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利妥昔单抗治疗肺泡蛋白沉积症:一例报告

Rituximab Therapy in Pulmonary Alveolar Proteinosis: A Case Report.

作者信息

Fikri Oussama, Hindi Meryem, Amro Lamyae

机构信息

Department of Pulmonology, Centre Hospitalier Universitaire (CHU) Mohammed VI, Arrazi Hospital, Faculté de Médecine et de Pharmacie de Marrakech, Laboratoire de Recherche Morpho Sciences, Université Cadi Ayyad (FMPM, Labo LRMS, UCA), Marrakech, MAR.

出版信息

Cureus. 2024 Nov 17;16(11):e73836. doi: 10.7759/cureus.73836. eCollection 2024 Nov.

Abstract

Autoimmune pulmonary alveolar proteinosis (PAP) is a rare lung condition characterized by the accumulation of surfactant proteins within the alveoli, leading to respiratory distress. We describe a 49-year-old female homemaker with a history of passive smoking and exposure to wood smoke and pigeon droppings. She presented with a dry cough and progressive dyspnea, experiencing significant deterioration in her condition over one year. Chest imaging revealed bilateral alveolar-interstitial syndrome with ground-glass opacities and alveolar condensations. Biopsy findings indicated type II pneumocytes and eosinophilic material, confirming a diagnosis of autoimmune PAP. After ruling out secondary causes of PAP, rituximab was administered successfully, leading to marked improvement in respiratory function and significant regression of radiological lesions within two months. This case highlights the importance of early diagnosis and treatment of autoimmune PAP, demonstrating the potential efficacy of rituximab in managing this challenging condition.

摘要

自身免疫性肺泡蛋白沉积症(PAP)是一种罕见的肺部疾病,其特征是肺泡内表面活性物质蛋白积聚,导致呼吸窘迫。我们描述了一名49岁的女性家庭主妇,有被动吸烟史,接触过木烟和鸽粪。她出现干咳和进行性呼吸困难,病情在一年多时间里显著恶化。胸部影像学显示双侧肺泡-间质综合征,伴有磨玻璃影和肺泡实变。活检结果显示II型肺泡上皮细胞和嗜酸性物质,确诊为自身免疫性PAP。排除PAP的继发原因后,成功给予利妥昔单抗治疗,两个月内呼吸功能显著改善,放射学病变明显消退。该病例强调了自身免疫性PAP早期诊断和治疗的重要性,证明了利妥昔单抗在治疗这种具有挑战性疾病方面的潜在疗效。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/c4d7/11650403/1d123fdf121d/cureus-0016-00000073836-i01.jpg

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