Allergic Bronchopulmonary Aspergillosis

Paul McWhinney.Jan, 2001. Allergic bronchopulmonary aspergillosis [online]. Bradford Royal Infirmary, UK. Available from http://www.cysticfibrosismedicine.com

Patients with cystic fibrosis can become chronically colonised with Aspergillus fumigatus. The fungus can act as an allergen and induce a hypersensitivity reaction within the lungs giving rise to allergic bronchopulmonary aspergillosis (ABPA) (Becker et al, 1996; Neuveglise et al, 1997; Wojnarowski et al, 1997). This is often associated with blood eosinophilia and increased respiratory symptoms due to wheeze, mucus plugging, and non specific infiltrates or segmental collapse on chest X-ray. Early recognition is important in preventing long term complications.

The clinical features of ABPA may be masked or be mimicked by the respiratory symptoms of cystic fibrosis and is likely to result in an over-diagnosis. As many as 50% of CF patients may have an isolated positive serological test at some time. ABPA may be associated with fever (Type III hypersensitivity with positive precipitins). Beware of other causes of blood eosinophilia such as drugs, Churg Strauss, Wegeners, worms and tropical eosinophilia.

Treatment: Individuals with ABPA often respond well to oral prednisolone (Simmonds et al, 1990, Simmonds et al, 1994). The main treatment is non-enteric coated prednisolone 40-60mg for at least 2 weeks (1 mg/kg). Repeat CXR and if infiltrates cleared taper dose to 0.5 mg to 1 mg/kg prednisolone on alternate days for the next couple of months. With more frequent exacerbations, consider inhaled steroids. If this fails start a maintenance dose of 10mg/day prednisolone. If a diagnosis is made and severe symptoms are present or patient is steroid dependent try a course of itraconazole. In non CF individuals with steroid dependent ABPA, the addition of itraconazole can lead to significant clinical improvement without added toxicity (Stevens et al 2000). Some patients have been found to respond to nebulised amphotericin which reduces allergen load. It may be used when response to conventional therapy is poor (Sanchez-Sousa et al, 1996).

References

Becker JW, Burke W, McDonald G, Greenberger PA, Henderson WR, Aitken ML. Prevalence of allergic bronchopulmonary aspergillosis and atopy in adult patients with cystic fibrosis. Chest 1996; 109: 1536-1540

Neuveglise C, Sarfati J, Debeaupuis JP et al. Longitudinal study of Aspergillus fumigatus strains isolated from cystic fibrosis patients. Eur J Clin Microbiol Infect Dis 1997; 16: 747-750

Sanchez-Sousa A, Alvarez ME, Maiz L, Escobar H, Sadaba B, Suarez L et al. Control of Aspergillus bronchial colonisation in cystic fibrosis patients: preliminary data using Ambisone aerosol therapy. Israel J Med Sci 1996; 32: S256

Simmonds EJ, Littlewood JM, Evans EGV. Cystic fibrosis and allergic broncho-pulmonary aspergillosis. Arch Dis Child 1990; 65: 507-511

Simmonds EJ, Littlewood JM, Hopwood V, Evans EGV. Aspergillus fumigatus colonisation and population density of place of residence in cystic fibrosis. Arch Dis Child 1994; 70: 139-140

Stevens DA, Schwartz HJ, Lee JY, Morskovitch BL, Jerome DC, Catanzaro A, Bamberger DM, Weinmann AJ, Tuazon CU, Judson MA, Platt-Mills TAE, DeGraff AC. A randomised trial of itraconazole in allergic bronchopulmonary aspergillosis. N Eng J Med 2000;342:756-62

Wojnarowski C, Eichler I, Gartner C et al. Sensitization to Aspergillus fumigatus and lung function in children with cystic fibrosis. Am J Resp Crit Care Med 1997; 155: 1902-1907

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