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Treatment of M avium pulmonary disease requires a three-drug, macrolide-based regimen that is administered for 12 months beyond culture conversion. The regimen can be administered 3 days a week in non-cavitary, nodular bronchiectatic disease but should be given daily when cavitary disease is present. For treatment refractory disease, amikacin liposome inhalation suspension is added to the regimen. Parenteral amikacin or streptomycin should be administered in the setting of extensive radiographic involvement or macrolide resistance. Recurrence of disease is common and often due to reinfection. Novel and repurposed agents are being evaluated in clinical trials.