SESSION TITLE: Monday Medical Student/Resident Case Report Posters SESSION TYPE: Med Student/Res Case Rep Postr PRESENTED ON: 10/21/2019 02:30 PM - 03:15 PM INTRODUCTION: Chronic granulomatous disease (CGD) is a primary immunodeficiency disease characterized by recurrent bacterial and fungal infections. We report a case of Burkholderia pneumonia (PNA) in CGD refractory to broad-spectrum antibiotics and antifungals requiring further aggressive intervention. CASE PRESENTATION: A 23-year-old woman with a history of CGD presented with persistent fevers, dyspnea, and productive cough for one month. She was discharged from the hospital less than a week prior with cefepime and voriconazole for right middle lobe (RML) PNA. At the time, bronchioalveolar lavage (BAL) cultures (Cx) showed no growth and transbronchial biopsy showed acute and chronic inflammation. She has a history of Aspergillus left lower lobe PNA and Burkholderia lymphadenitis. She has no family history of immunodeficiency disease and does not smoke or use illicit drugs. Exam was only remarkable for cyclical symptomatic fevers to 101 F. Labs showed leukocytosis of 11.18x10ˆ3/uL, C-reactive protein 14.75 mg/dL, procalcitonin <0.02 ng/mL. Computed tomography (CT) of the chest showed persistent RML consolidation and right hilar and subcarinal lymphadenopathy. Vancomycin, voriconazole, and cefepime were started and her prophylactic trimethoprim-sulfamethoxazole (Bactrim) was held. Endobronchial ultrasound with fine needle aspiration of station 7 lymph node, cryobiopsy, and BAL in the RML showed polymorphous lymphocytes, noncaseating granulomas, and no growth in Cx. Prednisone 30 mg twice a day was added. Her symptoms improved, but on hospital day 8, she had recurrent cough and fevers to 102.8 F with worsening RML consolidation. Wedge resection by thoracic surgery showed caseating granulomas and granulomatous lymphadenitis. Five days later, tissue Cx showed Burkholderia multivorans sensitive to ciprofloxacin and Bactrim, which were started, leading to symptom resolution. She was discharged with IV ciprofloxacin and voriconazole for 4 weeks, IV Bactrim for 6 weeks, and 6-week steroid taper. Chest CT after 4 weeks showed resolving consolidation of the right lung and no LAD. DISCUSSION: Pneumonia accounts for 79% of infections in CGD, the majority by Aspergillus spp. Our case illustrates the diagnostic dilemma encountered with CGD as fevers and abnormal chest imaging can occur from both CGD flares and infectious etiologies that have markedly different treatments. Our patient had refractory symptoms despite previously being on treatment with Bactrim, for which her Burkholderia was sensitive to, prior to the admission. CONCLUSIONS: In CGD, it is common for infections to be undetected by standard Cx, but in our patient she had multiple nondiagnostic studies from BAL and small and large tissue samples taken by bronchoscopy. Aggressive intervention to identify the cause and provide source control is essential when a patient has persistent symptoms and fevers. Reference #1: Song, E. et al. (2011). Chronic granulomatous disease: a review of the infectious and inflammatory complications. Clin and Mol Allergy, 9(1), p.10 Reference #2: Winkelstein, J. et al. (2000). Chronic granulomatous disease: report on a national registry of 368 patients. Medicine, 79(3):155-69 Reference #3: Seger, R. (2008). Modern management of chronic granulomatous disease. British Journal of Haematology, 140, 255-66 DISCLOSURES: No relevant relationships by Kimberly Cao, source=Web Response No relevant relationships by David Rutenberg, source=Web Response No relevant relationships by Yumeng Zhang, source=Web Response