Lauque D, Estivals M, Tubery M, Pourrat J, Carles P
Services de médecine interne, C.H.U. Purpan, Toulouse.
Rev Pneumol Clin. 1993;49(6):277-82.
Microscopic polyarteritis is an idiopathic necrotising vascularitis of the small vessels. Manifestations include diffuse alveolar haemorrhage, segmentary necrotising glomerulonephritis, and a cutaneous, articular, neurological or digestive vascularitis. The absence of a histological granulomas and medium calibre vessel involvement distinguishes Wegener's syndrome from polyarteritis nodulosa. The diagnosis is facilitated when anti-neutrophil cytoplasm antibodies are found in the serum. These auto-antibodies are suspected to be the cause based on clinical and experimental data, although this aetiology has not been confirmed. Corticosteroids, immunosuppressors and plasmapheresis can improve the prognosis of this severe and evolving affection which may be fatal.
显微镜下多血管炎是一种小血管的特发性坏死性血管炎。临床表现包括弥漫性肺泡出血、节段性坏死性肾小球肾炎以及皮肤、关节、神经或消化系统血管炎。组织学上无肉芽肿且不累及中等口径血管可将韦格纳肉芽肿与结节性多动脉炎区分开来。血清中发现抗中性粒细胞胞浆抗体时有助于诊断。尽管这一病因尚未得到证实，但基于临床和实验数据怀疑这些自身抗体是病因。皮质类固醇、免疫抑制剂和血浆置换可改善这种严重且病情进展的疾病的预后，该病可能会致命。
