Abstract:
Pulmonary alveolar proteinosis (PAP) is a rare pulmonary disorder characterized by surfactant accumulation in the alveolar spaces while sarcoidosis is a multisystem granulomatous disease of unknown etiology. The occurrence of PAP and sarcoidosis in the same patient is rare. A 37-year-old woman presented with cough and breathlessness and was diagnosed to have autoimmune PAP. She responded well to subcutaneous injections of recombinant granulocyte macrophage colony stimulating factor. Three years later, she developed fever, chest pain, cough, and facial palsy. The evaluation revealed a diagnosis of sarcoidosis that responded to immunosuppressive treatment. We discuss the link between PAP and sarcoidosis and review the literature on this association.
摘要:
肺泡蛋白沉积症(PAP)是一种罕见的肺部疾病,其特征是表面活性剂在肺泡腔中积聚,而结节病是一种病因不明的多系统肉芽肿性疾病。在同一患者中很少发生PAP和结节病。一名37岁的妇女出现咳嗽和呼吸困难,并被诊断出患有自身免疫性PAP。她对皮下注射重组粒细胞巨噬细胞集落刺激因子反应良好。三年后,她发烧了,胸痛,咳嗽,和面神经麻痹.评估显示结节病的诊断对免疫抑制治疗有反应。我们讨论了PAP与结节病之间的联系,并回顾了有关这种联系的文献。