Empyema in a Woman with Cystic Fibrosis: A Cautionary Tale

Cystic fibrosis (CF) is a disease which predisposes individuals to recurrent infective exacerbations of suppurative lung disease; however, empyema is a rare complication in these patients. Empyemas secondary to Staphylococcus aureus and Burkholderia cepacia have been described in patients with CF. We report the case of pleural empyema with mixed S. aureus and Pseudomonas aeruginosa infection in a 34-year-old woman with CF, which was managed with ultrasound-guided pigtail catheter insertion, fibrinolysis, and antibiotic therapy. Physicians should be aware of this unusual complication in CF patients, especially those receiving an immunosuppressive therapy. 1. Introduction Cystic fibrosis (CF) is an autosomal recessively inherited genetic disorder caused by mutations of the CF transmembrane conductance regulator (CFTR) gene. Abnormalities of the respiratory epithelium result in dehydrated, thickened secretions which provide a favorable environment for infection [1]. Although at one point considered a pediatric disease, as of 2008 greater than 45% of United States CF patients were greater than 18 years of age [2]. Typically, airway infection starts with H. influenzae and S. aureus with progression to chronic infection with mucoid Ps. aeruginosa in the vast majority of patients. Infection with more than one organism is common. Parapneumonic effusion is an accumulation of exudative pleural fluid associated with an ipsilateral pulmonary infection. This is a relatively common consequence of pneumonia in those without CF, occurring in 20–40% of non-CF patients admitted to the hospital with pneumonia [3]. An effusion is referred to as an empyema, when the concentration of leucocytes becomes macroscopically evident as thick and turbid fluid. A positive pleural fluid gram stain or culture also defines an empyema. CF patients rarely develop parapneumonic effusions or empyemas, despite their chronic airway infection. Taussig et al. [4] described 4 infants with CF who developed empyema secondary to S. aureus infection. Others have reported empyema in post-lung transplant patients [5, 6]. There has also been a single case report of pleural empyema in an immunocompetent adolescent with CF, chronically infected with Ps. aeruginosa and S. aureus [7]. We present a case of pleural empyema in an adult with CF who had mild lung disease at baseline and was not a transplant recipient. She was, however, receiving low-dose immunosuppression for treatment of allergic bronchopulmonary aspergillosis (ABPA). As anti-inflammatory therapy is further adopted as part of routine CF care,