Arimura Y, Minoshima S, Kamiya Y, Nakabayashi K, Kitamoto K, Nagasawa T
First Department of Internal Medicine, School of Medicine, Kyorin University, Tokyo, Japan.
Nihon Jinzo Gakkai Shi. 1992 Jun;34(6):731-8.
We report a case of microscopic polyarteritis nodosa associated with myeloperoxidase-antineutrophil cytoplasmic autoantibodies (MPO-ANCA). A 38 year-old female was admitted to our hospital, because of proteinuria, recurrent pyrexia, polyarthralgia, abdominal pain and purpura. She had a history of severe pulmonary hemorrhage and 4 kg weight loss for 8 months. On admission perinuclear ANCA without cytoplasmic ANCA was detected by indirect immunofluorescence assay and MPO-ANCA was detected by enzyme linked immunosorbent assay. But anti-nuclear antibodies, immune complexes and anti-glomerular basement membrane antibodies were not detected. Renal biopsy showed necrotizing crescentic glomerulonephritis without immune deposits. Skin biopsy revealed leukocytoclastic vasculitis. Diagnosis of microscopic polyarteritis nodosa was made by these clinical and histological evidence of vasculitis. As renal failure progressed after admission, corticosteroid and cyclophosphamide administration were started. Renal function and other symptoms improved paralleled with decreased MPO-ANCA titer to normal values. It is suggested that MPO-ANCA may be closely related to the pathogenesis of microscopic polyarteritis nodosa and it may be a good serological marker for diagnosis and disease activity of this disease.
我们报告一例与髓过氧化物酶抗中性粒细胞胞浆自身抗体(MPO-ANCA)相关的显微镜下结节性多动脉炎。一名38岁女性因蛋白尿、反复发热、多关节痛、腹痛和紫癜入住我院。她有严重肺出血病史,8个月内体重减轻4千克。入院时,通过间接免疫荧光法检测到核周型ANCA而无胞浆型ANCA,通过酶联免疫吸附测定法检测到MPO-ANCA。但未检测到抗核抗体、免疫复合物和抗肾小球基底膜抗体。肾活检显示为无免疫沉积物的坏死性新月体性肾小球肾炎。皮肤活检显示白细胞破碎性血管炎。根据这些血管炎的临床和组织学证据诊断为显微镜下结节性多动脉炎。入院后随着肾功能衰竭进展,开始给予皮质类固醇和环磷酰胺治疗。肾功能和其他症状随着MPO-ANCA滴度降至正常水平而改善。提示MPO-ANCA可能与显微镜下结节性多动脉炎的发病机制密切相关,且可能是该疾病诊断和疾病活动的良好血清学标志物。