Izzi Claudia, Ravani Pietro, Torres Diletta, Prati Elisabetta, Viola Battista Fabio, Guerini Simona, Foramitti Marina, Frascà Giovanni, Amoroso Antonio, Ghiggeri Gian Marco, Schena Francesco Paolo, Scolari Francesco
Divisione di Nefrologia, Università e Spedali Civili, Brescia, Italy.
Am J Kidney Dis. 2006 May;47(5):761-9. doi: 10.1053/j.ajkd.2006.01.010.
Immunoglobulin A (IgA) nephropathy is the most common form of glomerulonephritis worldwide. Familial and sporadic cases are recognized, and a locus associated with the familial form of the disease was mapped to chromosome 6. Recent data suggest the familial IgA nephropathy form may have a poorer outcome than the sporadic form.
We tested the hypothesis of unequal survival rates between the 2 forms of disease by analyzing time from biopsy to end-stage renal disease in patients of Italian ancestry; 589 patients with sporadic and 96 patients with familial IgA nephropathy.
Overall 10- and 20-year renal survival probabilities of the cohort as a whole were 71% and 50%, respectively. Macroscopic hematuria was the modality of clinical presentation in 51% of patients with familial IgA nephropathy and 39% of patients with sporadic IgA nephropathy. At univariable analysis, the sporadic form of IgA nephropathy was associated significantly with increased risk for renal death. However, patients with the sporadic form tended to be more hypertensive and diagnosed later, with signs of more advanced renal disease than those with familial disease at baseline. In the regression model, form of disease lost any independent effect. Only male sex, lower baseline glomerular filtration rate, greater proteinuria, and histopathologic score proved to be independent predictors of disease progression. Treatment with steroids or angiotensin-converting enzyme inhibitors was associated with improved outcomes.
Our study does not confirm that familial IgA nephropathy has a worse prognosis than the sporadic form. The similar renal phenotype may support a common pathogenic mechanism underlying familial and sporadic IgA nephropathy.
免疫球蛋白A（IgA）肾病是全球最常见的肾小球肾炎形式。已识别出家族性和散发性病例，且与该疾病家族形式相关的一个基因座已被定位到6号染色体。近期数据表明，家族性IgA肾病形式的预后可能比散发性形式更差。
我们通过分析意大利裔患者从活检到终末期肾病的时间，来检验这两种疾病形式生存率是否不同的假设；589例散发性IgA肾病患者和96例家族性IgA肾病患者。
整个队列的总体10年和20年肾脏生存率分别为71%和50%。肉眼血尿是51%的家族性IgA肾病患者和39%的散发性IgA肾病患者的临床表现形式。在单变量分析中，散发性IgA肾病形式与肾脏死亡风险增加显著相关。然而，散发性形式的患者往往血压更高且诊断较晚，与基线时的家族性疾病患者相比，有更晚期肾病的迹象。在回归模型中，疾病形式失去了任何独立影响。只有男性、较低的基线肾小球滤过率、较高的蛋白尿和组织病理学评分被证明是疾病进展的独立预测因素。使用类固醇或血管紧张素转换酶抑制剂治疗与改善预后相关。
我们的研究未证实家族性IgA肾病的预后比散发性形式更差。相似的肾脏表型可能支持家族性和散发性IgA肾病存在共同的致病机制。
