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Keywords:

  • allergic bronchopulmonary aspergillosis (ABPA);
  • Aspergillus fumigatus;
  • cystic fibrosis;
  • itraconazole;
  • liver function;
  • precipitating antibodies;
  • treatment

Background:  Allergic bronchopulmonary aspergillosis (ABPA) in cystic fibrosis (CF) patients is a potentially fatal inflammatory disease due to the dual-type immune response provoked by the fungal antigens. Despite serious side effects long-term treatment with corticosteroids is often required. Itraconazole has been reported to be a useful steroid-sparing agent.

Methods:  In a retrospective follow-up of 21 CF patients from a total of 250 treated once or twice within a five-year study period (1994–98), 9 patients were treated with systemic glucocorticosteroids in combination with itraconazole and 12 patients were treated with itraconazole (200–600 mg/day) as monotherapy.

Results:  During treatment the percentage of Aspergillus fumigatus (AF)-positive sputum cultures significantly reduced ( P  < 0.05); precipitating antibodies to AF decreased significantly in all patients ( P  < 0.05); forced expiratory volume (FEV 1) increased to pre-exacerbation level; total IgE levels decreased in 42% of patients on monotherapy and in 56% on combination therapy. Specific IgE (radioallergosorbant; RAST) level decreased in 6 of 21 patients. Eleven patients had transient increased levels of alanine transaminase (ALAT). One patient had isolated increase in alkaline phosphatase and another in aspartate transaminase (ASAT).

Conclusions:  High dose itraconazole as monotherapy or in combination with systemic glucocorticosteroids seems effective in CF patients with ABPA. No hepatotoxicity was observed during long-term therapy.