

Launched by BEIJING CHAO YANG HOSPITAL · May 12, 2025

Keywords

ClinConnect Summary

This clinical trial is studying a new treatment for a condition called fibrosing mediastinal pulmonary hypertension (FM-PH), which can cause symptoms like chest tightness and shortness of breath. Researchers want to find out if combining a medication called rituximab with a specific type of therapy that helps open up blood vessels in the lungs is more effective than using the therapy alone. People who are between 18 and 85 years old and have been diagnosed with FM-PH may be eligible to participate. They should also have signs of pulmonary hypertension that match their symptoms, as seen on a chest CT scan.

Participants in the study will be randomly assigned to one of two groups: one will receive both the rituximab injection and the vascular therapy, while the other group will only receive the vascular therapy. Throughout the year following the treatment, researchers will check how well the treatment is working by looking at improvements in symptoms and other health measures. Safety will also be monitored by comparing any side effects between the two groups. If you or someone you know is interested, it's important to discuss this with a doctor to see if they meet the eligibility criteria and can safely participate.

Gender

ALL

Eligibility criteria

• Inclusion Criteria:
• • 1. Diagnosed with fibrosing mediastinitis between November 2024 and November 2026, aged between 18 and 85 years.
• • 2. The patient presented with symptoms of chest tightness, shortness of breath, and reduced exercise tolerance.
• • 3. Chest CT revealed mediastinal lymph node compression of the pulmonary artery, with evidence of pulmonary hypertension consistent with the patient's symptoms.
• • 4. The subject signed the informed consent form prior to participation and is able to comply with the study protocol and one-year follow-up.
• Exclusion Criteria:
• • 1. Currently in the active phase of infection, including but not limited to tuberculosis, Histoplasma capsulatum, and Aspergillus infections;
• • 2. The underlying primary disease, such as sarcoidosis, Behcet's disease, or uncontrolled IgG4-related disease, is currently not well controlled.
• • 3. Before treatment, a large amount of pleural effusion was still present.
• • 4. Pulmonary function tests (PFT) showed FEV1 \<30% of the predicted value, FEV1/FVC \<30%, and DLCO \<30%.
• • 5. There are contraindications to bronchoscopy or endovascular intervention.
• • 6. Complicated by other end-stage organ dysfunction, such as Child-Pugh class C liver function or stage IV chronic renal failure.
• • 7. Complicated by severe immunosuppression.