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累及肺和肾的肉芽肿性疾病:诊断与治疗难题

Granulomatous Disorder With Pulmonary and Renal Involvement: A Diagnostic and Therapeutic Dilemma.

作者信息

Khan Merina, Saleem Nida, Mahmud Syed Nayer, Haneef Muhammad, Bukhari Hadia

机构信息

Nephrology, Shifa International Hospital Islamabad, Islamabad, PAK.

出版信息

Cureus. 2022 Mar 14;14(3):e23149. doi: 10.7759/cureus.23149. eCollection 2022 Mar.

DOI:10.7759/cureus.23149
PMID:35444884
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC9009989/
Abstract

Granulomatosis with polyangiitis (GPA) can present with a wide array of clinical signs and symptoms; therefore, it should be differentiated from other mimicking clinicopathological entities. We report a case of a 66-year-old gentleman who was found to have a mediastinal mass and histopathological examination showed chronic necrotizing granulomatous inflammation. The patient was managed on lines of pulmonary tuberculosis for 12 months and remained in remission for two years. Later, workup showed cytoplasmic antineutrophil cytoplasmic antibodies (c-ANCA)-associated granuloma with marked renal impairment, which responded to immunosuppression. From this, we suggest that in a patient with radiological evidence of mediastinal mass, the remote possibility of GPA must be kept in mind.

摘要

肉芽肿性多血管炎(GPA)可表现出各种各样的临床体征和症状;因此,它应与其他具有相似临床病理特征的疾病相鉴别。我们报告一例66岁男性患者,其被发现有纵隔肿块,组织病理学检查显示为慢性坏死性肉芽肿性炎症。该患者按照肺结核治疗方案治疗了12个月,并缓解了两年。后来,检查发现其存在与胞浆型抗中性粒细胞胞浆抗体(c-ANCA)相关的肉芽肿,且有明显的肾功能损害,经免疫抑制治疗后有反应。由此,我们建议,对于有纵隔肿块影像学证据的患者,必须考虑到GPA这种罕见的可能性。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/ed13/9009989/a6dbf02e89ef/cureus-0014-00000023149-i02.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/ed13/9009989/f702e889d1a0/cureus-0014-00000023149-i01.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/ed13/9009989/a6dbf02e89ef/cureus-0014-00000023149-i02.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/ed13/9009989/f702e889d1a0/cureus-0014-00000023149-i01.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/ed13/9009989/a6dbf02e89ef/cureus-0014-00000023149-i02.jpg

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Mycophenolate mofetil versus cyclophosphamide for remission induction in ANCA-associated vasculitis: a randomised, non-inferiority trial.霉酚酸酯与环磷酰胺诱导 ANCA 相关性血管炎缓解的随机对照非劣效试验。
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