PDF(Pubmed)
Abstract：
Pulmonary alveolar proteinosis (PAP) is a rare disease which involves the accumulation of insoluble lipoproteinaceous material in the alveoli leading to impaired gas exchange and even respiratory failure. Autoimmune PAP is the most common type and is characterized by the presence of anti-granulocyte-monocyte colony stimulating factor (anti GM-CSF) antibody. Whole lung lavage has been traditionally used as first-line management of PAP but there is a lack of clarity especially in the treatment of relapsing cases of PAP. Rituximab is an anti Cluster of Differentiate 20 (CD 20) monoclonal antibody that has been tried as salvage therapy for relapsing cases of PAP. We present a case of 35 years old female patient who was diagnosed as a case of relapsing PAP who was managed initially with neoadjuvant rituximab. This is a retrospective observational report showing novel use of neoadjuvant rituximab in a difficult case of relapsing PAP.
摘要：
肺泡蛋白沉积症（PAP）是一种罕见的疾病，涉及肺泡中不溶性脂蛋白物质的积累，导致气体交换受损甚至呼吸衰竭。自身免疫性PAP是最常见的类型，其特征在于存在抗粒细胞-单核细胞集落刺激因子（抗GM-CSF）抗体。传统上，全肺灌洗已被用作PAP的一线管理，但缺乏清晰度，尤其是在PAP复发病例的治疗中。利妥昔单抗是一种抗分化簇20（CD20）单克隆抗体，已被尝试用作PAP复发病例的挽救疗法。我们介绍了一例35岁的女性患者，该患者被诊断为复发性PAP，最初接受新辅助利妥昔单抗治疗。这是一项回顾性观察报告，显示了新辅助利妥昔单抗在复发性PAP的困难病例中的新用途。
