1352例狼疮性肾炎的临床与免疫学特征

Clinical and immunological features of 1352 Chinese patients with lupus nephritis

目的：单中心分析1352例狼疮性。肾炎（LN）的流行病学特点、。肾脏病理类型与临床表现和免疫学异常的联系。方法：1986至2005年间在解放军。肾脏病研究所住院并行肾活检的LN患者1352例。肾活检病理类型按照2003年ISN／RPS分型方案，Ⅴ型与Ⅳ型或Ⅲ型并存的类型记录为Ⅴ＋Ⅳ型或Ⅴ＋Ⅲ型。临床资料来自病史记载，实验室指标取自首次肾活检时的检查结果。回顾性分析患者一般资料、病理类型分布、各病理类型的临床和免疫学特征。结果：1352例患者均为汉族，女性1193例（占88．2％），男性159例（11．8％），年龄8—60岁[平均（27．3±9．2）岁]，18—50岁者占80％。各病理类型比例分别为Ⅳ型49．1％，Ⅴ型14．4％、Ⅱ型14．2％、V＋Ⅳ型11．7％、Ⅲ型5．6％和Ⅴ＋Ⅲ型5．0％。女性Ⅱ型和Ⅲ型比例显著高于男性，男性Ⅳ型比例高于女性（59．7％vs47．6％，P〈0．01）。各类型。肾脏和。肾外脏器损害的临床及免疫学特征：Ⅱ型以蛋白尿，面部红斑、发热、关节损害为特征，血清抗-dsDNA抗体和低补体血症比例低；Ⅲ型患者主要表现为血尿，关节炎、皮肤血管炎和血清抗中性粒细胞胞浆抗体（ANCA）阳性率高（10．3％）。Ⅳ型患者肉眼血尿和急性。肾衰发生率最高，浆膜腔炎、血清抗-dsDNA抗体和低C4血症比例显著高于其它类型。Ⅴ型患者主要表现为蛋白尿或伴少量血尿，肾外脏器损害、血清抗-dsDNA抗体阳性和低C4血症比例低。Ⅴ＋Ⅳ型临床和免疫学特点与Ⅳ型类似，但。肾病性蛋白尿比例显著增高（52．6％掷31．6％），ANCA阳性率低（5．7％vs0）。Ⅴ＋Ⅲ型。肾病性蛋白尿比例与Ⅴ型相似，血尿程度与Ⅲ型相同，ANCA阳性和低C4血症比例低于Ⅲ型。抗心磷脂抗体可见于各型LN，但Ⅱ型、Ⅲ型和Ⅴ型阳性率较高（分别为34．1％，34．8％，32．6％）。结论：本组LN病理类型以Ⅳ型为主，Ⅴ型与Ⅳ或Ⅲ型并存者并不少见。且尿蛋白较单一病变者更重。Ⅲ型和Ⅱ型主要见于女性，男性狼疮Ⅳ和Ⅴ＋Ⅳ型比例高于女性。LN不同病理类型具有一定的临床和免疫学特征，反映了不同的免疫发病机制，从而影响免疫抑制治疗方案。

Objective：To analyze the clinical and immunological features of 1 352 Chinese patients with lupus nephritis in a single-center. Methodology：1352 LN patients in Jingling Hospital from 1986 to 2005 were included in this study. All patients had renal biopsy. The histological classifications were categorized according to 2003 ISN/RPS classification （Class Ⅰ and Ⅵ were not included in the study）. The cmbined class of Ⅳor Ⅲ and Ⅴwere recorded as Ⅳ＋ Ⅴor Ⅴ＋Ⅲ. Results：l 193 （88.2%） females, and 159（11.8%） males were all Han Chinese, 80% of them were from l8 to 50 years old （ mean 27.3 ＋ 9. 2 years）. The renal histological classes showed class Ⅱ （ 14. 2% ） ,Ⅲ （5.6%） , Ⅳ（49. 1%）, Ⅴ（14.4%）, Ⅴ＋Ⅳ（11.7%） and Ⅴ＋Ⅲ（5%）. The proportion of class Ⅱ （15.2%） and Ⅲ （6. 2%） in females was higher than that in males （6. 9% and 0. 6% respectively, P 〈0. 05） , while class Ⅳ was more common in males than that in females （59. 7% vs 47.6%, P 〈0.01 ）. The incidence of extra-renal manifestations including malar rash, fever, arthritis and anemia were higher in class Ⅱ than that in other types. The Serositis, positive anti-dsDNA antibody and low CA were more frequent in class Ⅳ and Ⅴ＋ Ⅳ, but less in class Ⅴ. The skin vasculitis and serum ANCA （ all MPO-ANCA） were more common in class Ⅲ（ 10. 3% ）, Ⅳ（5.7%） and Ⅴ＋ Ⅲ（3.2%）. The anticardiolipin antibodies were detected in all types but higher in class Ⅱ （34. 1%）, Ⅲ （34. 8%）, Ⅴ（32.6%） and type Ⅳ（26. 1%）, while Cryoglobulinemia was more prominent in class Ⅴ ＋ Ⅲ（69.4%）, type Ⅴ （59%） and Ⅴ ＋ Ⅳ （46. 3% ）. The patients with hematuria in class Ⅱ（18%） and Ⅴ （30%） were much less than that in Ⅳ （89%）, Ⅴ ＋Ⅳ （87%）, Ⅴ ＋ Ⅲ （79%） and Ⅲ （63%）, while gross hematuria and RPGN were only found in class Ⅳ （10%, 27%）, Ⅴ ＋ Ⅳ （6. 3%, 20. 9% ）, Ⅴ ＋ Ⅲ（6.3%, 6%） and Ⅲ （3.9%, 1.3%） but none in class Ⅱ and Ⅴ. The incidence ofnephrotic syndrome was higher in class Ⅴ ＋Ⅳ （52.5%） than that in class Ⅳ （31.6%）, class Ⅴ （34. 9%） and Ⅴ ＋ Ⅲ （35.8%）. Conclusion： Lupus nephritis class Ⅳ was the most common type in this study. The segmental or diffuse glo- merular proliferation combined with membranous lesion was also common and should be identified. The Sex difference influ- enced the pattern of histological types. The correlation between histological class and clinical-immunological features might be associated with unique immunopathogenesis.