Allergic Bronchopulmonary Aspergillosis in Cystic Fibrosis

CORRESPONDENCE

Allergic bronchopulmonary aspergillosis in cystic ﬁbrosis

To the Editor: separately the immunological data, with and without clinical suspicion of ABPA, as they were not collated The main criticism arising from kastelik et al. [1] on separately. the study of allergic bronchopulmonary aspergillosis As regards the role of ABPA in disease progression, (ABPA) based on the data of the European Registry our study has shown that the decline in lung function of Cystic Fibrosis (ERCF) [2] regards the criteria of CF patients with ABPA is not more rapid than that adopted for the ABPA diagnosis: positive markers of observed in patients without ABPA, even if the ABPA hypersensitivity to Aspergillus fumigatus plus a phy- patients presented on average at the enrollment a sician9s suspicion that the patient has ABPA. We lower forced expiratory volume in one second value agree that the clinical suspicion is subject to some compared to the non-ABPA subjects. There is a diagnostic inaccuracy. However, the ERCF required strong suspicion that ABPA affects, as a rule, patients that the clinical suspicion was based to some extent on already clinically compromised: worse lung function "reversible bronchoconstriction, pulmonary infiltra- and nutritional status, more bacterial colonization tions, elevated serum immunoglobulin (Ig)-E and/or and more antibiotic treatment. However, a real IgG specific for A. fumigatus, peripheral eosinophilia understanding of the true role of ABPA on the (w1000 mL-1), recovery of A. fumigatus from sputum disease progression should be based on prospective or hyphae present on smear, and response to steroids". longitudinal studies of large cohorts of CF patients. The true problem for the ABPA diagnosis in cystic This is not possible, even with the best intentions, fibrosis (CF) is that ABPA shares many clinical through a wide CF registry supported by more than characteristics with poorly controlled CF lung disease, 200 CF centres. which makes the recognition of this entity very However, we believe that the allergic bronchopul- difficult [3, 4]. monary aspergillosis information, derived from the Airway obstruction/wheezing, fleeting pulmonary European Registry of Cystic Fibrosis database, has infiltrates and central bronchiectasis, which are still given, yet with some approximation, a panoramic the three classical clinical findings required for the view of allergic bronchopulmonary aspergillosis in ABPA diagnosis in non-CF patients [5], are really very European cystic fibrosis patients, which could stimu- common in CF patients. Conversely, this difficulty late both an improved attention to detect this was met in any publication concerning this issue in complication by cystic fibrosis physicians and further CF, also in the most recent ones, in which ABPA clinical and biological studies aimed to better define patients are characterized only on the basis of the true nature of this complication and its true immunological markers [6 – 8]. influence on the course of cystic fibrosis lung disease. We believe that a clear hypersensitivity to A. fumigatus, combined with a significant rise in the total serum IgE level and the presence of some clinical G. Mastella*, M. Rainisio#, H.K. Harms}, M.E. markers of disease, particularly if they are refractory Hodsonz, C. Koch§, J. Navarroƒ, B. Strandvik**, to antibiotics and regress after corticosteroid therapy, S.G. McKenzie## is at the moment the most acceptable compromise for *Cystic Fibrosis Center, Verona, Italy, #Statistics for Research SFR, Basel, Switzerland, }Children9s Hospital, Munich University, an epidemiological study to be carried out on a large z Germany, Cystic Fibrosis Dept, Brompton National Hearth & CF population, in which the large number of patients Lung Hospital, London, UK, §Dept Paediatrics, Rigshospitalet, tends to compensate for both overestimation and University Hospital, Copenhagen, Denmark, ƒDept Gastroenterol- underestimation of diagnosis. Even a recent study by ogy, R. Debre´ Hospital, Paris, France, **Dept Pediatrics, Go¨teborg Nepomuceno et al. [9], using similar criteria to our University, Go¨teborg, Sweden and ##Pharmaceutical Division F. study, showed a comparable ABPA prevalence in CF Hoffman – La Roche Ltd, Basel, Switzerland. of 9%. The future of ABPA diagnosis in CF relies perhaps on some serological markers which are still under study [10, 11]. Both Ig subclasses specific for A. References fumigatus [7, 8] and serum or skin IgE which bind to some A. fumigatus allergens, rAspf2, rAspf4 and 1. Kastelik JA, Aziz I, Redington AE, Morice AH. rAspf6 [10, 11], are promising specific markers for Allergic bronchopulmonary aspergillosis in cystic ABPA disease. However, these markers once again fibrosis. Eur Respir J 2001; 17: 156. have to be faced with the difficulty of characterizing 2. Mastella G, Rainisio M, Harms HK, et al. Allergic exactly the patients with ABPA before being accu- bronchopulmonary aspergillosis in cystic fibrosis. A rately validated as disease-specific. european epidemiological study. Eur Respir J 2000; In our study, it was not possible to present 16: 464 – 471. CORRESPONDENCE 1053

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