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胸腔病例(三八七)肉芽腫併多發性動脈炎合併肺部侵犯

摘要


肉芽腫性多血管炎(granulomatosis with polyangiitis, GPA)是系統性血管炎的一種,主要侵犯小血管。該疾病目前之病生理機轉仍不清楚,常侵犯上、下呼吸道、腎臟、神經系統、皮膚系統等。這種疾病常以非特異性系統性症狀表現,包括發燒、疲倦、食慾減退、體重下降等,因此時常被當作感染、關節炎及惡性疾病診療。透過典型器官侵犯、血清檢驗、器官切片檢驗出典型病灶達成診斷。此類疾病主要透過糖皮質素及免疫調節劑進行治療。

參考文獻


Watts RA, Mahr A, Mohammad AJ, et al. Classification, epidemiology and clinical subgrouping of antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis. Nephrology Dialysis Transplantation 2015;30:i14-22.
Kitching AR, Anders H-J, Basu N, et al. ANCA-associated vasculitis. Nature Reviews Disease Primers 2020;6.
Wu CS, Hsieh CJ, Peng YS, et al. Antineutrophil cytoplasmic antibody-associated vasculitis in Taiwan: A hospital-based study with reference to the population-based National Health Insurance database. J Microbiol Immunol Infect 2015;48:477-82.
Scott J, Hartnett J, Mockler D, et al. Environmental risk factors associated with ANCA associated vasculitis: A systematic mapping review. Autoimmun Rev 2020;19:102660.
Mendel A, Ennis D, Go E, et al. CanVasc Consensus Recommendations for the Management of Antineutrophil Cytoplasm Antibody-associated Vasculitis: 2020 Update. J Rheumatol 2021;48:555-66.

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