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Rituximab as a Treatment for Fibrosing Mediastinitis Associated Pulmonary Vein Stenosis
Description
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A1797 - Rituximab as a Treatment for Fibrosing Mediastinitis Associated Pulmonary Vein Stenosis
Author Block: A. Mittal1, M. Wilson2, T. Peikert2, U. Specks2; 1Pulmonary and critical care medicine, Mayo clinic, Rochester, MN, United States, 2Pulmonary and critical care medicine, Mayo Clinic, Rochester, MN, United States.
Introduction
Fibrosing mediastinitis is an often progressive disease without known effective therapies. Rituximab, a monoclonal antibody against CD20 B-lymphocytes, may have a therapeutic role in targeting B-lymphocyte infiltration in fibrosing mediastinitis. We present a case where initiation of off-label Rituximab led to marked improvement in a patient with pulmonary vein stenosis caused by fibrosing mediastinitis.
Case report
A 42-year old man had a 7-year history of fibrosing mediastinitis. He initially presented to an outside institution with a left hilar mass which was thought to represent malignancy and underwent a left pneumonectomy. Surgical pathology showed fibrosing mediastinitis. Several years later, he developed recurrent gross hemoptysis (upto 1 cup daily) and dyspnea, and presented to our institution for evaluation. CT chest showed severe stenosis of the right superior pulmonary vein. Two bare metal stents were placed into the right pulmonary vein and the patient’s hemoptysis resolved. Over the next 12 months, he developed recurrent episodes of dyspnea and hemoptysis, underwent a total of 8 balloon dilatation procedures, each with temporary resolution of his dyspnea and hemoptysis. A decision was made to administer Rituximab 1000 mg IV for 2 doses. Following Rituximab administration, the patient required two additional pulmonary vein balloon dilations (at 3 and 7 months after his first dose of Rituximab respectively). Over the subsequent 2 years, the patient received 7 additional doses of Rituximab to maintain depleted B cells. During this time, his rate of hemoptysis dramatically reduced, his dyspnea was at baseline, and he did not require any further dilations of his pulmonary vein. Serial PET scans showed decreased FDG avidity of his right pulmonary vein (standardized uptake values of 6.5 gradually down to 4.0). The patient remains at his level of baseline health to this day.
Discussion
In a patient with severe pulmonary vein stenosis due to fibrosing mediastinitis who was requiring frequent balloon dilation procedures, the administration of Rituximab was associated with a marked reduction and (now for the past 24 months) elimination of balloon dilation procedures, a reduction in the episodes of recurrent dyspnea and hemoptysis, and a reduction in the FDG avidity of his right pulmonary vein on PET scan. Fibrosing mediastinitis is associated with an accumulation of CD 20 positive B-lymphocytes and the administration of Rituximab (a monoclonal antibody against CD20 B-lymphocytes) may prove to be a promising agent in the treatment of this disease process.
Author Block: A. Mittal1, M. Wilson2, T. Peikert2, U. Specks2; 1Pulmonary and critical care medicine, Mayo clinic, Rochester, MN, United States, 2Pulmonary and critical care medicine, Mayo Clinic, Rochester, MN, United States.
Introduction
Fibrosing mediastinitis is an often progressive disease without known effective therapies. Rituximab, a monoclonal antibody against CD20 B-lymphocytes, may have a therapeutic role in targeting B-lymphocyte infiltration in fibrosing mediastinitis. We present a case where initiation of off-label Rituximab led to marked improvement in a patient with pulmonary vein stenosis caused by fibrosing mediastinitis.
Case report
A 42-year old man had a 7-year history of fibrosing mediastinitis. He initially presented to an outside institution with a left hilar mass which was thought to represent malignancy and underwent a left pneumonectomy. Surgical pathology showed fibrosing mediastinitis. Several years later, he developed recurrent gross hemoptysis (upto 1 cup daily) and dyspnea, and presented to our institution for evaluation. CT chest showed severe stenosis of the right superior pulmonary vein. Two bare metal stents were placed into the right pulmonary vein and the patient’s hemoptysis resolved. Over the next 12 months, he developed recurrent episodes of dyspnea and hemoptysis, underwent a total of 8 balloon dilatation procedures, each with temporary resolution of his dyspnea and hemoptysis. A decision was made to administer Rituximab 1000 mg IV for 2 doses. Following Rituximab administration, the patient required two additional pulmonary vein balloon dilations (at 3 and 7 months after his first dose of Rituximab respectively). Over the subsequent 2 years, the patient received 7 additional doses of Rituximab to maintain depleted B cells. During this time, his rate of hemoptysis dramatically reduced, his dyspnea was at baseline, and he did not require any further dilations of his pulmonary vein. Serial PET scans showed decreased FDG avidity of his right pulmonary vein (standardized uptake values of 6.5 gradually down to 4.0). The patient remains at his level of baseline health to this day.
Discussion
In a patient with severe pulmonary vein stenosis due to fibrosing mediastinitis who was requiring frequent balloon dilation procedures, the administration of Rituximab was associated with a marked reduction and (now for the past 24 months) elimination of balloon dilation procedures, a reduction in the episodes of recurrent dyspnea and hemoptysis, and a reduction in the FDG avidity of his right pulmonary vein on PET scan. Fibrosing mediastinitis is associated with an accumulation of CD 20 positive B-lymphocytes and the administration of Rituximab (a monoclonal antibody against CD20 B-lymphocytes) may prove to be a promising agent in the treatment of this disease process.
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