Relationship between anti-inflammatory therapy and hepatobiliary abnormalities in cystic fibrosis patients

Anti-inflammatory therapy is a frequent but not obligatory component of cystic fibrosis (CF) lung disease treatment. The aim of this investigation was to compare the frequency of hepatobiliary abnormalities, the most common CF complication, in patients treated with anti-inflammatory drugs and those who did not receive anti-inflammatory treatment. This study involved a review of hospital medical records of CF patients (n = 183) younger than 19 years. Ninety-six patients were treated with basic therapy only and 87 individuals received besides basic therapy anti-inflammatory treatment with azithromycin (AZ; 48 patients) or prednisolone (PD; 39 patients) in the form of alternated course (0.3-0.5 mg/kg body weight every other day). Plasma levels of ACTH and cytokines (tumor necrosis factor-alpha, interferon-gamma, interleukin-10, transforming growth factor-beta 1) in CF patients were compared with those in healthy children. A lower frequency of hepatobiliary abnormalities in the subjects treated with PD or AZ compared to patients without anti-inflammatory therapy (WAT) was found. Cirrhosis was diagnosed in 38 of 96 WAT patients whereas only 7 of 39 (p = 0.007) and 4 of 48 (p = 0.01) children treated with PD and AZ, respectively, demonstrated such abnormality. Among 38 WAT patients with cirrhosis 24 demonstrated ultrasound changes, 11 portal hypertension, and 3 biliary cirrhosis. All studied CF patients revealed the same tendencies to change in ACTH and blood cytokine levels with the exception of interleukin-10, which was significantly higher in WAT patients than in normal children and anti-inflammatory drug treated CF individuals. In conclusion, anti-inflammatory therapy restores inflammation control and reduces the frequency of hepatobiliary abnormalities in CF patients.