儿童IgA血管炎肾损伤临床病理特点与预后

Clinicopathological features and prognosis of renal involvement in children with IgA vasculitis

IgA血管炎是儿童最常见血管炎,长期预后决定于肾损伤程度,IgA血管炎肾损伤多见于10岁以上男性,血管炎反复(皮疹、腹痛、关节症状)的患儿,97%肾损伤在皮疹后6个月内出现。临床表现多以血尿和蛋白尿为主。肾脏病理是IgA沉积为主的系膜增生为主要病变的肾小球病,临床表现为肾病综合征或早期存在急性肾损伤患儿进展至终末期肾衰竭(ESRD)风险更高,即使表现为轻微血尿和蛋白尿也可能进展至CKD。病理Ⅳ和Ⅴ级更易进展至ESRD,早期逆转肾病状态对长期预后有益。

IgA vasculitis( IgAV) is the most common vasculitis in children,in which long-term prognosis is mostly dependent upon the severity of renal involvement.The risk factors of developing to IgAV nephritis( IgAVN) are age above 10 years at onset,male,abdominal pain and recurrence of vasculitis. 97% of IgAVN developed renal symptoms within 6 months after purpura presentation. Haematuria,mostly microscopic,company with or without microproteinuria is the most common resentation of IgAVN in children.The pathology of IgAVN is characterized by intrarenal,mesangial or vascular deposits of IgA and mesangial cells and mesangial matrix proliferation,which ia similar with IgA nephropathy.The important risk factors of poor kidney outcome were IgAVN presented with nephrotic syndrome or kidney insufficiency at onset. However some IgAVN patients who present with mild initial symptoms also eventually developed CKD.The patients with the ISKDC class Ⅳ and Ⅴare more likely to progress to ESRD.Control of nephrotic range proteinuria is beneficial to long-term prognosis.Improved treatment strategies and detection of IgAVN patients at risk of CKD at an early stage are necessary.