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Fibrosing Mediastinitis: A Rare Case in the Ohio R ...
Fibrosing Mediastinitis: A Rare Case in the Ohio River Valley
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This case report describes fibrosing mediastinitis (FM), a rare and potentially life-threatening fibroinflammatory disease that causes dense fibrosis in the mediastinum and hilar regions, leading to compression or occlusion of airways and blood vessels. The authors present a 50-year-old African-American man from the Ohio River Valley with hypertension who came to the emergency department with three days of worsening substernal chest pain and shortness of breath. He had no infectious symptoms or other notable review-of-systems findings.<br /><br />Imaging showed pleural thickening in the left chest, narrowing of the left central bronchi, and complete occlusion of the left superior and inferior pulmonary veins with extensive collateral vessels. FDG-PET/CT demonstrated hypermetabolic activity in the left hilum. Bronchoscopy with EBUS-TBNA found no malignancy but did show extensive fibrosis, chronic inflammation, and dystrophic calcifications. A ventilation-perfusion scan revealed essentially absent perfusion to the left lung. Testing for tuberculosis, histoplasma, and autoimmune disease was negative.<br /><br />Based on these findings, the patient was diagnosed with FM and started on prednisone 40 mg daily with TMP-SMX prophylaxis. Pulmonology did not place an airway or vascular stent, and the patient was discharged with outpatient follow-up.<br /><br />The discussion emphasizes that FM is most often associated with prior histoplasma infection or is idiopathic, with increased risk in the Ohio River Valley where Histoplasma capsulatum is endemic. Symptoms can vary widely and may include dyspnea, chest pain, hemoptysis, dysphagia, or swelling. Because several conditions can mimic FM, careful work-up is required to exclude active infection, autoimmune disease, lymphoma, and granulomatous disorders. There is no gold-standard treatment; management is individualized and often involves a multidisciplinary team focused on relieving obstruction and considering corticosteroids or antifungal therapy.
Asset Subtitle
Keir F. MacKay
Meta Tag
Author List
Keir F. MacKay, Roger Johnson
Category
Clinical Vignettes
Concept
Fibrosing Mediastinitis
Concept
Fibroinflammatory Infiltration
Concept
Mediastinum
Concept
Hilar Region
Concept
Airway Compression
Distinguished
Non-Finalist
Presenter Organization
Southwest Indiana Internal Medicine Residency (Indiana University)
Presenting Author
Keir F. MacKay
Track
Adult
Keywords
fibrosing mediastinitis
mediastinal fibrosis
Ohio River Valley
Histoplasma capsulatum
pulmonary vein occlusion
bronchoscopy
EBUS-TBNA
FDG-PET/CT
corticosteroid therapy
dyspnea
Fibrosing Mediastinitis
Fibroinflammatory Infiltration
Mediastinum
Hilar Region
Airway Compression
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