Therapy With Macrolides in Patients With Cystic Fibrosis

Allyson S. Gaylor, Pharm.D., Joan C. Reilly, Pharm.D.

Pharmacotherapy. 2002;22(2) 

In This Article

Clinical Benefits

In a long-term, low-dosage study, patients with DPB were given erythromycin 400-600 mg/day for at least 2 months (mean ± SD 14.2 ± 11.7 mo).[73] Significant increases in FEV1 (p<0.01), vital capacity (p<0.01), and partial pressure of oxygen (p<0.01) were observed after treatment. Sixteen of 19 patients had relief of productive cough and dyspnea, as well as disappearance of nodular shadows and improvement in overinflation on chest radiographs. Three patients had no change in symptoms or respiratory function after treatment and were considered nonresponders.

In an open study, children with end-stage cystic fibrosis lung disease and patients with chronic airflow limitation unresponsive to conventional therapy were treated with azithromycin for more than 3 months and showed a significant increase in FVC and FEV1.[43] Two abstracts also reported beneficial effects of azithromycin. Fourteen patients receiving azithromycin for an average of 22.3 months initially were treated with 250 mg every other day, but eight patients had the dosage empirically increased to 250 mg/day.[74] They experienced significant increases in FEV1 (p=0.002) and forced expiratory fraction 25-75 (p=0.02). Improvements were seen in FVC and percentage ideal body weight, although the differences were not statistically significant. Liver and renal functions were monitored closely throughout the study, and no patient experienced significant side effects. Resistance patterns and bacterial flora were not significantly altered. Improvements in pulmonary function (% predicted FEV1, p=0.007; % predicted FVC, p=0.003) and body weight (p=0.002) were reported after treatment with azithromycin compared with placebo.[75]


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