过敏性紫癜性肾炎与IgA肾病的临床及病理的对比研究

Clinicopathological study of Henoch-Schonlein purpura nephritis and IgA nephropathy

目的对比研究过敏性紫癜性肾炎(HSPN)和免疫球蛋白A(IgA)肾病在临床、实验室检查及肾脏病理改变上的异同,探讨两者的关系。方法对经肾活检证实的210例IgA肾病及67例HSPN患者进行详细的临床及病理对比分析。结果两组患者临床表现类型分布上相似,但HSPN肾外症状多,均有皮肤紫癜,59·2%有胃肠症状,46·7%有关节痛,而IgA肾病仅3·2%有腹痛。在肾脏病理改变上,IgA肾病46·2%出现球性硬化、40·0%节段硬化,HSPN分别为4·5%及7·5%,但HSPN有56·7%出现节段内皮增生,IgA肾病仅12·9%,差异均有非常显著意义(P<0·01)。电镜下HSPN电子致密物稀疏、松散,沉积部位分布较为广泛,位于肾小球系膜、内皮下甚至基底膜内,而IgA肾病电子致密物成密集团块状,主要局限于系膜区及旁系膜区。HSPN患者中42例(62·7%)肾小球免疫沉积物中含有IgG,而IgA肾病肾小球免疫沉积物中有IgG沉积的仅为8·1%,其多为IgA伴IgM和(或)C3沉积。平均随访24个月,62·7%HSPN完全缓解,而IgA肾病在随访20个月时仅18·6%完全缓解,48·6%留有无症状性血尿和(或)蛋白尿,23·3%为活动性肾病,差异有显著意义(P<0·05)。结论IgA肾病与HSPN在临床病理改变及转归上有着明显的差异,这种差异性不支持两者为同一疾病的假说,二者可能为有相似免疫异常的二个疾病实体。

Objective Henoch-Schonlein purpura nephritis （HSPN） and IgA nephropathy are very similar in irmnunopatho-logical changes, and therefore some nephrologists considered that they are substantially one disease entity caused by IgA immune abnormalities, and IgA nephropathy is, in fact, a kind of HSPN without rashes. The present study aimed to characterize their relationship through chnicopathological comparison between IgA nephropathy and HSPN. Methods Two hundred and ten patients with IgA nephropathy and 67 patients with HSPN were enrolled in tiffs study.Their clinical manifestations, blood biochemical test, serum immunology and follow-up data were collected and analyzed. Results The clinical patterns of IgA nepttropathy and HSPN were similar, but extra-renal manifestations were seen more often in HSPN, all of them had skin purpura, 59% had gastrointestinal symptoms and 47% suffered from arthralgia. While the abdominal pain occurred only in 3.2% patients with IgA nephropathy.The renal pathological investigation showed global sclerosis in 46.2 % of IgA nephropathy and 4.5 % of HSPN, mesangial sclerosis in 40% of IgA nephropathy and 7.5% of HSPN, but endothelial proliferation in 56.7% of HSPN and 12.9% of IgA nephropathy.The differences were extremely significant （ P〈0.01 ） .The electronic dense deposits in HSPN were sparse, loose and widely spread in glomemlar mesangium,subendothelial area and even intrabasement membrane. While the deposits were dense, lumpy and mostly limited in mesangium and paramesangium in IgA nephropathy. IgG was found in glomerular immune deposits in 62.7 % of HSPN, but only 8.1% of IgA nephropathy.No IgG deposit was observed in 91.9% of IgA nephrothy, among them most showed IgA and IgM and/or C3 de.posit.The follow up date of average 24 months showed complete remission in 62.7% of HSPN and 18.6% in IgA nephropathy after 20 months followsup.Moreover 48.6% of IgA nephropathy had consistent hematuria and proteinuria, 23.3% had active nephritides, the difference was significant （P〈0.05） . Conclusions Significant elinicopatholngical differences were found between HSPN and IgA nephropathy, which does not support the one disease entity hypothesis. HSPN and IgA nephropathy are probably two diseases with similar immune abnormalities.