Ono Manabu, Saito Ryoko, Tominaga Junya, Okada Yoshinori, Ohkouchi Shinya, Takemura Tamiko
Respiratory Medicine Tohoku University, Graduate School of Medicine Sendai Japan.
Comprehensive Education Center for Community MedicineTohoku University, Graduate School of Medicine Sendai Japan.
Respirol Case Rep. 2017 Jul 21;5(5):e00255. doi: 10.1002/rcr2.255. eCollection 2017 Sep.
Association of fibrosis with autoimmune pulmonary alveolar proteinosis (aPAP) is rare. However, prognoses of such cases are poor and the process of the formation of fibrosis is still unknown. In this study, we report a case of aPAP with progressive fibrosis occurring in a 46-year-old woman. She had undergone several repetitions of whole lung lavage (WLL) for 7 years and granulocyte-macrophage colony-stimulating factor (GM-CSF) inhalation for 3 months; however, the progression of fibrosis was not hindered. Eventually, she was treated with bilateral lung transplantation. The computed tomography (CT) image suggested pulmonary fibrotic changes in her lung similar to usual interstitial pneumonia. However, the pathological analyses of explant lungs revealed that the fibrosis was not similar to ordinary interstitial pneumonias and suggested that the dysfunction of alveolar macrophage in removing the excess surfactant of alveolar spaces played an important role in the fibrogenesis in aPAP.
纤维化与自身免疫性肺泡蛋白沉积症（aPAP）的关联较为罕见。然而，此类病例的预后较差，且纤维化的形成过程仍不明晰。在本研究中，我们报告了一例发生在一名46岁女性身上的伴有进行性纤维化的aPAP病例。她接受了7年的多次全肺灌洗（WLL）以及3个月的粒细胞巨噬细胞集落刺激因子（GM-CSF）吸入治疗；然而，纤维化的进展并未得到阻止。最终，她接受了双肺移植治疗。计算机断层扫描（CT）图像显示她肺部的纤维化改变类似于普通间质性肺炎。然而，移植肺的病理分析表明，该纤维化与普通间质性肺炎并不相似，并提示肺泡巨噬细胞在清除肺泡腔过量表面活性剂方面的功能障碍在aPAP的纤维化形成中起重要作用。
