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ABPA Dr S Sen SpR Respiratory Paediatrics Booth Hall Hospital

Allergic Bronchopulmonary Aspergillosis : 

Allergic Bronchopulmonary Aspergillosis Allergic bronchopulmonary aspergillosis (ABPA) is a lung disease resulting from a hypersensitivity to Aspergillus fumigatus,clinically characterized by impaired mucociliary clearance, mucoid impactions, episodic bronchial obstruction, and pulmonary infiltrates. ABPA may afflict patients with asthma (2), and an increasing prevalence of ABPA in patients with cystic fibrosis (CF), Reported incidence of colonisation in CF ranges from 5% to almost 100%,with a cumulative incidence of approximately 50% & prevalence of ABPA approximately 1 to 15%

Why CF patients affected? : 

Why CF patients affected? In CF, the abnormal mucus might promote the trapping of A. fumigatus spores within the bronchial airway, presumably promoting growth of A. fumigatus mycelia, thus contributing to an increased A. fumigatus colonization of the lung.

What does it do to the lungs? : 

What does it do to the lungs? The inflammatory process initiated by the immune responses to fungal colonization is related to the production of specific IgE and IgG antibodies against A. fumigatus The consequences of such an airway inflammatory process may be a progressive course of development of pulmonary hyperinflation,ventilation inhomogeneities, chronic obstructive lung disease,trapped gases, and gas exchange disturbances.

Diagnostic Criteria : 

Diagnostic Criteria Clinical pulmonary deterioration that does not respond to antibacterial treatment, New and flitting pulmonary infiltrates, Mucoid impaction or central bronchiectasis on chest radiography, Elevated IgE (> 1,000 ng/mL) Presence of specific IgE and IgG anti-A fumigatus, Elevated eosinophil count >(1000 cells/microl) Positive skin-prick test result to A fumigatus allergen Growth of A fumigatus in sputum cultures or Bronchoalveolar lavage cultures

Treatment : 

Treatment The first line medical treatment of ABPA is Steroids Steroids- Two weeks of daily therapy of oral prednisone (0.5 mg/kg/day), followed by gradual tapering, has been recommended for new ABPA infiltrates The first-line antifungal treatment for A fumigatus is itraconazole,a triazole antifungal agent with a broad activity spectrum Voriconazole is a newer drug Total serum IgE serves as a marker of ABPA disease activity, and should be checked 6–8 weeks after the initiation of therapy, then every 8 weeks for 1 year after that to determine a baseline range for each individual patient

Papers related to ABPA in CF : 

Papers related to ABPA in CF Effect of Allergic Bronchopulmonary Aspergillosis on Lung Function in Children with Cystic Fibrosis Richard Kraemer, Natascha Delose´a, Pietro Ballinari, Sabina Gallati, and Reto Crameri Departments of Paediatrics and Psychiatry, and Division of Human Genetics, University of Bern, Bern; and Swiss Institute of Allergy and Asthma Research, Davos, Switzerland Methods: Serial annual lung function tests performed in 122 children with CF (62 males; 60 females; age: 6–18 yr) provided data pertainingto FRC measured by plethysmography, lung clearance index,volume of trapped gas, effective specific airway resistance, and forced expiratory indices (FEV1, FEF at 50% VC). Conclusions: Airway narrowing, gas trapping, and small airway disease are the major targets for functional derangement in ABPA.

Slide 8: 

Allergic Bronchopulmonary Aspergillosis in Cystic Fibrosis -Role of Atopy and Response to Itraconazole Ilynn B. Nepomuceno, MS; Silvia Esrig, MS; and Richard B. Moss, MD, Study objectives: (1) To determine the relationship between IgE levels and the prevalence of allergic bronchopulmonary aspergillosis (ABPA) in cystic fibrosis (CF) patients, (2) to establish the usefulness of assessing atopy as an identifying risk factor for ABPA, (3) to evaluate the clinical course of patients receiving and not receiving itraconazole as reflected in oral steroid dose requirements and number of acute episodes of ABPA, and (4) to determine the role of acute episodes of ABPA in pulmonary exacerbations of CF. Patients: One hundred seventy-two patients with CF for whom serial serum total IgE levels were measured over a 5-year study period, 1992 to 1996. Conclusions: Screening for atopy may be a cost-effective way to select CF patients for periodic monitoring with total serum IgE levels, since there is an increased risk of ABPA developing in atopic CF patients. Itraconazole treatment of ABPA is safe and associated with fewer acute episodes of ABPA despite reduction in average daily oral steroid dose.

Slide 9: 

Voriconazole therapy in children with cystic fibrosis Tom Hilliard a,*, Siaˆn Edwards b, Roger Buchdahl a, Jacqueline Francis a, Mark Rosenthal a,Ian Balfour-Lynn a, Andrew Bush a, Jane Davies Department of Paediatric Respiratory Medicine, Royal Brompton Hospital, London, UK,Department of Pharmacy, Royal Brompton Hospital, London, UK Methods: A retrospective case note review of children with CF treated with voriconazole in a single centre over an 18 month period. Conclusions: Voriconazole may be a useful adjunctive therapy for ABPA in CF. Voriconazole monotherapy appears to be an alternative treatment strategy when oral corticosteroids may not be suitable.

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