Foo Chuan Tai, Chhor Louis, Thien Francis
Eastern Health Melbourne VIC Australia.
Eastern Health Clinical School Monash University Melbourne VIC Australia.
Respirol Case Rep. 2020 May 5;8(5):e00569. doi: 10.1002/rcr2.569. eCollection 2020 Jul.
We report the first case of autoimmune pulmonary alveolar proteinosis (PAP) associated with and preceding myelodysplastic syndrome. A 74-year-old female with a history of polymyalgia rheumatica presented with six months history of progressive exertional breathlessness. Examination revealed bilateral chest crackles and exertional desaturation. A diagnosis of autoimmune PAP was made based on the presence of autoantibodies to granulocyte-macrophage colony-stimulating factor and characteristic findings on chest computed tomography, bronchoalveolar lavage, and transbronchial biopsies. Bilateral whole lung lavage was performed with prompt improvement in symptoms. Fourteen months later, she presented with new breathlessness and was diagnosed with myelodysplasia on bone marrow biopsy. No recurrence of alveolar proteinosis was detected. This case highlights the importance of follow-up and screening of patients with autoimmune PAP for haematological conditions.
我们报告了首例与骨髓增生异常综合征相关且先于该综合征出现的自身免疫性肺泡蛋白沉积症（PAP）病例。一名有风湿性多肌痛病史的74岁女性，出现进行性劳力性呼吸困难6个月。检查发现双侧胸部有啰音及劳力性血氧饱和度下降。基于存在抗粒细胞巨噬细胞集落刺激因子自身抗体以及胸部计算机断层扫描、支气管肺泡灌洗和经支气管活检的特征性表现，诊断为自身免疫性PAP。进行了双侧全肺灌洗，症状迅速改善。14个月后，她出现新的呼吸困难，骨髓活检诊断为骨髓增生异常。未检测到肺泡蛋白沉积症复发。该病例强调了对自身免疫性PAP患者进行血液系统疾病随访和筛查的重要性。
