Study on the Treatment of Nonthrombotic Obstructive Pulmonary Hypertension

Официальное название

Analysis of Clinical Characteristics and Follow-Up Study on Treatment of Nonthrombotic Obstructive Pulmonary Hypertension: Interventional Study

Обзор

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.

Вмешательства

• Процедура Rituximab Lymph Node Injection Combined with Pulmonary Vascular Intervention
  Based on the degree of vascular stenosis, individualized and periodic pulmonary vascular interventions are performed to reopen obstructed vessels. During the treatment cycle, one lymph node drug injection is administered: 50mg of rituximab is dissolved in 15ml of 5% glucose solution and injected at multiple sites into the identified enlarged mediastinal lymph nodes under ultrasound-guided bronchoscopy.
• Процедура Pulmonary Vascular Interventional Therapy
  According to the degree of vascular stenosis, individualized and periodic pulmonary vascular interventions are performed to reopen obstructed pulmonary vessels.

Первичные конечные точки

• Change from Baseline in the Six Minutes Walk Distance at 12 Months. [Срок оценки: From enrollment to the end of treatment at 12 months]
• The incidence of treatment-related serious adverse events (SAE) [Срок оценки: During the one-year follow-up period after treatment completion]

Вторичные конечные точки (12)

• Change from baseline in the World Health Organization Functional Classification of Pulmonary Hypertension at 3, 6 and 12 months. [Срок оценки: From enrollment to the end of treatment at 3, 6 and 12 months.]
• Change from Baseline in the mean pulmonary artery pressure (mPAP) at 3 and 12 Months. [Срок оценки: From enrollment to the end of treatment at 3 and12 months]
• Change from Baseline in the Cardiac Index (CI) at 3 and 12 Months. [Срок оценки: From enrollment to the end of treatment at 3 and12 months]
• Change from Baseline in the Pulmonary Vascular Resistance (PVR) at 3 and 12 Months. [Срок оценки: From enrollment to the end of treatment at 3 and12 months]
• Change from Baseline in the forced vital capacity (FVC) at 3, 6 and 12 Months. [Срок оценки: From enrollment to the end of treatment at 3, 6 and 12 months]
• Change from Baseline in the forced vital capacity of predicted (FVC%pred) at 3, 6 and 12 Months. [Срок оценки: From enrollment to the end of treatment at 3, 6 and 12 months]
• Change from Baseline in the forced expiratory volume in 1 second (FEV1) at 3, 6 and 12 Months. [Срок оценки: From enrollment to the end of treatment at 3, 6 and 12 months]
• Change from Baseline in the forced expiratory volume in 1 second of predicted (FEV1%pred) at 3, 6 and 12 Months. [Срок оценки: From enrollment to the end of treatment at 3, 6 and 12 months]
• Change from Baseline in the FEV1/FVC ratio at 3, 6 and 12 Months. [Срок оценки: From enrollment to the end of treatment at 3, 6 and 12 months]
• Change of the BNP/NT-Pro BNP from baseline to the end of treatment at 3, 6 and 12 months. [Срок оценки: From enrollment to the end of treatment at 3, 6 and 12 months.]
• The restenosis rate of blood vessels after interventional therapy at the end of treatment at 3 and 12 months [Срок оценки: From enrollment to the end of treatment at 3 and 12 months.]
• Change of the mediastinal lesion volume from baseline to 3 and 12 months after the end of treatment. [Срок оценки: From enrollment to the end of treatment at 3 and12 months.]

Критерии участия

Критерии включения

• Diagnosed with fibrosing mediastinitis between November 2024 and November 2026, aged between 18 and 85 years.
• The patient presented with symptoms of chest tightness, shortness of breath, and reduced exercise tolerance.
• Chest CT revealed mediastinal lymph node compression of the pulmonary artery, with evidence of pulmonary hypertension consistent with the patient's symptoms.
• The subject signed the informed consent form prior to participation and is able to comply with the study protocol and one-year follow-up.

Критерии исключения

• Currently in the active phase of infection, including but not limited to tuberculosis, Histoplasma capsulatum, and Aspergillus infections;
• The underlying primary disease, such as sarcoidosis, Behcet's disease, or uncontrolled IgG4-related disease, is currently not well controlled.
• Before treatment, a large amount of pleural effusion was still present.
• Pulmonary function tests (PFT) showed FEV1 <30% of the predicted value, FEV1/FVC <30%, and DLCO <30%.
• There are contraindications to bronchoscopy or endovascular intervention.
• Complicated by other end-stage organ dysfunction, such as Child-Pugh class C liver function or stage IV chronic renal failure.
• Complicated by severe immunosuppression.

Критерии приведены из реестра в оригинале (на английском). Окончательную оценку соответствия проводит исследовательский центр.

Здоровые добровольцы: Нет

Дизайн исследования

Распределение

Рандомизированное

Модель

Параллельные группы

Маскирование

Открытое

Основная цель

Лечение

Центры проведения

Китай · 1 центр

• Beijing Chaoyang Hospital, Capital Medical University — Пекин

Публикации

• 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. PMID 39397011
• 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. PMID 39106522

Идентификаторы

NCT: NCT06980584 · 2025-KE-61_Int

Первоисточники (государственные реестры)

• ClinicalTrials.gov (NIH, США)

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