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• W2895577668 abstract "SESSION TITLE: Disorders of the Mediastinum 2 SESSION TYPE: Med Student/Res Case Rep Postr PRESENTED ON: 10/09/2018 01:15 pm - 02:15 pm INTRODUCTION: Fibrosing mediastinitis (FM) is an excessive fibrotic reaction in the mediastinum, usually related to histoplasmosis infection. FM is rare, affecting less than 1% of patients with histoplasmosis. Idiopathic FM is considered a separate disorder, and has been related to sarcoidosis, autoimmune disease, radiation or infection. Etiology requires differentiation given treatment differences for histoplasmosis-related and idiopathic FM. CASE PRESENTATION: A 47 year old female presented with 2 weeks of dyspnea, night sweats, dry cough, dysphagia, and pleuritic chest pain. She had been prescribed steroids without improvement. CTPA on admission was negative for embolus, but showed a large partially calcified mediastinal mass which displaced the right pulmonary artery with associated narrowing of the bronchus intermedius. Bronchoscopy with biopsy was performed, however sample was inadequate. She developed fever of 102.6°F and empiric antibiotics were started. Infectious evaluation was negative except for Histoplasma antibody of 1:8, with negative urine histoplasmosis antigen. Beta-D-Glucan level was <31pg/mL (+ >80pg/mL). Mediastinoscopy showed necrotic granulomas, granulomatous inflammation, and dense hyalinized fibrosis consistent with FM. Rare histoplasma yeast was identified. Short-term voriconazole was started with improvement of symptoms. Subsequent CT scans have shown near resolution of her mediastinal mass. DISCUSSION: FM typically affects younger patients. It is thought to occur secondary to an abnormal immunologic response to fungal antigens. Increased risk has been associated with MHC class 1, HLA-A2, and class II human leukocyte antigen. Symptoms are non-specific, and include cough, dyspnea, wheezing, chest pain, dysphagia, and hemoptysis. Diagnosis is based on clinical and radiographic findings, supported by serologic testing; biopsy is not always needed. Mediastinal collagen proliferation, fibrotic changes and inflammatory cells are noted in both FM from histoplasmosis and idiopathic FM, however, invasive calcification of lymph nodes is suggestive of histoplasmosis infection. Unlike in our patient, histoplasmosis titers are typically > 1:32. Anti-fungals are often used for FM from histoplasmosis, but supporting evidence is lacking. Glucocorticoid therapy is limited to use in idiopathic FM. Prognosis is uncertain; patients may remain stable for years, experience spontaneous regression, or die from progressive disease. CONCLUSIONS: FM is a rare complication of histoplasmosis infection and must be distinguished from idiopathic FM as treatment differs. Reference #1: Peikert et al. Fibrosing Mediastinitis: Clinical Presentation, Therapeutic Outcomes, and Adaptive Immune Response. Medicine 2011; 90(6):412-423. Reference #2: Wheat LJ et al. Pulmonary Histoplasmosis Syndromes: Recognition, Diagnosis, and Management. Seminars in Respiratory and Critical Care Medicine 2004; 25(2):129-144. DISCLOSURES: No relevant relationships by Kim Jordan, source=Web Response No relevant relationships by Vincent Kang, source=Web Response No relevant relationships by Andrew MacMillan, source=Web Response" @default.
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• W2895577668 date "2018-10-01" @default.
• W2895577668 modified "2023-09-26" @default.
• W2895577668 title "FIBROSING MEDIASTINITIS: A RARE COMPLICATION OF HISTOPLASMOSIS INFECTION" @default.
• W2895577668 doi "https://doi.org/10.1016/j.chest.2018.08.429" @default.
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