前言及简单文献复习本症为原因不明,较为少见,予后一般不佳之临床病理症候群。临床特点为病人多以呼吸道的症状开始,继而有肾小球肾炎及泛发性血管炎的表现,多数终因尿毒症或呼吸衰竭死亡。病理解剖上的特点为有呼吸道之坏死性肉芽肿,全身性坏死性血管炎及灶性坏死性肾小球肾炎之所见。病的初发虽属徐缓,但病程多较短,每在发病后5～6个月内,多数在三个月内死亡。 Preface and simple literature review of the disease is unknown, relatively rare, to post-clinical poor clinical pathology syndrome. Clinical features of patients with respiratory symptoms and more, followed by glomerulonephritis and vasculitis performance, the majority of patients eventually died of uremia or respiratory failure. Pathological anatomical features of respiratory necrosis granulomatous, systemic necrotizing vasculitis and focal necrotic glomerulonephritis seen. Although the initial onset of disease is slow, but shorter duration, each within 5 to 6 months after onset, the majority of death within three months.