210例原发性IgA肾病临床病理分析

The clinicopathologic analysis of 210 patients with idiopathic IgA nephropathy

目的探讨原发性IgA肾病患者的临床表现、病理特点及其相关性。方法回顾性总结分析本院1999年1月至2010年6月经肾活检确诊为原发性IgA肾病的210例患者的临床表现及病理特点。结果506例肾活检患者中IgA肾病210例，检出率为41．5％。患者平均年龄为28．9±10．3岁，以20～39岁为高发，占59．5％（125／210）。其临床表现多样化，非大量蛋白尿伴水肿和（或）高血压者最多，为83例（39．5％）；其次为大量蛋白尿表现为肾病综合征者57例（27．1％）；血肌酐升高表现为慢性肾衰竭者24例（11．4％）。病理上包括多种原发性肾小球疾病的病理类型，以局灶节段增生型或弥漫系膜增生型最为常见，分别为27．6％（58／210）、23．3％（49／210）。免疫病理方面IgA强度以＋＋最为常见，约占35．2％，＋＋＋次之，约占31．4％。单纯IgA阳性者占13．3％（28／210）；合并IgG沉积者13例（6．2％）；95例患者合并IgM的沉积（45．2％）；合并补体C3沉积者最为多见，占80．5％（169／210）。结论IgA肾病在中青年发病率高，危害性较大。其临床表现及病理改变多种多样，且两者不完全相一致。临床疑是IgA肾病患者建议尽早肾活检以明确诊断、积极治疗，阻止或延缓慢性肾脏病进展。

Objectives To study the clinical manifestation, pathological features and their correlation in patients with idiopathic IgA nephropathy（IgAN）. Methods The clinicopathological data of 210 biopsy - proven cases of IgAN from Jan. 1999 to Jun. 2010 in our hospital were retrospectively analyzed. All the patients exclude secondary IgAN such as lupus nephritis, purpura nephritis, sjogren syndrome, HBV -associated glomerolonephritis. Results There were 210 patients with IgAN in 506 patients who accepted renal biopsy and the frequency of IgAN was 41.5%. 56.2% patients were male and 43.8 % female. The average age of the patients was 28.9 ± 10.3 years old. The incidence of IgAN was the highest in patients at the age of 20± 29 years（59.5% ）. Clinically, chronic nephritis syndrome type was the most common clinical manifestation （39.5 % ,83/210 ）, followed by nephritic syndrome （27.1% ,57/210） , chronic renal failure（ 11.4% ） ,gross hematuria（ 10.9% ） and latent glomerulonephritis （ 10. 9% ）. The pathological types of IgAN included various pathological types of primary glomerular disase. The frequency of focal proliferative type （ 27.6% ,58/210 ） was higher than those of diffuse proliferative type （ 23.3 % ,49/ 210 ）, minor lesion type （ 15.7% , 33/210） , and early sclerotic type （ 13.8% , 29/210）. The IgA fluorescence intensity of ＋ ＋ was the most common seen in IgAN, accounting for 35.2% and ＋ ＋ ＋ was the next in order, accounting for 31.4%. Immunopathological results showed that merely staining of IgA in mesangial area was in 28 patients, accounting forl3.3%. Staining of IgA combined with IgG was in 6.2% of 210 patients. Staining of IgA combined with IgM was in 45.2%. 169 patients had C3 deposit, accounting for 80.5%. Conclusions The high incidence of IgAN occurs on the period of youngs. IgAN has diverse clinical manifestation and pathological changes, the former didn＇t correspond with the latter completely. The prognosis is assoeiated with pathological changes closely. Early renal biopsy should be performed in the patients who are doubted to be IgAN and appropriate therapy administered according to the pathological features in order to put off the development of chronic kidney diseases.