Two cases of autoimmune and secondary pulmonary alveolar proteinosis during immunosuppressive therapy in dermatomyositis with interstitial lung disease
Two cases of autoimmune and secondary pulmonary alveolar proteinosis during immunosuppressive therapy in dermatomyositis with interstitial lung disease
复制标题
DOI：
10.3109/14397595.2016.1153443
复制
发表时间：
2018-01-01
影响因子：
2.2
通讯作者：
Mimori, Tsuneyo
中科院分区：
文献类型：
--
作者：
Imura, Yoshitaka;Yukawa, Naoichiro;Mimori, Tsuneyo
间质性肺病（ILD）合并皮肌炎通常需要强化免疫抑制治疗。在此，我们报告两例皮肌炎合并间质性肺病的肺泡蛋白沉积症。1例为继发性PAP，另1例为自身免疫性PAP，抗粒细胞巨噬细胞集落刺激因子抗体阳性。免疫抑制治疗期间出现PAP，减弱免疫抑制后症状消失。强化免疫抑制治疗期间肺部病变的加重可将PAP与ILD恶化区分开来，应考虑减弱免疫抑制。
Interstitial lung disease (ILD) with dermatomyositis often requires intensive immunosuppressive therapy. Here, we report two cases of pulmonary alveolar proteinosis (PAP) in dermatomyositis with ILD. One case was secondary PAP, and the other was autoimmune PAP positive for the anti-granulocyte macrophage-colony-stimulating factor antibody. PAP arose during immunosuppressive therapy and symptoms ceased by attenuating immunosuppression. Exacerbation of pulmonary lesions during intensive immunosuppressive therapy may distinguish PAP from worsening ILD and attenuating immunosuppression should be considered.