Successful management of fibrosing mediastinitis with severe vascular compromise: Report of two cases and literature review

Published on Jan 1, 2020in Respiratory medicine case reports
· DOI :10.1016/J.RMCR.2019.100987
Franklin A. Argueta1
Estimated H-index: 1
(University of Texas Health Science Center at San Antonio),
David Villafuerte1
Estimated H-index: 1
(University of Texas Health Science Center at San Antonio)
+ 2 AuthorsCarlos S. Restrepo22
Estimated H-index: 22
(University of Texas Health Science Center at San Antonio)
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Abstract
Abstract Fibrosing mediastinitis is a rare disorder characterized by the invasive proliferation of fibrous tissue within the mediastinum. This fibrosis can result in compression of intrathoracic structures including the pulmonary vasculature leading to clinical symptoms and complications like pulmonary hypertension. Here, we present two cases of young patients with fibrosing mediastinitis complicated by pulmonary artery stenosis requiring medical and endovascular management with excellent results.
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References19
Newest
#1Satoshi Takanashi (Keio: Keio University)H-Index: 5
#2Mitsuhiro AkiyamaH-Index: 12
Last. Tsutomu TakeuchiH-Index: 68
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AbstractRationale:Immunoglobulin G4-related disease (IgG4-RD) is a fibroinflammatory disease characterized by elevated serum IgG4 levels with infiltration of IgG4+ plasma cells and severe fibrosis in affected tissues. Recently, idiopathic fibrosing mediastinitis (FM), an extremely rare fibroinflamma
12 CitationsSource
#1Shamaita Majumdar (WashU: Washington University in St. Louis)H-Index: 1
#2Ramy Shoela (WashU: Washington University in St. Louis)H-Index: 1
Last. Olaguoke Akinwande (WashU: Washington University in St. Louis)H-Index: 7
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Purpose:To assess the outcomes of endovascular management for superior vena cava (SVC) syndrome secondary to fibrosing mediastinitis (FM).Methods:Between January 2004 and December 2016, 10 consecutive patients with endovascularly managed SVC syndrome secondary to FM were identified in an institutional database. Venograms were performed to assess the severity and location of the lesion and allow measurement for stent selection. Standard stenting and angioplasty techniques were utilized to establi...
3 CitationsSource
#1Jérémy BardetH-Index: 1
#2Dominique FabreH-Index: 21
Last. Elie FadelH-Index: 23
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Purpose:To report the endovascular reconstruction of the superior vena cava (SVC), innominate and internal jugular veins following stenosis due to mediastinal fibrosis.Case Report:A 36-year-old fem...
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#1Yidan LiH-Index: 6
#2Xiangli MengH-Index: 3
Last. Xiuzhang LuH-Index: 3
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INTRODUCTION: Fibrosingmediastinitis (FM) is caused by a proliferation of fibrous tissue in the mediastinum encasing the mediastinal viscera that results in compression of mediastinal bronchovascular structures. Pulmonary hypertension (PH) is a severe complication of FM caused by extrinsic compression of pulmonary blood vessels. CASE PRESENTATION: Here, we present the case of a 47-year-old man who presented with a 10-year history of progressive hemoptysis and a 2-year history of shortness of bre...
3 CitationsSource
#1Junjing Su (AU: Aarhus University)H-Index: 1
#2Ulf Simonsen (AU: Aarhus University)H-Index: 40
Last. Søren Mellemkjær (Aarhus University Hospital)H-Index: 15
view all 4 authors...
Unusual forms of pulmonary hypertension include pulmonary hypertension related to mediastinal fibrosis and the use of serotonergic drugs. Here, we describe a patient with diffuse mediastinal fibrosis and pulmonary hypertension while she was on dopamine agonist therapy. A young woman, who was treated with cabergoline and bromocriptine for hyperprolactinemia, presented with progressive dyspnea over several months. Based on the clinical investigation results, in particular, elevated pulmonary arter...
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#1Abdulaziz U. Joury (King Fahd Medical City)H-Index: 2
#2Ahmad A. Al Boukai (King Khalid University)H-Index: 2
Last. Tarek Kashour (King Khalid University)H-Index: 16
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Fibrosing mediastinitis (FM), also known as sclerosing mediastinitis, is an uncommon but serious disease involving the mediastinal structures. A high index of suspicion is essential to establish the diagnosis of FM and starting the appropriate therapy for patients. Here, we report a case of a young female who presented with chest symptoms and subsequently underwent different laboratory and radiologic investigations and an excisional biopsy. The findings of these investigations were consistent wi...
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Mediastinal fibrosis is a rare disease characterised by fibrous proliferation in the mediastinum. It can be idiopathic or secondary to several conditions such as infections and malignancies. Anecdotal reports have described idiopathic mediastinal fibrosis (IMF) in association with other fibro-inflammatory or autoimmune diseases. We report nine new IMF cases recently seen at our Fibro-Inflammatory Disease Clinic and reviewed the IMF cases reported in the English language literature throughout 200...
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#1Samantha C. Shapiro (JHUSOM: Johns Hopkins University School of Medicine)H-Index: 1
#2Armen Khararjian (Johns Hopkins University)H-Index: 4
Last. Rebecca L. Manno (JHUSOM: Johns Hopkins University School of Medicine)H-Index: 10
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Background We report a case in which the extraintestinal manifestations of inflammatory bowel disease preceded development of gastrointestinal symptoms by nearly 9 months in the context of an unusual autoantibody panel, mimicking granulomatosis with polyangiitis. This case highlights the intricacies and overlap of autoimmune diseases, and illustrates an interesting clinical phenotype: cytoplasmic anti-neutrophil cytoplasmic antibody positive inflammatory bowel disease with predominantly extraint...
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#1Andrei Seferian (University of Paris-Sud)H-Index: 11
#2Alexandru Steriade (University of Paris-Sud)H-Index: 2
Last. David MontaniH-Index: 59
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Fibrosing mediastinitis is caused by a proliferation of fibrous tissue in the mediastinum with encasement of mediastinal viscera and compression of mediastinal bronchovascular structures. Pulmonary hypertension (PH) is a severe complication of fibrosing mediastinitis caused by extrinsic compression of the pulmonary arteries and/or veins. We have conducted a retrospective observational study reviewing clinical, functional, hemodynamic, radiological characteristics, and outcome of 27 consecutive c...
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