1. Summary of included studies.
| Study Name | Design | Intervention | Duration | Dosing Regimen | Number | Age | PA infection | Risk of bias | Primary Outcome | Main results |
| Equi 2002 | RCT X‐over | azithromycin versus placebo | 6 months each arm (2 month washout) | 250 mg/day (500 mg if weight >40 kg) | 41 | 8‐18 years | +/‐ | Low | comparative change in FEV1 | mean relative difference of 5.4% in favour of azithromycin |
| Wolter 2002 | RCT Parallel | azithromycin versus placebo | 3 months | 250 mg/day | 60 | adults | +/‐ | Low | relative change in FEV1 | mean difference 3.6% in favour of azithromycin |
| Saiman 2003 | RCT Parallel | Azithromycin versus placebo | 6 months | 250 mg 3 times a week (500 if weight >40kg) | 185 | 6‐adults | + | Low | relative change in FEV1 | mean difference, 6.2% in favour of azithromycin |
| Rotschild 2005 | RCT Parallel | azithromycin versus placebo | 3 months | 250 mg twice a week | 21 | 5‐36 years | +/‐ | Low | BPI‐ANCA levels | no difference |
| Clement 2006 | RCT Parallel | azithromycin versus placebo | 12 months | 250 mg 3 times a week (500 if weight >40 kg) | 82 | 6‐adults | +/‐ | Low | relative change in FEV1 | no difference |
| McCormack 2007 | RCT Parallel | weekly versus daily azithromycin | 6 months | 250 mg daily versus 1200 mg weekly | 208 | 6‐adults | +/‐ | Low | relative change in FEV1 | equivalence |
| Steinkamp 2007 | RCT Parallel | azithromycin versus placebo | 2 months | 500‐1250 mg weekly based on weight | 38 | 8‐adults | +/‐ | Low | absolute change in FEV1 | no difference |
| O'Connor 2009 | RCT X‐over | azithromycin versus placebo | 4 months each arm (2 month washout) | 250 mg 3 times a week (500 if weight >40 kg) | 17 | 6‐18 years | +/‐ | High | comparative change in FEV1 | no difference |
| Kabra 2010 | RCT Parallel | azithromycin, low versus high dose | 6 months | 5 versus 15 mg/kg/day | 47 | children | +/‐ | High | change in FEV1 from baseline | no difference |
| Saiman 2010 | RCT Parallel | azithromycin versus placebo | 6 months | 250 mg 3 times a week (500 if weight >36 kg) | 260 | 6‐18 years | ‐ | Low | relative change in FEV1 | no difference |
BPI‐ANCA: bactericidal or permeability‐increasing protein FEV1: forced expiratory volume at one second PA: Pseudomonas aeruginosa RCT: randomised controlled trial X‐over: cross‐over