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- W2894584317 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 a rare entity with unspecified incidence and pathophysiology. FM is an excessive fibrotic reaction presumably caused by a reaction to granulomatous disease, infection, connective tissue disease or malignancy (1). Presentation of FM is a consequence of compression of the airways, heart and great vessels, or esophagus, leading to postobstructive pneumonia, atelectasis, bronchial erosions, pulmonary HTN, constrictive pericarditis, SVC syndrome. We report an unusual case of FM presented with chronic lower back pain and acute paraplegia CASE PRESENTATION: A 44 year old Ecuadorian female with a history of rheumatoid arthritis presented with chronic back pain and acute paraplegia. Thoracolumbar spine CT and MRI showed epidural mass extending from T4 to T10. TTE showed massive pericardial effusion and estimated PASP of 110 mmHg. CT chest revealed mediastinal mass and transesophageal FNA was performed. Patient was started on IV steroids, regaining most of her lower extremity strength with TTE showing shrinking of pericardial effusion and PASP of 45 mmHg. Results from FNA mass biopsy showed non-specific inflammatory findings. VATS with pericardial, mediastinal mass, lymph nodes and chest wall biopsies was obtained with non-specific results. Lymphoma panel was negative with 41% of CD20+ B cells. Blood analysis were RF and ANA positive (titer 1:80, speckled pattern), ACE and IgG subsets normal, RPR, HIV, Quantiferon, Aspergillus and Histoplasma antigen negative. She was discharged on oral steroids, but was lost to follow up. Four years later, patient presented to the hospital with progressively worsening chest pain, found to be in decompensated RV failure. CT chest showed worsening of the mediastinal mass, with stenosis of the proximal main right and left pulmonary arteries, and narrowing of the proximal left subclavian artery. TTE showed PASP of 150 mmHg. Gallium scan findings were suspicious for foci of mediastinal inflammation. Patient left the hospital and was again lost to follow up DISCUSSION: So far there is no evidence-based treatment for FM. Surgical interventions in FM are mainly palliative. Several case reports suggested potential improvement of FM with antifungal medication in Histoplasma positive patients (1) or with steroids in patients with sarcoidosis or autoimmune FM (2). In case series with 3 patients with CD20+ B cells in tissue samples, Rituximab caused therapeutic response (3) CONCLUSIONS: Given negative extensive workup our patient likely had idiopathic FM but with gallium scan results and patient’s neurological and cardiovascular response to steroids, autoimmune etiology is still possible. Benefit of Rituximab therapy is still open with serial PET scans are needed for therapy follow up. To our knowledge this is the first report of chronic lower back pain and acute paraplegia as a initial presentation of FM Reference #1: Garrett HE, Roper CL. Surgical intervention in histoplasmosis. Ann Thorac Surg [Internet]. 1986 Dec [cited 2018 Mar 3];42(6):711–22 Reference #2: Seferian A, Steriade A, Jaïs X, Planché O, Savale L, Parent F, et al. Pulmonary Hypertension Complicating Fibrosing Mediastinitis. Medicine (Baltimore) [Internet]. 2015 Nov [cited 2018 Mar 2];94(44):e1800 Reference #3: Westerly BD, Johnson GB, Maldonado F, Utz JP, Specks U, Peikert T. Targeting B lymphocytes in progressive fibrosing mediastinitis. Am J Respir Crit Care Med [Internet]. 2014 Nov 1 [cited 2018 Mar 3];190(9):1069–71 DISCLOSURES: No relevant relationships by Eugenio Capitle, source=Web Response No relevant relationships by Barbara Danek, source=Web Response No relevant relationships by CONSTANTINOS LOVOULOS, source=Web Response No relevant relationships by James Maher, source=Web Response No relevant relationships by Monali Patel, source=Admin input No relevant relationships by Luka Petrovic, source=Web Response No relevant relationships by Rastko Rakocevic, source=Web Response" @default.
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- W2894584317 date "2018-10-01" @default.
- W2894584317 modified "2023-09-25" @default.
- W2894584317 title "FIBROSING MEDIASTINITIS: A RARE PRESENTATION OF A RARE DISEASE" @default.
- W2894584317 doi "https://doi.org/10.1016/j.chest.2018.08.436" @default.
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