title: Differential cytokine profile in children with cystic fibrosis.
abstract: The previously observed occurrence of antineutrophil cytoplasmic autoantibodies (ANCA) in patients who have cystic fibrosis (CF), together with the reported decrease in IgG2, a Th1-controlled isotype, suggests a potential for Th1/Th2 imbalance in CF patients with a possible Th2 predominance. 48 CF patients and 16 controls had levels of IFNgamma, IL-4, and IL-10 measured in supernatants of whole blood cell cultures stimulated by lipopolysaccharide (LPS) and phytohemaglutinine (PHA). The patients were divided into 2 groups: "low responders", having negligible secretion of cytokines (IFNgamma: 10.0-200.0 pg/ml, IL-4: 0.0-0.3 pg/ml) and "high responders", producing high levels of both IFNgamma (500.0-2000.0 pg/ml) and IL-4 (1.0-200.0 pg/ml). There was a statistically significant (P < 0.01) deterioration of lung function measured by an FEV(1) decline by 11.2% over 3 years in the "low responder" group. 10 of 16 "low responders" had chronic lung infections with P. aeruginosa while such infection was less prevalent in the "high responder" group where only 13 of 32 CF patients had positive cultures. A shift towards Th2 response was observed in the "high responder" group as children chronically infected with P. aeruginosa had greater IL-4 production than non-infected CF patients within the same cohort. ANCA autoantibodies were found only in the "high responder" group. Th2 immune response predominance in a subset of CF patients is associated with chronic P. aeruginosa infection.
authors.affiliation: Institute of Immunology, University Hospital Motol, Prague, Czech Republic. jitka.brasova@centrum.cz
authors.forename: Jitka
authors.initials: J
authors.lastname: Brazova
authors.forename: Anna
authors.initials: A
authors.lastname: Sediva
authors.forename: Dagmara
authors.initials: D
authors.lastname: Pospisilova
authors.forename: Vera
authors.initials: V
authors.lastname: Vavrova
authors.forename: Petr
authors.initials: P
authors.lastname: Pohunek
authors.forename: Milan
authors.initials: M
authors.lastname: Macek
authors.forename: Jirina
authors.initials: J
authors.lastname: Bartunkova
authors.forename: Hynek
authors.initials: H
authors.lastname: Lauschmann
mesh_terms: D000293:Adolescent; D000328:Adult; D019268:Antibodies, Antineutrophil Cytoplasmic / Q000096:biosynthesis; D016022:Case-Control Studies; D002648:Child; D002675:Child, Preschool; D003550:Cystic Fibrosis / Q000276:immunology* / Q000382:microbiology / Q000503:physiopathology; D005260:Female; D006801:Humans; D007132:Immunoglobulin Isotypes / Q000097:blood; D007223:Infant; D007371:Interferon-gamma / Q000097:blood*; D016753:Interleukin-10 / Q000097:blood*; D015847:Interleukin-4 / Q000097:blood*; D008297:Male; D011552:Pseudomonas Infections / Q000453:epidemiology / Q000276:immunology* / Q000503:physiopathology; D012129:Respiratory Function Tests; D018418:Th2 Cells / Q000276:immunology
chemical_list: D019268:Antibodies, Antineutrophil Cytoplasmic; D007132:Immunoglobulin Isotypes; D016753:Interleukin-10; D015847:Interleukin-4; D007371:Interferon-gamma