摘要
目的:回顾性分析Ⅳ型狼疮性肾炎(LN)伴肾间质血管血栓性微血管病变(TMA)患者的临床、病理及预后。方法:33例在解放军肾脏病研究所住院、经肾活检诊断为Ⅳ型LN(参照2003年ISN/RPS分型方案)伴肾间质血管TMA病变的患者,女性27例,男性6例,平均年龄(31.8±10.9)岁,回顾性分析其临床特点(肾脏表现、肾外脏器受累、免疫学异常及病理特征)及预后特征(近期疗效、远期人、肾存活率及终末期肾衰发生的危险因素)。结果:(1)27例(81.8%)入院即表现为肾功能不全[SCr(274.0±176.8)μmol/L],其中14例(42.4%)需要肾脏替代治疗。高血压、肾病范围蛋白尿、肉眼血尿的比例分别为93.9%、57.6%和24.2%。(2)微血管病性溶血性贫血(MHA)比例为60.6%,神经系统损害、心、肺受累及浆膜腔炎的发生率分别为:18.1%、36.3%、15.1%和66.7%。(3)血清ANA、抗ds-DNA及抗心磷脂抗体阳性率分别为90.9%,75.8%和33.3%。96.9%和42.4%的患者伴有低C3及C4血症。(4)肾活检病理分型Ⅳ(G)型25例(75.8%)、Ⅳ+Ⅴ型7例(21.2%)、Ⅳ(S)型1例(3.0%)。袢坏死、Fribin阳性的袢内血栓及新月体比例分别为:51.5%,69.7%和60.6%。20例(60.7%)肾间质小动脉有血栓形成。中-重度小管-间质慢性化损害者达78.8%。(5)患者中位随访时间为13月(1～101月),6例(42.9%)摆脱肾脏替代治疗,6月及12月治疗有反应率分别为38.9%和55.2%。死亡3例(感染、不明死因和多器官功能衰竭各1例),10例(38.5%)进展为终末期肾衰。患者1年、3年、5年人生存率分别为96.2%,69.2%和69.2%;肾存活率分别为:62.3%,62.3%和46.7%。结论:伴TMA的Ⅳ型LN患者肾脏损害严重,部分患者可无典型MHA表现,肾小球病理类型以Ⅳ(G)和Ⅳ+Ⅴ型多见,Ⅳ(S)型少见,患者预后差。应加强LN伴TMA的早期诊断,探索有效的治疗手段。
Objective：To investigate the clinicopathologic characteristics and outcomes in patients with Class Ⅳ lupus nephritis presented with thrombotic microangiopathy （TMA）. Methodology：A retrospective study was performed on patients presenting TMA in Class Ⅳ LN, using the ISN/RPS classification, from June 1987 to May 2006. Their clinicopathologic and outcomes were analyzed. Results： （1） Thirty three patients （F：27, M：6, mean age 31.8 years ±10. 9） were from 822 patients with Class Ⅳ LN. （ 2 ） Twenty seven cases （ 81.8% ） had renal dysfunction [ serum creatinine （SCr） ： （274. 0 ± 176. 8） μmol/L], and 14 （42.4%） required initial renal replacement treatment on admission. The nephrotic range of proteinuria, gross hematuria and hypertension were found in 19 （57.6%）, 8 （24. 2% ） and 31 （93.9%）, respectively. （ 3 ） The heart disorders （ 36. 3% ）, pulmonary lesions （ 15. 1% ）, neurological involvement （ 18. 1% ）, high incidence of microangiopathic hemolytic anemia （MHA） （ 60.6% ） and serositis （ 66. 7 % ） were observed,（4） Serum ANA, ds-DNA and anticardiolipin antibodies were positive in 90. 9%, 75. 8% and 33.3%, and low level of serum C3 and CA in 96.9% and 42. 4% of patients. （5） Renal biopsy showed class Ⅳ-G in 25 （75.8%）, Ⅳ superimposed on membranous lesion in 7 （21.2%）, and Ⅳ-S in one. The glomerular segmental necrosis, fibrin microthromb and crescents were found in 17 （51.5%）, 23 （69. 7% ） and 20 （60. 6% ）, respectively. 20 patients （60. 7% ） revealed arterial and/or arteriolar thrombosis and most patients （78.8%） showed moderate to severe degrees of tubular-interstitial lesion. （6） Patients were followed from 1 to 101 months （median 13 months）, only 50% of patients had response to treatment. 3 patients died, and 10 （38.5%）developed end stage renal failure. The lyr, 3yr and 5yr patient survival was 96. 2%, 69. 2% and 69.2%. The lyr, 3 yr and 5yr renal survival was 62. 3% ,62. 3% and 46. 7%. Conclusion： TMA in LN was associated with one of the most severe forms of glomerulonephritis, carried the most vicious prognosis.
出处
《肾脏病与透析肾移植杂志》
CAS
CSCD
2007年第4期322-328,共7页
Chinese Journal of Nephrology,Dialysis & Transplantation
