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• W2894822463 abstract "SESSION TITLE: Medical Student/Resident Pulmonary Manifestations of Systemic Disease 3 SESSION TYPE: Med Student/Res Case Rep Postr PRESENTED ON: 10/09/2018 01:15 PM - 02:15 PM INTRODUCTION: Erdheim-Chester disease (ECD) is a rare non-Langerhans cell histiocytosis in which CD68+/CD1a- histiocytes invade multiple organs. Pulmonary disease is an uncommon initial sign but is identified in over 40% of patients with ECD. Specific patterns of lung fibrosis and cell-surface markers make ECD distinct from Langerhans cell histiocytosis. CASE PRESENTATION: A 64-year-old male with a history of pulmonary fibrosis was admitted with worsening dyspnea and hypoxemia. He had first presented 5 years earlier with declining functional capacity, dyspnea, and CT findings suggestive of ILD. No etiology had been found despite infectious and autoimmune workup. Admission chest imaging revealed reticular interstitial sagittal thickening, patchy ground glass opacities, moderate pericardial effusion, and enlarged mediastinal lymph nodes. He was started on diuretics and underwent endobronchial ultrasound (EBUS) with transbronchial biopsies, followed by EBUS and TBC. He then received empiric high-dose steroids. The lymph node biopsies were nondiagnostic but the TBC specimens revealed interstitial and peribronchial fibrosis in a perilymphatic distribution and diffuse histiocytosis. Immunohistochemistry showed CD68+/CD1a- histiocytes in areas of fibrosis consistent with pulmonary ECD. These cells also carried the BRAF V600E mutation at a 4% allele frequency. Reevaluation of his initial chest CT angiogram identified perinephric stranding and aortic wall thickening suggestive of infiltrative disease due to ECD. With steroid and diuretic therapy the patient was discharged on his baseline supplemental oxygen. He was referred to oncology to start treatment with peginterferon alfa-2a. DISCUSSION: Erdheim-Chester disease is a rare histiocytosis seldom identified based on pulmonary symptoms alone. Our case is unusual due to the primary pulmonary symptoms and uniquely employed TBC for diagnosis. Extrapulmonary involvement is a more common cause of initial symptoms and can lead to diagnosis before lung disease is recognized. In our case this was reversed but histiocytic infiltration of the kidneys and aortic wall was present. Diagnosis of lung disease relies on biopsy but traditional transbronchial approaches may be inadequate. TBC improves yield and preserves architecture with rates of bleeding and pneumothorax similar to transbronchial biopsy. Interferon-α is the best-studied treatment for ECD, but identification of the BRAF V600E mutation in ECD histiocytes has prompted study of the selective BRAF inhibitor vemurafenib as an additional therapeutic option. CONCLUSIONS: Pulmonary Erdheim-Chester disease is a challenging diagnosis that requires high clinical suspicion or sufficient biopsy material. In rare instances this diagnosis prompts recognition of extrapulmonary disease. Transbronchial cryobiopsy has advantages over traditional transbronchial biopsy and can aid clinicians in diagnosing causes of ILD. Reference #1: Mazor RD, Manevich-Mazor M, Shoenfeld Y. Erdheim-Chester Disease: a comprehensive review of the literature. Orphanet J Rare Dis. 2013;8:137. Reference #2: Rush WL, Andriko JA, Galateau-Salle F, et al. Pulmonary pathology of Erdheim-Chester disease. Mod Pathol. 2000;13(7):747-754. Reference #3: Bango-Álvarez A, Ariza-Prota M, Torres-Rivas H, et al. Transbronchial cryobiopsy in interstitial lung disease: experience in 106 cases – how to do it. ERJ Open Res. 2017;3(1). DISCLOSURES: No relevant relationships by Sarathi Bhattacharyya, source=Web Response No relevant relationships by Ariss DerHovanessian, source=Web Response No relevant relationships by Oscar Estrada, source=Web Response No relevant relationships by Bryan Garber, source=Web Response Consultant relationship with Boston Scientific, Auris, Veracyte Please note: $5001 - $20000 Added 02/01/2018 by Scott Oh, source=Web Response, value=Consulting fee No relevant relationships by W. Dean Wallace, source=Web Response" @default.
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• W2894822463 date "2018-10-01" @default.
• W2894822463 modified "2023-10-18" @default.
• W2894822463 title "AN ATYPICAL PRESENTATION OF ERDHEIM-CHESTER DISEASE DEMONSTRATING THE VALUE OF TRANSBRONCHIAL CRYOBIOPSY" @default.
• W2894822463 doi "https://doi.org/10.1016/j.chest.2018.08.810" @default.
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