Complications of COVID 19 in a Patient with Birt Hogg Dube Syndrome

Abstract

Birt Hogg Dube (BHD) syndrome is an autosomal dominant disease characterized by pulmonary cysts, spontaneous pneumothorax, skin hamartomas of the head/neck, and renal malignancies. We present a case of complications secondary to COVID 19 in a patient with BHD syndrome. A 55 year old male presented with fevers, chills and left sided pleuritic chest discomfort for 1 day. He was recently hospitalized for bacterial pneumonia and had COVID Pneumonia about 5 weeks ago. The patient was diagnosed with a lung bulla several months ago and is awaiting elective surgical resection. Vitals showed blood pressure 135/91 mmHg, temperature 36.5 Celsius, heart rate 74 and respiratory rate 18. Physical exam significant for decreased breath sounds of the left lower lobe. Labs showed white cell count 10.7 K/mcL, D-dimer 0.81 mcg/mL, lactic acid 0.7 mmol/L. SARS COV 2 PCR positive. Chest Xray showed left lower lobe multilobulated cavitary mass. CT Angiography Chest showed air-fluid level development within pre-existing large multiseptated bulla in the left lower lobe and dependently layering left pleural effusion (Figure 1). The patient was initiated on Vancomycin and Piperacillin-Tazobactam on admission. Thoracentesis was unable to be done due to low amount of pleural fluid. Patient stabilized with antibiotic treatment and supportive care, with subsequent discharge on a 2 week course of Piperacillin-Tazobactam. Diagnosis of BHD involves one or more of the following: greater than 2 or more fibrofolliculomas or trichodiscomas, multiple bilateral pulmonary cysts in the basilar lung regions, bilateral multifocal renal carcinomas or oncocytic renal tumors, pathogenic FLCN gene variant or family history of the disease [4]. Initial presentation of these patients is often via spontaneous pneumothorax. 70%-80% of BHD patients develop numerous, bilateral pulmonary cysts with majority having normal pulmonary function or mild obstructive disease [3,4]. On imaging, these thin-walled, irregularly shaped cysts are seen in the medial basilar lung regions. This is in contrast to the apical region air blebs seen in those patients with pneumothorax due to COPD or primary spontaneous pneumothorax. Our patient is unique in that he had recently developed COVID 19 Pneumonia with a superimposed bacterial pneumonia. The patient's previously known bullae became infected with subsequent abscess formation. To our knowledge, this is the first known case of Birt Hogg Dube Syndrome complicated by pulmonary cyst infection and abscess formation.