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Survey of criteria used to diagnose allergic bronchopulmonary aspergillosis in cystic fibrosis
  1. S CUNNINGHAM,
  2. S L MADGE,
  3. R DINWIDDIE
  1. Portex Anaesthesia
  2. Intensive Care and Respiratory Medicine
  3. Great Ormond Street Hospital for Children
  4. Great Ormond Street, London WC1N 3JH, UK
  5. steve.cunningham{at}talk21.com

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    Editor,—Allergic bronchopulmonary aspergillosis (ABPA) creates a difficult diagnostic and management problem in patients with cystic fibrosis (CF). The six major diagnostic criteria for ABPA in CF were adapted from asthma guidelines.1Retrospective studies report significant variability in prevalence and the numbers of criteria for diagnosis.2 This is important as CF databases (UK CF database, European Registry, and the North American CF database) report ABPA frequency either without ascertaining the criteria used, or using limited diagnostic criteria. We have assessed consensus current practice of criteria used by UK clinicians to support a diagnosis of ABPA and how cases were treated.

    This retrospective, descriptive postal questionnaire survey was addressed to senior consultants in the 58 CF specialist clinics identified by the UK CF Trust.

    A total of 45 replies were received (78%); three were illegible/incomplete. Results are based on 42 replies (72%) from 14 adult clinics (33%), 23 paediatric (55%) clinics, and five (12%) mixed adult/paediatric clinics. Units had a median of 100 patients (interquartile range (IQR) 63 to 160).

    Of six ABPA major criteria investigations (table 1), centres routinely tested (at least yearly) a median of four (mode five).

    Table 1

    Replies to questionnaire (% of all units)

    Clinicians were also asked how many of eight factors (table 1) associated with ABPA diagnosis must be present, are preferred to be present, or were not considered important. It was considered that a median of two factors (IQR 1 to 4) must be present, three preferred to be present (IQR 2 to 5), and one factor was not considered important (IQR 1 to 2.3). Forty per cent of centres considered one or more further factors in addition to those provided.

    Thirty eight per cent of centres would begin treatment without clinical deterioration (62% treat on deterioration). Initial treatment in all centres (100%) was prednisolone: in paediatric patients 1 mg/kg in 21% and 2 mg/kg in 76%; in adults 30 mg/day in 50% (range 20–60 mg/day). In response to failure of steroid treatment 33% would add an antifungal agent, 17% would increase steroid dose (17% no experience of steroid failure, 12% other, 21% no reply). Oral antifungals had been used by 69% of respondents, itraconazole in all cases. Paediatric centres were much more likely to use oral antifungals (88%v 31%, p = 0.004, Mann–Whitney U test). Nebulised antifungals were used by 21%, amphotericin in all cases.

    We also asked how many patients would currently be diagnosed as having ABPA in that unit using: (a) criteria stated as “must be present” earlier in the questionnaire; and (b) if major criteria were strictly adhered to. Clinicians considered that they had a median of 5% of patients with ABPA (IQR 1 to 8), using their own criteria, falling to a median of 0% (IQR 0 to 3) when all major criteria were strictly adhered to.

    This questionnaire shows considerable variability in the criteria used to diagnosis ABPA in CF. Prospective reporting of cases with defined criteria will be the only way to reliably identify the true prevalence of ABPA. Database surveys may overestimate the true prevalence.

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