Chest
Volume 130, Issue 1, July 2006, Pages 222-226
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Original Research
Aspergillus Bronchitis in Cystic Fibrosis

https://doi.org/10.1378/chest.130.1.222Get rights and content

Aspergillus fumigatus, a widely distributed spore-bearing fungus, is commonly grown in sputum cultures of patients with cystic fibrosis (CF). A fumigatus

may cause allergic bronchopulmonary aspergillosis (ABPA), a complex condition that leads to worsening of airway inflammation and progressive damage and is diagnosed by specific criteria. In this report, we present six CF patients with respiratory deterioration that did not respond to appropriate antibiotic treatment. All had had A fumigatus in sputum cultures but did not fulfill the criteria of ABPA. Treatment with antifungal agents was followed by improvement in clinical condition. We suggest that in patients with CF, A fumigatus should be considered as a pathogen that may directly cause respiratory exacerbations. Antifungal therapy should be considered when deteriorating respiratory function is not responding to antibacterial therapy and A fumigatus is growing in sputum cultures.

Section snippets

Materials and Methods

The patients received diagnosis and treatment from 2002 to 2003 at the CF Center, Hadassah University Hospital, Mount Scopus, Jerusalem, Israel (4 of 80 patients in this clinic) and Pediatric Regional Cystic Fibrosis Centre, St James’ and Seacroft University Hospitals, Leeds, UK (2 of 151 pediatric patients in this clinic). Specific IgE anti-A fumigatus were measured by enzyme-linked immunosorbent assay (Dr. Fooke Laboratories; Neuss, Germany) and were scored from 0 to 4: score 0, 0 to 0.34

Case 1

A 22-year-old man with CF (G524X/ΔF508) and pancreatic insufficiency (Table 1) presented after 6 months of clinical deterioration despite intensive physiotherapy, inhaled antibiotics, and several courses of treatment with IV antibiotics according to bacterial sensitivity. He had lost 5 kg of weight, from 54 to 49 kg, and showed decreased expiratory flow rates (FEV1, 32% of predicted). Breath sounds were wet with crackles over the lower lobes of both lung, but there were no wheezes. Sputum

Case 2

A 13-year-old boy with CF (3849 + 10kbC->T/ΔF508) and pancreatic sufficiency (Table 1) presented with a respiratory exacerbation with increased sputum production, weight loss, and decreased expiratory flow rates. Breath sounds were normal, with no crackles or wheezes. Sputum cultures grew P aeruginosa, M simiae, and A fumigatus. He was treated with oral ciprofloxacin and gentamicin inhalations and subsequently with IV antibiotics according to the sensitivity of the P aeruginosa culture. His

Case 3

A 15-year-old boy with CF (W1282X/ΔF508) and pancreatic insufficiency (Table 1) presented with increased sputum production, decreased expiratory flow rate, and weight loss. Breath sounds were reduced with a few dry crackles mainly in the left and right lower lobes. Sputum cultures grew P aeruginosa, M abscessus, and A fumigatus. There was no improvement in respiratory function tests or weight after treatment with IV antibiotics chosen according to the sensitivity profile of the P aeruginosa

Case 4

A 30-year-old woman with CF (W1282X/unknown), pancreatic sufficiency, and chronic sinusitis (Table 1) presented with increased cough and sputum production. No significant changes in expiratory flow rates or weight were observed. Lung examination revealed a few wet crackles in the left lung. IgE was 542 IU/mL; FEV1, 57% of predicted; ESR, 25 mm/h. Serum Aspergillus-specific IgE and skin-prick test results to A fumigatus were negative. Sputum culture grew A fumigatus, P aeruginosa, and B cepacia

Case 5

A 10-year-old boy, homozygous ΔF508 (Table 1), presented with a productive cough, severe exercise restriction, lethargy, and weight loss. Wheeze was not a feature. FEV1 fell from 79 to 47% of predicted over a 9-month period. Sputum samples were consistently positive for A fumigatus, and no other respiratory pathogens were identified. There was no improvement in clinical parameters or respiratory function with broad-spectrum IV antibiotics. Aspergillus-specific IgE was negative, while total IgE

Case 6

A 12-year-old boy (ΔF508\Y1898 + 1) [Table 1] presented with chest pain, breathlessness, a productive cough, and weight loss. FEV1 and FVC had fallen from 80 to 47% of predicted and 88 to 54% of predicted, respectively; and oxygen saturation decreased from 98 to 92% over a 3-month period. Sputum culture results were repeatedly positive for A fumigatus. Total IgE was 123 IU/mL, Aspergillus-specific IgE was negative, and eosinophil count remained normal. Aspergillus-precipitating antibodies

Discussion

In this report, six patients with CF and positive sputum culture findings for A fumigatus but not meeting criteria for ABPA had acute or subacute clinical deterioration. The patients did not respond to appropriate antibiotic treatment directed to microorganisms identified in sputum cultures, but responded over varying periods of time and degree to antifungal medications. All patients grew A fumigatus in sputum culture, but none met the criteria required for diagnosis of ABPA1 (Table 3).

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