Abstract
Pulmonary Alveolar Proteinosis (PAP) is a rare lung disease characterized by accumulation of a lipoproteinous material in the alveoli and distal airways. Antibodies against granulocyte macrophage colony stimulant factor (GM-CSF) are thought to be responsible for its pathogenesis. Antineutrophilic cytoplasmic antibody (ANCA) associated vasculitis (AAV) is a group of vasculitis affecting small and medium vessels and often characterized by lung and kidney involvement. Here, we present a patient with PAP diagnosis who has been in remission for many years, developing AAV and associated pulmonary alveolar hemorrhage.