REFRACTORY SARCOIDOSIS: A CASE OF CHALLENGING THERAPEUTIC DILEMMA

نویسندگان

چکیده

TOPIC: Lung Pathology TYPE: Fellow Case Reports INTRODUCTION: Sarcoidosis is a multi-system granulomatous disorder with characteristic pulmonary manifestations and typical histopathologic features. Treatment of sarcoidosis typically based on an assessment the extent, severity activity disease. Corticosteroids are considered first-line therapy for many decades; however, early initiation steroid-sparing agents gaining popularity especially in progressive cases that do not respond well to steroids [1]. We present case refractory requiring multiple cytotoxic biologic therapies. CASE PRESENTATION: A 56-year-old Caucasian female presented dyspnea, fever leukocytosis was found have bilateral large masses mediastinal hilar adenopathy concerning infection or malignancy. She did antibiotic underwent endobronchial ultrasound transbronchial needle aspiration (EBUS-TBNA) lymph nodes twice followed by CT guided biopsy one masses, all showed non-caseating granulomas absence malignancy infectious stains cultures. Atypical presentation bronchoalveolar lavage (BAL) cellular analysis showing 36% lymphocytes CD4/CD8 ratio 4.24. Serologic work up no connective tissue diseases vasculitis. The patient started corticosteroids but continued progress persistent fevers leukocytosis. Azathioprine then changed methotrexate because intolerance. Despite adequate doses prednisone, progressed became oxygen dependent. Repeat scan enlargement lung development cavitations. EBUS-TBNA BAL still agents. also had elevated interlukin-2 receptors suggesting increased Infliximab which led improvement symptoms stability disease; could tolerate last infusion hypersensitivity reaction. Mycophenolate mofetil later added, it final decision start rituximab. Her symptoms, function findings stabilized rituximab she being evaluated transplant. DISCUSSION: diagnosis can be challenging when clinical response atypical. should always individual patient's needs response. Poor prompt reevaluation, investigation other causes consideration [2]. CONCLUSIONS: Refractory sarcoidosis, where treatments fail, remain real challenge poorly understood pathophysiology. REFERENCE #1: Wijsenbeek MS, Culver DA. Sarcoidosis. Clin Chest Med. 2015 Dec;36(4):751-67. doi: 10.1016/j.ccm.2015.08.015. PMID: 26593147 #2: El Jammal, Thomas, et al. "Refractory sarcoidosis: review." Therapeutics risk management 16 (2020): 323. DISCLOSURES: No relevant relationships Salim Daouk, source=Web Response Ahel Haj Chehade, Ahmad Hassan, Abed Madanieh, Chirin Orabi,

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ژورنال

عنوان ژورنال: Chest

سال: 2021

ISSN: ['0012-3692', '1931-3543']

DOI: https://doi.org/10.1016/j.chest.2021.07.1510