Recently,new findings have been clarified concerning both pathogenesis and treatment of IgA nephritis.The four hits theory has been confirmed but several genetic wide association studies have allowed finding several genes connected with the pathogenesis of the disease.All these new genes apply to each of the four hits.Additionally,new discoveries concerning the microbiota and its connection with immune system and IgA generation have allowed finding out the role of the mucosa in IgA nephropathy pathogenesis.The IgA treatment is also changed included the future possibilities.The treatment of the chronic kidney disease,associated with the nephropathy,is mandatory,since the beginning of the disease.The classical immunosuppressive agents have poor effect.The corticosteroids remain an important cornerstone in any phase of the disease.More effect is related to the treatment of B cells and plasma cells.In particular,in very recent studies have been documented the efficacy of anti B cell-activating factor and anti A proliferation-inducing ligand agents.Most of these studies are to date in phase II/III.Finally,new agents targeting complement are arising.These agents also are still in randomized trials and act principally in hit 4 where the immunocomplexes in the mesangium activate the different pathways of the complement cascade.
IgA肾病(immunoglobulin A nephropathy,IgAN)属于常见的原发性肾小球肾炎之一,也是终末期肾病(end-stage renal disease,ESRD)的主要病因之一。IgAN的治疗仍然存在挑战,干预相关的危险因素,可延缓疾病的进展。近年发现,高尿酸血症是IgAN患者常见的临床特点,也是疾病进展的危险因素之一,但高尿酸血症与IgAN病理改变及疾病进展的关系仍不明确。因此,明确高尿酸血症与IgAN的关系具有重要的临床意义。本文从高尿酸血症在IgAN中的患病率、相关的发病机制与IgAN进展的关系及治疗靶点等方面进行了较为详尽的综述。
免疫球蛋白A血管炎(IgAV)是一种白细胞碎裂性免疫复合体介导的小血管炎,可导致多脏器受累。急性期可能出现严重的胃肠道出血、肠套叠或穿孔等并发症甚至危及生命。现就IgAV的病因、机制、危险因素、治疗的研究进展进行综述,旨在早期识别IgAV患儿,避免进展为重症病例,有利于临床有针对性的早期预防和治疗。Immunoglobulin A vasculitis (IgAV) is a small vasculitis mediated by leukocyte fragmentation immune complex, which can lead to multiple organ involvement. Severe gastrointestinal bleeding, intussusception, perforation and other complications may occur in the acute stage, even life-threatening. This article reviews the etiology, mechanism, risk factors and treatment of IgAV, aiming at early identification of children with IgAV and avoiding progression to severe cases, which is conducive to targeted early prevention and treatment.
