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Cystic fibrosis and allergic bronchopulmonary aspergillosis
E J Simmonds1, J M Littlewood, E G Evans
1Regional Cystic Fibrosis Unit, St James's University Hospital, Leeds.
Abstract:
We have prospectively screened our patients with cystic fibrosis for allergic bronchopulmonary aspergillosis. Over a three year period eight patients were identified, an incidence of 5.8%. Patients were clinically well at the time of diagnosis (Shwachman scores 70-90, Chrispin-Norman chest x ray scores 2-15) and they responded rapidly to treatment with oral prednisolone. There has been little deterioration in their respiratory function and nutrition over the study period. We conclude that allergic bronchopulmonary aspergillosis is not uncommon in patients with cystic fibrosis. It is a potential cause of lung damage and prospective screening could lead to earlier detection and treatment.
Insights
Allergic bronchopulmonary aspergillosis is common in cystic fibrosis patients, with screening identifying cases early. Prompt treatment with oral prednisolone preserves respiratory function and nutrition.
Area of Science:
- Pulmonology
- Infectious Diseases
- Allergy Immunology
Background:
- Cystic Fibrosis (CF) is a genetic disorder affecting lungs and other organs.
- Allergic Bronchopulmonary Aspergillosis (ABPA) is a hypersensitivity reaction to Aspergillus fumigatus.
- Early detection of ABPA in CF patients is crucial to prevent lung damage.
Observation:
- A three-year prospective screening study was conducted on cystic fibrosis patients.
- Eight cases of ABPA were identified, indicating an incidence of 5.8%.
- Patients diagnosed with ABPA were clinically stable with good Shwachman and Chrispin-Norman scores.
Findings:
- All patients with ABPA responded rapidly to oral prednisolone treatment.
- Minimal deterioration in respiratory function and nutritional status was observed post-treatment.
- ABPA is a significant comorbidity in cystic fibrosis patients.
Implications:
- ABPA is not uncommon in cystic fibrosis, necessitating proactive screening.
- Early diagnosis and treatment of ABPA can prevent irreversible lung damage.
- Implementing routine screening protocols for ABPA in CF care is recommended.