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目的分析冷球蛋白血症性肾炎的临床病理特点及预后。方法回顾性分析2002—2006年北京大学第一医院肾内科经肾活检诊断的8例冷球蛋白血症性肾炎的病例资料。结果 8例冷球蛋白血症患者中7例为中年男性,多数伴有系统受累表现,发病至确诊时间平均19个月,其中5例是在肾脏病理提示后查血冷球蛋白明确诊断;确诊时5例(5/8)蛋白尿程度已达肾病综合征,3例(3/8)已进展至肾衰竭;随访2年,4例(4/8)死亡,其中3例死于感染,1例死于肾衰竭。肾脏病理以膜增生性肾炎为主(7/8),4例(4/8)冷球蛋白血症继发于浆细胞病,仅1例继发于 HCV 感染。结论冷球蛋白血症性肾炎不罕见,但存在明显的临床认识不足与延误诊断,由于该病预后差,故对临床存在明显系统受累表现为血尿、蛋白尿患者应考虑本病,及早进行标准化的冷球蛋白测定有助于临床确诊。
Objective To analyze the clinicopathological characteristics and prognosis of cryoglobulinemic nephritis. Methods The data of 8 cases of cryoglobulinemic nephritis diagnosed by renal biopsy in Department of Nephrology, Peking University First Hospital from 2002 to 2006 were retrospectively analyzed. Results Among the 8 patients with cryoglobulinemia, 7 were middle - aged men, most of whom had systemic involvement. The average time from onset to diagnosis was 19 months, of which 5 were diagnosed clinically after renal pathology. Five cases (5/8) of proteinuria were confirmed to have nephrotic syndrome at the time of diagnosis, three cases (3/8) had progressed to renal failure; 2 years after follow-up, 4 (4/8) died and 3 died of infection , 1 died of renal failure. Kidney pathology was mainly based on membranoproliferative glomerulonephritis (7/8), and 4 (4/8) cryoglobulinemia secondary to plasma cell disease, only 1 case secondary to HCV infection. Conclusion Cryoglobulinemic nephritis is not uncommon, but there is a clear lack of clinical understanding and delay diagnosis. Due to the poor prognosis of the disease, patients with clinically significant manifestations of hematuria and proteinuria should consider this disease and standardize as soon as possible The determination of cryoglobulins contributes to the clinical diagnosis.