The spectrum ofNocardialung disease in cystic fibrosis

Abstract

Introduction: Nocardiosis in Cystic Fibrosis (CF) associated with chronic antibiotic therapy, a changing pulmonary microbiome, improving diagnosis and increasing length of survival.Its role as a pathogen is debated and a recent report suggested that it doesn9t require antibiotic treatment. In our experience, however, Nocardia infection in CF can sometimes lead to inexorable pulmonary deterioration and even result in lung transplantation. Aim: To describe the spectrum of CF pulmonary nocardiosis and to assess whether steroid treatment or Allergic Broncho Pulmonary Aspergillosis (ABPA) are associated with pulmonary nocardiosis in CF. Methods: A retrospective chart review from 2002 - 2013, at two CF centers in Israel. Sputum was acquired every clinic visit for Ziehl Neelsen staining and culture on blood agar for 3 weeks. we considered Nocardia to be eradicated if 3 consecutive sputum cultures were negative. Results: We reviewed charts of 200 CF patients (2 months-63 years). Eight (4%) had at least one positive sputum culture for Nocardia . Two patterns of Nocardia infection were observed: a) four patients experienced a stormy clinical course, with respiratory deterioration, one requiring lung transplantation. Multiple courses of antibiotics were required and eradiacation was successful only in two. 3 of them had associated ABPA; b) 4 patients had a milder course with no or gradual deterioration. Treatment with Trimetoprim-sulfamethoxazol was initiated if clinical symptoms were present. Conclusions: Nocardia is often associated with significant lung disease in CF, possibly associated with ABPA or steroids. Aggressive multi-drug therapy should be instituted early when there is such deterioration.