We report the case of a 64-year-old woman with severe alveolar haemorrhage secondary to granulomatosis with polyangiitis (GPA), who was unknowingly treated with only penicillin and macrolide antibiotics, resulting in successful initial disease remission. The immunomodulatory effects of the macrolide antibiotic used could underlie this novel observation, which may shed new light on the pathogenesis and potential treatment of anti-neutrophil cytoplasm antibody (ANCA) associated vasculitis. The role of macrolides as an adjunct to conventional therapy in cases of AAV with respiratory involvement may be worthy of further investigation.