University of Health Sciences, Bakırköy Dr. Sadi Konuk Training and Research Hospital Division of Rheumatology, Istanbul, Turkey.
Clin Ter. 2021 Sep 29;172(5):389-391. doi: 10.7417/CT.2021.2344.
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 macropha-ge 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. 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 macropha-ge 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.
肺泡蛋白沉积症（PAP）是一种罕见的肺部疾病，其特征是肺泡和远端气道中脂蛋白物质的积累。人们认为针对粒细胞巨噬细胞集落刺激因子（GM-CSF）的抗体与其发病机制有关。抗中性粒细胞胞质抗体（ANCA）相关血管炎（AAV）是一组影响小血管和中等血管的血管炎，常伴有肺和肾受累。在这里，我们介绍了一位 PAP 诊断患者，他已经缓解多年，现出现 AAV 并伴有肺泡性出血。
