Iusova O I, Krivosheev O G, Semenkova E N, Kogan E A
I. M. Sechenov Moscow Medical Academy, Moscow.
Arkh Patol. 2000 Jul-Aug;62(4):51-4.
A postmortem case of cryoglobulinemic vasculitis is reported. A 67-year-old male had hemorrhagic component, affection of the kidneys, skin, lungs and gastrointestinal organs. The disease began in 1994 with extreme weakness, headache, fever, skin eruption. Antibodies to B and C hepatitis were found. The condition of the patient worsened since 1997 when renal deficiency reached the degree of uremia. Before the autopsy the diagnosis was: chronic glomerulonephritis with cryoglobulinemia in context of systemic vasculitis with involvement of the skin, gastrointestinal tract, fever syndrome. Pathomorphologically, the patient had proliferative vasculitis with leukoclasia in the skin, chronic mesangiocapillary glomerulonephritis in the kidney, fibrosing alveolitis and vasculitis with affection of small vessels in the lung, chronic hepatitis in the liver. The anatomopathological diagnosis was as follows: cryoglobulinemic vasculitis of the hemorrhagic vasculitis type with involvement of the kidneys, skin, gastrointestinal tract and the lungs. Differential diagnosis with other microscopic vascilitides: main distinctions of cryoglobulinemic vasculitis are immune deposits consisting of cryoglobulins in the wall of small vessels. The most frequent components of cryoglobulins are IgM and IgG. These are main morphological, classifying and differential-diagnostic signs.
报告了一例冷球蛋白血症性血管炎的尸检病例。一名67岁男性出现出血成分，累及肾脏、皮肤、肺和胃肠道器官。该病始于1994年，表现为极度虚弱、头痛、发热、皮疹。检测到乙肝和丙肝抗体。自1997年患者肾功能衰竭达到尿毒症程度后病情恶化。尸检前诊断为：慢性肾小球肾炎伴冷球蛋白血症，合并系统性血管炎累及皮肤、胃肠道、发热综合征。病理形态学上，患者存在皮肤增殖性血管炎伴白细胞破碎，肾脏为慢性系膜毛细血管性肾小球肾炎，肺为纤维化肺泡炎和小血管受累的血管炎，肝脏为慢性肝炎。解剖病理学诊断如下：出血性血管炎型冷球蛋白血症性血管炎，累及肾脏、皮肤、胃肠道和肺。与其他显微镜下血管炎的鉴别诊断：冷球蛋白血症性血管炎的主要区别在于小血管壁中由冷球蛋白组成的免疫沉积物。冷球蛋白最常见的成分是IgM和IgG。这些是主要的形态学、分类和鉴别诊断标志。
