Abstrakt
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 IFN-γ, 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 (IFN-γ: 10.0-200.0 pg/ml, IL-4: 0.0-0.3 pg/ml) and "high responders", producing high levels of both IFNγ (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 FEV1 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 autoanitbodies 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.