1. ¹Respiratory Disease Division, Department of Clinical and Biological Sciences, University of Turin, Turin, Italy
2. ²Division of Genetics, San Giovanni Battista Hospital, Turin, Italy
3. ³Department of Genetics, Biology and Biochemistry, University of Turin, Turin, Italy
4. ⁴Cystic Fibrosis Centre, Istituti Clinici di Perfezionamento, University of Milan, Milan, Italy
5. ⁵Cystic Fibrosis Centre, Children's Hospital, Brescia
6. ⁶Cystic Fibrosis Centre, OIRM Sant'Anna Hospital, Turin, Italy
7. ⁷Institute of Paediatrics, University of Milan, Milan, Italy

Correspondence: Dr V De Rose, Clinica di Malattie dell'Apparato Respiratorio, Dipartimento di Scienze Cliniche e Biologiche, Università di Torino, Ospedale S.Luigi Gonzaga, Regione Gonzole, 10, 10043 Orbassano, Torino, Italy. Tel: +39 011 9026 416; Fax: +39 011 9038674; E-mail: virginia.derose@unito.it

Received 3 July 2004; Revised 22 July 2004; Accepted 23 July 2004; Published online 15 September 2004.

Abstract

It has been suggested that genes other than CFTR could modulate the severity of lung disease in cystic fibrosis (CF). Neutrophil Fc receptor II (FcRII) is involved in host defense against microorganisms and in inflammatory response. We evaluated the association between genetic variability of this gene and both airway infection with Pseudomonas aeruginosa and severity of lung disease in patients with CF. We studied 167 Italian unrelated patients with CF and 50 control subjects. The distribution of FcRIIA genotypes in CF patients was compared with that in control subjects and the different genotypes were related with the presence or absence of P. aeruginosa infection and markers of disease severity in CF patients. The distribution of FcRIIA genotypes was not significantly different between CF patients and controls. We observed that in CF patients with the same CFTR genotype (F508/F508), those carrying the R allele of FcRIIA had an increased risk of acquiring chronic P. aeruginosa infection (P=0.042, R.R.: 4.38; 95% CI: 1.17÷22.4). Moreover, the frequency of R/R genotype in patients with chronic P. aeruginosa infection seems to be higher than that of control subjects and patients without chronic infection. The observation that CF patients carrying the R allele of FcRIIA are at higher risk of acquiring chronic P. aeruginosa infection suggests that the FcRII loci genetic variation is contributing to this infection susceptibility.