Division of Nephrology, Istituto Clinico Humanitas, Rozzano, Milano, Italy.
Nephrol Dial Transplant. 2012 Jul;27(7):2610-3. doi: 10.1093/ndt/gfs093.
IgA nephritis (IgAN) is an autoimmune disease characterized by deposits of IgA in the glomerular mesangium. Clinically, the disease may be punctuated by episodes of macroscopic haematuria often associated with pharingotonsillitis or may be oligosyntomatic with microscopic haematuria and mild proteinuria. The natural course of IgAN may be indolent and benign; however, some 30-50% of patients may progress to end-stage renal disease when follow-up is extended to ≥20 years. In patients with IgAN, circulating IgA1 molecules have an aberrant structure of O-glycans in the hinge region, which is characterized by abbreviated glycans composed of N-acetylgalactosamine, with or without sialic acid. These aberrant IgA1 trigger the production of autoantibodies, with formation of immune complexes that deposit in the mesangium causing inflammation and production of extracellular matrix. A number of experimental and clinical data outlined a possible pathogenetic role of tonsillitis. As a consequence, tonsillectomy has been frequently performed in Japan. Observational studies, made in patients with normal renal function and mild proteinuria, reported that tonsillectomy could reduce the episodes of macrohaematuria as well as the entity of microhaematuria and proteinuria. However, the available studies had short-term follow-up and could not asses the role of tonsillectomy in protecting from renal function deterioration. In a longitudinal retrospective study, Isseki et al. compared the outcome of tonsillectomized patients with IgAN with that of IgAN patients who did not receive tonsillectomy. Tonsillectomized patients had a higher number of remissions and a better slope of glomerular filtration rate in comparison with controls. These data are interesting and suggest that tonsillectomy may prevent renal dysfunction in patients with IgAN and normal renal function. However, the retrospective nature of the study and the presence of some confounding factors require further investigations to confirm these promising data.
IgA 肾病 (IgAN) 是一种自身免疫性疾病,其特征是 IgA 在肾小球系膜沉积。临床上,该疾病可能因常伴有咽炎或扁桃体炎的反复发作性肉眼血尿而间歇性出现,也可能因出现镜下血尿和轻度蛋白尿而症状较轻。IgAN 的自然病程可能是惰性和良性的;然而,在随访时间延长至≥20 年时,约 30-50%的患者可能进展至终末期肾病。在 IgAN 患者中,循环 IgA1 分子在铰链区具有 O-聚糖结构的异常,其特征是短聚糖组成的 N-乙酰半乳糖胺,有或没有唾液酸。这些异常的 IgA1 触发自身抗体的产生,形成免疫复合物沉积在系膜中,引起炎症和细胞外基质的产生。大量的实验和临床数据概述了扁桃体炎的可能发病机制。因此,在日本经常进行扁桃体切除术。在肾功能正常和轻度蛋白尿的患者中进行的观察性研究报告称,扁桃体切除术可减少反复发作性肉眼血尿以及镜下血尿和蛋白尿的程度。然而,现有研究的随访时间较短,无法评估扁桃体切除术在保护肾功能恶化方面的作用。在一项纵向回顾性研究中,Isseki 等人比较了接受扁桃体切除术的 IgAN 患者与未接受扁桃体切除术的 IgAN 患者的结局。与对照组相比,扁桃体切除术后患者的缓解率更高,肾小球滤过率的斜率也更好。这些数据很有趣,表明扁桃体切除术可能预防肾功能正常的 IgAN 患者的肾功能障碍。然而,该研究的回顾性性质和存在一些混杂因素需要进一步的研究来证实这些有前途的数据。