Kessler M, Frimat L, Hestin D, Renoult E, Grignon Y, Grignon G
Service de néphrologie, CHU de Nancy, hôpitaux de Brabois, Vandoeuvre, France.
Rev Med Interne. 1994;15(7):471-8. doi: 10.1016/s0248-8663(05)81471-7.
Idiopathic IgA nephropathy of Berger's disease is characterized by prominent and diffuse IgA deposits in the mesangium. In many countries, it is the most common type of primary chronic glomerulonephritis. Typically, it is revealed by recurrent episodes of gross hematuria in association with ENT infection, but it can progress insidiously with microscopic hematuria and proteinuria. Serum IgA levels are increased in about 50% of cases. IgA nephropathy is not a minor condition: 20% of patients develop end-stage chronic renal failure 10 years after diagnosis and 50% after 20 years. IgA nephropathy can recur in a transplanted kidney suggesting that this disease is a systemic disorder although it has a remarkable tropism for the kidney. Even though many points remain to be elucidated, its pathogenesis appears to be linked to a genetic factor responsible for a lymphocyte dysfunction and an acquired environmental factor such as penetration of an antigen via the mucosa which may give rise to an excessive and inappropriate IgA immune response with the deposition of IgA in the mesangium and the development of progressive renal alterations. No treatment has been shown to be effective but tonsillectomy advised in case of a recurrent tonsillar focal infection is most often accompanied by a decrease in the incidence of gross hematuria. Corticosteroid therapy can be of benefit in cases involving a nephrotic syndrome associated with minimal glomerular lesions. In all cases, control of possible hypertension is of value in slowing the progression of this disease.
伯杰氏病(又称特发性IgA肾病)的特征是系膜区有显著且弥漫性的IgA沉积。在许多国家,它是原发性慢性肾小球肾炎最常见的类型。通常,它表现为与耳鼻喉感染相关的反复肉眼血尿发作,但也可能隐匿进展,出现镜下血尿和蛋白尿。约50%的病例血清IgA水平升高。IgA肾病并非轻症:20%的患者在诊断后10年发展为终末期慢性肾衰竭,20年后这一比例为50%。IgA肾病可在移植肾中复发,这表明该疾病是一种全身性疾病,尽管它对肾脏有明显的趋向性。尽管仍有许多问题有待阐明,但其发病机制似乎与一个导致淋巴细胞功能障碍的遗传因素以及一个后天环境因素有关,比如抗原通过黏膜侵入,这可能引发过度且不适当的IgA免疫反应,导致IgA在系膜区沉积以及进行性肾脏病变的发展。尚无治疗方法被证明有效,但对于反复扁桃体局部感染的病例,建议行扁桃体切除术,这通常会伴随肉眼血尿发生率的降低。皮质类固醇疗法对涉及微小肾小球病变的肾病综合征病例可能有益。在所有病例中,控制可能存在的高血压对于减缓该疾病的进展具有重要意义。