Marwah Vikas, Choudhary Robin, Devarakonda Bhargava V, Malik Virender
Professor & Head (Pulmonary, Critical Care & Sleep Medicine), Army Institute of Cardio Thoracic Sciences (AICTS), Pune, India.
Senior Resident (Pulmonary, Critical Care & Sleep Medicine), Army Institute of Cardio Thoracic Sciences (AICTS), Pune, India.
Med J Armed Forces India. 2024 May-Jun;80(3):369-371. doi: 10.1016/j.mjafi.2022.10.005. Epub 2022 Nov 28.
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.
肺泡蛋白沉积症(PAP)是一种罕见疾病,其特征是肺泡内积聚不溶性脂蛋白物质,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,其特点是存在抗粒细胞-单核细胞集落刺激因子(抗GM-CSF)抗体。传统上,全肺灌洗一直被用作PAP的一线治疗方法,但在治疗PAP复发病例时,情况并不明确。利妥昔单抗是一种抗分化簇20(CD 20)单克隆抗体,已被尝试用于治疗PAP复发病例的挽救治疗。我们报告一例35岁女性患者,被诊断为复发性PAP,最初采用新辅助利妥昔单抗治疗。这是一份回顾性观察报告,展示了新辅助利妥昔单抗在复发性PAP疑难病例中的新应用。