Boudes P, Andre C, Belghiti D, Sobel A
Laboratory of Hematology, Henri Mondor Hospital, Creteil, France.
J Rheumatol. 1990 Oct;17(10):1412-4.
We describe a case of Wegener's granulomatosis in which the disease was manifested with crescentic glomerulonephritis, upper airway ulcerations, and microangiopathic hemolytic anemia with consumptive coagulopathy. No granuloma was identified but antibodies to cytoplasmic components of neutrophils were strongly positive with a diffuse pattern. Because microscopic vessels were predominantly involved (capillaritis), and granuloma were absent, were refer to this particular form of the disease as "microscopic Wegener's disease."
我们描述了一例韦格纳肉芽肿病,该疾病表现为新月体性肾小球肾炎、上呼吸道溃疡以及伴有消耗性凝血病的微血管病性溶血性贫血。未发现肉芽肿,但抗中性粒细胞胞浆成分抗体呈强阳性且为弥漫性模式。由于主要累及微血管(毛细血管炎)且无肉芽肿,我们将这种特殊形式的疾病称为“显微镜下型韦格纳病”。