CFDB - Cystic Fibrosis DataBase

primary studies published RCT

Autoantibodies against bactericidal/permeability-increasing protein (BPI-ANCA) in cystic fibrosis patients treated with azithromycin.

Study design (if review, criteria of inclusion for studies)

Single centre randomised placebo controlled study.

Participants

18 CF patients (5.5-36.3 years, median 15.1). Diagnosis not clear, mean sweat chloride, 74.5 mg/L. One patient had 5T mutation (associated with a milder phenotype).

Interventions

Azithromycin (10 patients) 250 mg twice a week for 12 weeks versus placebo (8 patients).

Outcome measures

BPI-ANCA levels in the blood (a possible marker of inflammation). Also respiratory function. Also secondary outcomes not fully reported (authors contacted). Overall weight gain in both groups, but wide range and differences not reported.

Main results

BPI-ANCA was found in 12 CF patients (67%) and four (22%) healthy subjects (P<0.001). The mean BPI-ANCA level was 3.94+/-6.15 U/ml (mean+/-SD) in healthy subjects and 38.11+/-42.34 U/ml in CF patients (P=0.023). The mean BPI-ANCA level was higher in patients with Pseudomonas aeruginosa compared to those without (64+/-35 U/ml and 25+/-41 U/ml respectively, P=0.032). No change in BPI-ANCA levels occurred in the AZM-treated patients [35 (0-127) U/ml (median (range) and 30 (0-120) U/ml, respectively] or in the placebo group [10 (0-66) U/ml and 13 (0-83) U/ml, respectively]. BPI-ANCA levels are significantly higher in patients with CF compared to healthy controls.

Authors' conclusions

BPI-ANCA levels are higher among patients colonised with P. aeruginosa. Twelve weeks of AZM therapy did not lower the BPI-ANCA level in patients with CF.

Keywords: Adolescent; Adult; Anti-Bacterial Agents; Azithromycin; Child; pharmacological_intervention; placebo; Proteins; Bacterial Infections; Respiratory Tract Infections; Respiratory Tract Diseases; Infection; Pseudomonas aeruginosa; Pseudomonas; Macrolides; Anti-Inflammatory Agents; Anti-Inflammatory Agents - excl Steroids;