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 Retsch‐Bogart
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 well‐established
diagnosis and represents an entity in the ill‐defined 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 Aspergillus‐related airway
eosinophilic inflammation and speculate this unique presentation of
Aspergillus bronchitis, which may be the precursor of ABPA.
2 | CASE REPORT
A 7‐year‐old 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
2015‐2017 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;1–4. 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.