Monteiro Renato C
Inserm U1149 centre de recherche sur l'inflammation, 16, rue Henri-Huchard, 75018 Paris, France; CNRS ERL8252, 16, rue Henri-Huchard, 75018 Paris, France; Laboratoire d'excellence Inflamex, faculté de médecine Bichat, université Paris-Diderot, 16, rue Henri-Huchard, 75018 Paris, France; Service d'immunologie, DHU Fire, hôpital Bichat, 16, rue Henri-Huchard, 75018 Paris, France.
Nephrol Ther. 2018 Apr;14 Suppl 1:S1-S8. doi: 10.1016/j.nephro.2018.02.004.
Immunoglobulin A nephropathy or Berger's disease is the most common type of primary glomerulonephritis, which is characterized by IgA1-containing immune-deposits in the glomerular mesangium. Microscopic haematuria and proteinuria are the most common presentations. Mesangial cell proliferation with IgA deposition is found on renal biopsy. Mechanims of the disease implicate at least four key molecules have been implicated in immune complex formation: galactose-deficient IgA1, autoantibodies anti-galactose-deficient-IgA1, soluble CD89 (Fc receptor for IgA) and the CD71 mesangial IgA receptor (transferrin receptor). These factors associated with environmental factors (antigens, food and microbiota) are correlated with disease progression and recurrence after transplantation. This review exploits recent data on the role of these molecular players of the disease, which may improve future therapeutic management of immunoglobulin A nephropathy.
免疫球蛋白A肾病或伯杰氏病是原发性肾小球肾炎最常见的类型，其特征是在肾小球系膜中有含IgA1的免疫沉积物。镜下血尿和蛋白尿是最常见的表现。肾活检发现系膜细胞增殖伴IgA沉积。该疾病的发病机制涉及至少四种与免疫复合物形成有关的关键分子：缺乏半乳糖的IgA1、抗缺乏半乳糖IgA1的自身抗体、可溶性CD89（IgA的Fc受体）和CD71系膜IgA受体（转铁蛋白受体）。这些与环境因素（抗原、食物和微生物群）相关的因素与疾病进展及移植后的复发相关。本综述利用了关于该疾病这些分子参与者作用的最新数据，这可能会改善免疫球蛋白A肾病未来的治疗管理。
