Received: 15 January 2019 | Revised: 26 February 2019 | Accepted: 10 March 2019 DOI: 10.1002/ppul.24325 COMMENTARY Nonsystemic allergic bronchopulmonary aspergillosis in cystic fibrosis: A suggested paradigm for the evolution from topical to systemic disease Carolina Miranda 1 | Monica Cardenas 1 | Mariana Bedoya 1 | George RetschBogart 2 | Andrew A. Colin 1 1 Division of Pediatric Pulmonology, Miller School of Medicine, University of Miami, Miami, Florida 2 Division of Pediatric Pulmonology, University of North Carolina, Chapel Hill, North Carolina Correspondence Andrew A. Colin, Division of Pediatric Pulmonology, 1580 NW 10th Ave, Miami, FL 33136. Email: acolin@med.miami.edu KEYWORDS: allergic bronchopulmonary aspergillosis, ABPA, cystic fibrosis 1 | INTRODUCTION Allergic bronchopulmonary aspergillosis (ABPA) is a wellestablished diagnosis and represents an entity in the illdefined spectrum of fungal disease in patients with cystic fibrosis (CF). We are describing a case of a child with a unique presentation of recurrent eosinophilic inflammation of the airways with Aspergillus and diffuse airway obstruction with severe morbidity. He had positive immunoglobulin E (IgE) specific for Aspergillus fumigatus (AF), however, the conventional systemic markers of ABPA; notably increased total IgE, specific immunoglobulin G (IgG) for AF, and the skin testing were negative. We proposed to define this as localized Aspergillusrelated airway eosinophilic inflammation and speculate this unique presentation of Aspergillus bronchitis, which may be the precursor of ABPA. 2 | CASE REPORT A 7yearold boy presented with a lifetime diagnosis of CF, pancreatic insufficiency, chronic sinusitis (S/P four sinus surgeries), and hypogammaglobulinemia (on weekly subcutaneous immunoglo- bulin therapy). The child underwent multiple bronchoscopies predominantly for surveillance when he had endotracheal intubation for sinus surgery or performed to manage recurrent episodes of atelectasis. The bronchoalveolar lavage (BAL) fluid samples obtained from different lung segments in repeated bronchoscopies were negative for bacteria, except once when Haemophilus influenzae (01 May 2015) was isolated, treated, and subsequently resolved. The culture and cytologic analyses of bronchoscopies over the period 20152017 are shown in Table 1. Total serum IgE was followed routinely and was always within the normal range. Specific AF IgE was positive (1.3 kU/L), yet specific AF IgG and skin testing for Aspergillus were negative. He had intermittent eosinophilia in peripheral blood ranging from 9% to 15%. During 2015, he had two episodes of right middle lobe (RML) opacification consistent with atelectasis. He underwent broncho- scopy on the first occasion (September 2015) revealing RML bronchial mucosal erythema and scant secretions. Both events resolved with intensification of chest physical therapy (CPT), enhanced lung clearance, antibiotic therapy, bronchodilator inhala- tion, and systemic corticosteroids. During these events, he com- plained of chest tenderness over the right anterior chest during his routine CPT and his mother reported nocturnal sweating, both of which resolved as the radiographic abnormalities improved. In August 2016, he presented with sinus tenderness, increasing cough, and again complained of right chest tenderness with CPT. After failing to improve with 2 weeks of oral antibiotic therapy, he was admitted for treatment of acute CF pulmonary exacerbation and acute worsening of chronic sinusitis. He was treated with intravenous antibiotic therapy targeting the bacterial pathogens previously isolated from his sinuses; Pseudomonas aeruginosa and Methicillin sensitive Staphylococcus aureus. Pediatric Pulmonology. 2019;14. wileyonlinelibrary.com/journal/ppul © 2019 Wiley Periodicals, Inc. | 1 Abbreviations: ABPA, allergic bronchopulmonary aspergillosis; AF, Aspergillus fumigatus; BAL, bronchoalveolar lavage; CF, cystic fibrosis; CPT, chest physiotherapy; RML, right middle lobe. Miranda and Cardenas have contributed equally to this work.