儿童紫癜性肾炎的发病机制与治疗进展

Pathogenesis and therapy of pediatric Henock-Schonlein purpura nephritis

儿童紫癜性肾炎以含有免疫球蛋白IgA的免疫复合物在肾脏系膜区沉积为主要特征，临床表现为血尿和蛋白尿，是影响过敏性紫癜患儿预后最重要的因素。紫癜性肾炎患儿体内半乳糖基化缺陷的IgA水平明显升高，且以其为抗原产生相应的免疫球蛋白构成大分子量的免疫复合物，这种大分子免疫复合物在肾脏的沉积是促使儿童紫癜性肾炎发病的主要因素。儿童紫癜性肾炎的治疗以激素和免疫抑制疗法为主，激素治疗对儿童紫癜性肾炎的预防效果仍存在争议，但激素是治疗儿童紫癜性肾炎的一线药物，尤其对处于急进期肾炎有明显疗效，对激素治疗发生抵抗的患儿应考虑使用免疫抑制疗法，现有的免疫抑制剂中环磷酰胺有比较好的疗效，环孢素A的治疗效果尚待明确，硫唑嘌呤、霉酚酸酯、血管紧张素Ⅱ抑制剂、尿激酶等作为新兴的治疗儿童紫癜性肾炎的药物，仍需大量的试验研究来确定其治疗效果。

Henock-Schonlein purpura nephritis （HSPN） is characterized by the renal mesangial deposition of immune complexes containing IgA, with hematuria and proteinuria as its clinical feature, which is the most pivotal factor determining the prognosis of anaphylactoid purpura. The level of galactose-deficient IgA is elevated in the patients of HSPN. The immunoglobulin against such galactose-deficient IgA is synthetized. Furthermore, they bind with each other and the large-molecular mass immune complexes are formed, which is the major factor inducing HSPN. Steroid and immunosuppressive agents are the main therapeutic strategies for HSPN. The prophylactic effects of steroid are still conflicting. But steroid is the first-line treatment for HSPN, especially for rapid progressive nephritis. The immunosuppressive drugs should be considered when the steroid resistant occurs, among which cyelophosphamide is proved effective and cyclosporine A remains uncertain. Azathioprine, mycophenolatc motefil, angiotensin I1 inhibitors and urokinase are promising therapeutic drugs for HSPN. However, much work and researches are needed to confirm their therapeutic effect.