继发性IgA肾病常见病因及其病理特征分析

Diseases associated with IgA nephropathy and the clinicopathological features

目的:分析继发性IgA肾病(IgAN)的病因、肾活检组织学及免疫病理特征。方法:回顾性分析解放军肾脏病研究所1985年3月至2007年2月符合以下条件:(1)组织学及免疫病理改变符合IgAN特点:主要病变为系膜区增宽、系膜细胞和(或)基质增多,无弥漫肾小球外周袢病变,可伴节段外周袢病变如袢坏死、瘢痕或新月体;(2)免疫荧光染色以系膜区IgA沉积为主,可伴IgG、IgM或C3沉积,不包括弥漫外周袢IgA沉积者;(3)存在与肾脏病变相关的其他疾病。患者总计1324例。分析引起上述病变的病因、肾活检组织学改变特点、肾小球免疫球蛋白沉积的种类与部位以及组织学改变、免疫球蛋白沉积种类、部位与尿液检查之间的关系。结果:(1)本组引起肾小球继发性IgA沉积的病因包括:过敏性紫癜(88.5%)、银屑病相关肾损害(1.9%)、甲状腺疾病(1.9%)、乙型肝炎病毒感染(1.7%)、类风湿性关节炎(1.4%)、干燥综合征(1.2%)、强直性脊柱炎(1.2%)等;(2)本组患者肾脏受损的相似的组织学特点是均存在肾小球系膜区病变,肾小球毛细血管袢的病变多为节段性分布,急性病变为血管袢纤维素样坏死、新月体形成;慢性化病变则为肾小球毛细血管袢瘢痕形成导致肾小球球性/节段废弃;此外肾小球内皮细胞肿胀、成对,球内浸润细胞亦较常见。除这些共同的组织学改变外,不同病因的继发性IgAN还有其各自的组织学特点;(3)除IgA沉积外,尚见多种免疫球蛋白沉积即IgA合并IgG或IgM沉积,IgA、IgG、IgM均可阳性,免疫球蛋白分布的部位从单纯系膜区延及节段外周袢;(4)组织学病变轻、重与肾小球免疫球蛋白沉积部位、种类及尿液改变有一定联系。结论:继发性IgAN是一组病因不同的疾病,他们有相似的组织学、免疫病理特征,但也有其各自的特点。

Objective：To investigate the etiology, histopathological and immunofluorescence features in patients with secondary IgA nephropathy. Methodology ： 1324 patients from Mar. 1985 to Feb 2007 were enrolled in this retrospective study. All of the patients were met the following diagnosis criteria： 1 ） the light microscopic examination showed an increase in both mesangial cells and matrix, including mesangial expansion, with or without segmental peripheral loops lesions such as necrosis, scar and crescents. 2） Immunofluorescence microscopy revealed the definitive characteristic dominant or co-dominant deposits of IgA in mesangial area, IgG, IgM and/or C3 might be also present, and not included IgA deposition in diffuse peripheral loops. 3 ） The renal lesions resulted in original diseases. The etiology, histological, immunofluorescent features and uremia analysis were investigated and compared. Results：l ）In this study, the patients suffered from original diseases were Henoch-Schoenlein purpura in 1 172 （ 88. 5% ）, Psoriasis Vulgaris in 25 （ 1.9% ）, Thyroid Gland disease in 25 （ 1.9% ）, Hepatitis B infection in 22 （ 1.7% ）, Rheumatoid arthritis in 22 （ 1.4% ）, Sjogren syndrome in 16 （1.2%）, ankylosing spondylitis in 16 （1.2%） and so on. 2 ） In histological examination, all patients presented mesangial lesions, with or without segmental peripheral loops lesions. The acute lesions showed fibrlnoid necrosis and crescents, and the chronic lesions were peripheral loops scar. The swelling and pairing endothelial cells and infiltrating cells were also found. In addition to, the patients with original disease had the respective features. 3 ） The diffuse IgA deposition in mesangial area was the defining hallmark. IgG and/or IgM might be coeposited. IgA, IgG and IgM might also be found in the same distribution. Immunoglobulin deposit might prolong the segmental peripheral loops occasionally. 4） The degree of pathological lesions was associated with the site and category of immunoglobulin deposit and urinalysis. Conclusion： Secondary IgA-nephropathy was a series of the original diseases. Although the similar histological and immunofluorescent features presented in these patients, there were respective features of original diseases.