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具有类似于非特异性间质性肺炎特征的自身免疫性肺泡蛋白沉积症。

Autoimmune pulmonary alveolar proteinosis with features similar to nonspecific interstitial pneumonia.

作者信息

Fujii Koki, Takeshima Hideyuki, Nishimura Taku, Sakatani Toshio, Masuda Yoshio, Morikawa Teppei, Usui Kazuhiro

机构信息

Division of Respirology, NTT Medical Center Tokyo, Tokyo, Japan.

Department of Diagnostic Pathology, NTT Medical Center Tokyo, Tokyo, Japan.

出版信息

Respir Med Case Rep. 2022 Feb 3;36:101591. doi: 10.1016/j.rmcr.2022.101591. eCollection 2022.

Abstract

A 58-year-old woman with cough and dyspnea who was suspected of having idiopathic interstitial pneumonia had been treated with corticosteroids and cyclosporine, but the symptoms had worsened. There were no findings to suspect pulmonary alveolar proteinosis (PAP) in the bronchoalveolar lavage fluid, 17 months after the start of treatment. The transbronchial lung biopsy specimens showed eosinophilic bodies that strongly stained with periodic acid-Schiff staining. Anti-granulocyte macrophage colony-stimulating factor (anti-GM-CSF) antibodies were detected in her serum. We diagnosed the patient with autoimmune PAP. Thus, we present a rare case of PAP presenting atypical radiological images and bronchoalveolar lavage fluid findings.

摘要

一名58岁咳嗽、呼吸困难的女性,疑似患有特发性间质性肺炎,曾接受皮质类固醇和环孢素治疗,但症状仍加重。治疗开始17个月后,支气管肺泡灌洗 fluid中未发现提示肺泡蛋白沉积症(PAP)的 findings。经支气管肺活检标本显示嗜酸性小体,对过碘酸希夫染色呈强阳性。在她的血清中检测到抗粒细胞巨噬细胞集落刺激因子(抗GM-CSF)抗体。我们诊断该患者为自身免疫性PAP。因此,我们报告一例罕见的PAP病例,其呈现非典型的影像学图像和支气管肺泡灌洗 fluid findings。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/497c/9076885/c60d360674e4/gr1.jpg

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