Clinical trial NCT06980584: Study on the Treatment of Nonthrombotic Obstructive Pulmonary Hypertension
Official title: Analysis of Clinical Characteristics and Follow-Up Study on Treatment of Nonthrombotic Obstructive Pulmonary Hypertension: Interventional Study
Study type: INTERVENTIONAL
Overall status: RECRUITING
Phase: NA
Conditions: Fibrosing Mediastinitis
Pulmonary Hypertension
Keywords: Rituximab
Endobronchial Ultrasound
Pulmonary Vascular Intervention
Lead sponsor: Beijing Chao Yang Hospital
Sponsor class: OTHER
Collaborators: name: Beijing Municipal Health Commission
First posted: 2025-05-20
Enrollment: 48
Sex: ALL
Minimum age (years): 18
Maximum age (years): 85
Standard ages: ADULT
OLDER_ADULT
Accepts healthy volunteers: no
Allocation: RANDOMIZED
Intervention model: PARALLEL
Primary purpose: TREATMENT

Brief summary: Through a randomized controlled trial (RCT) design, this study aiming to evaluated the efficacy and safety of rituximab lymph node injection combined with pulmonary vascular interventional therapy in treating fibrosing mediastinal pulmonary hypertension (FM-PH).Eligible participants were randomly assigned to either the combined treatment group, receiving both pulmonary vascular intervention and rituximab lymph node injection, or the interventional-only group, which received pulmonary vascular intervention alone. At 3, 6, and 12 months post-treatment, the efficacy was assessed based on symptom improvement, hemodynamic changes, lesion volume reduction, etc. Safety was mainly evaluated by comparing adverse event incidence between the two groups.


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.

Primary outcomes:
measure: Change from Baseline in the Six Minutes Walk Distance at 12 Months.
timeFrame: From enrollment to the end of treatment at 12 months
measure: The incidence of treatment-related serious adverse events (SAE)
timeFrame: During the one-year follow-up period after treatment completion
Secondary outcomes:
measure: Change from baseline in the World Health Organization Functional Classification of Pulmonary Hypertension at 3, 6 and 12 months.
timeFrame: From enrollment to the end of treatment at 3, 6 and 12 months.
measure: Change from Baseline in the mean pulmonary artery pressure (mPAP) at 3 and 12 Months.
timeFrame: From enrollment to the end of treatment at 3 and12 months
measure: Change from Baseline in the Cardiac Index (CI) at 3 and 12 Months.
timeFrame: From enrollment to the end of treatment at 3 and12 months
measure: Change from Baseline in the Pulmonary Vascular Resistance (PVR) at 3 and 12 Months.
timeFrame: From enrollment to the end of treatment at 3 and12 months
measure: Change from Baseline in the forced vital capacity (FVC) at 3, 6 and 12 Months.
timeFrame: From enrollment to the end of treatment at 3, 6 and 12 months
measure: Change from Baseline in the forced vital capacity of predicted (FVC%pred) at 3, 6 and 12 Months.
timeFrame: From enrollment to the end of treatment at 3, 6 and 12 months
measure: Change from Baseline in the forced expiratory volume in 1 second (FEV1) at 3, 6 and 12 Months.
timeFrame: From enrollment to the end of treatment at 3, 6 and 12 months
measure: Change from Baseline in the forced expiratory volume in 1 second of predicted (FEV1%pred) at 3, 6 and 12 Months.
timeFrame: From enrollment to the end of treatment at 3, 6 and 12 months
measure: Change from Baseline in the FEV1/FVC ratio at 3, 6 and 12 Months.
timeFrame: From enrollment to the end of treatment at 3, 6 and 12 months
measure: Change of the BNP/NT-Pro BNP from baseline to the end of treatment at 3, 6 and 12 months.
timeFrame: From enrollment to the end of treatment at 3, 6 and 12 months.
measure: The restenosis rate of blood vessels after interventional therapy at the end of treatment at 3 and 12 months
timeFrame: From enrollment to the end of treatment at 3 and 12 months.
measure: Change of the mediastinal lesion volume from baseline to 3 and 12 months after the end of treatment.
timeFrame: From enrollment to the end of treatment at 3 and12 months.
measure: Change from baseline of the quality-of-life score at 3, 6 and 12 months after the end of treatment.
timeFrame: From enrollment to the end of treatment at 3, 6 and 12 months
measure: Number of participants with complications of bronchoscopy and pulmonary intervention.
timeFrame: Immediately or in the short term after treatment, and within 12 months after treatment
Other outcomes:
Locations:
city: Beijing
country: China
facility: Beijing Chaoyang Hospital, Capital Medical University
Study officials:
References:
citation: Jia M, Su H, Jiang K, Wang A, Guo Z, Zhu H, Zhang F, Sun X, Shi Y, Pan X, Cao Y. Incidence and predictors of in-stent restenosis following intervention for pulmonary vein stenosis due to fibrosing mediastinitis. Orphanet J Rare Dis. 2024 Oct 14;19(1):379. doi: 10.1186/s13023-024-03391-8.
citation: Varghese C, Johnson GB, Eiken PW, Edell ES, Specks U, Larson NB, Peikert T. A Retrospective Evaluation of the Treatment Effects of Rituximab in Patients with Progressive and Symptomatic Fibrosing Mediastinitis. Ann Am Thorac Soc. 2024 Nov;21(11):1533-1541. doi: 10.1513/AnnalsATS.202405-533OC.