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维基百科,自由的百科全书

诊断

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在血液检查上,患者常会出现红细胞沉降率(ESR)上升、C反应蛋白(CRP)上升、贫血等现象。若患者的肾脏遭到进犯,病人的血清肌酸酐数值会上升,尿液检查则可以看到蛋白尿及血尿等情形。


患者的神经病变,由"肌电图"(EMG)可显示出一种"感觉运动性周围神经病"(sensorimotor peripheral neuropathy)。


An important diagnostic test is the presence of perinuclear antineutrophil cytoplasmic antibodies英语perinuclear antineutrophil cytoplasmic antibodies (p-ANCA) with myeloperoxidase specificity[1] (a constituent of neutrophil granules)

Depending on which organ is affected special tests can be performed, such as renal biopsy in patients with kidney failure or electromyography in patients with peripheral neuropathy [2]

Differential diagnosis

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The signs and symptoms of microscopic polyangiitis may resemble those of granulomatosis with polyangiitis (GPA) (another form of small-vessel vasculitis) but typically lacks the significant upper respiratory tract involvement (e.g., sinusitis) frequently seen in people affected by GPA.[来源请求]

治疗

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常规治疗涉及长效剂量的泼尼松,或与之交替性的使用或组合性使用的药物,如与细胞毒性药物、比如环磷酰胺硫唑嘌呤交替使用或组合成复方药物(Combination drug)使用。

血浆置换法(Plasmapheresis),也可以使用在急性期以消除ANCA抗体(Anti-neutrophil cytoplasmic antibody)。

至于ANCA血管炎的分类标准,则有DCVAS标准及



Treatment

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Immunsuppressive treatment is the gold standard management in order to achieve remission of the blood vessel inflammation that occurs in active microscopic polyangitis. The current immunosuppressive protocols consists of a combination of high dose of glucocorticoids in combination with either cyclophosphamide or Rituximab.[3] In cases of life threatening disease treatment with plasmapheresis can also be applied.

The immunosuppressive treatment is slowly tapered down under a period of several months but there is at the moment no consensus about the total duration of the therapy. Discontinuation of immunosuppression can be related to increased risk for disease flares.

  1. ^ Seishima M, Oyama Z, Oda M. Skin eruptions associated with microscopic polyangiitis. Eur J Dermatol. 2004, 14 (4): 255–8. PMID 15319159. 
  2. ^ Houben, Eline; Bax, Willem A.; van Dam, Bastiaan; Slieker, Walentina A.T.; Verhave, Gideon; Frerichs, Fenneke C.P.; van Eijk, Izhar C.; Boersma, Wim G.; de Kuyper, Guido T.M.; Penne, Erik L. Diagnosing ANCA-associated vasculitis in ANCA positive patients: A retrospective analysis on the role of clinical symptoms and the ANCA titre. Medicine. October 2016, 95 (40): e5096. ISSN 0025-7974. PMC 5059091可免费查阅. PMID 27749588. doi:10.1097/MD.0000000000005096 (英语). 
  3. ^ Geetha, Duvuru; Jefferson, J. Ashley. ANCA-Associated Vasculitis: Core Curriculum 2020. American Journal of Kidney Diseases. 2020-01-01, 75 (1): 124–137. ISSN 0272-6386. PMID 31358311. S2CID 198983998. doi:10.1053/j.ajkd.2019.04.031可免费查阅 (English).