Henoch Schonlein purpura with nephritis in adults: adverse prognostic indicators in a UK population
Henoch Schonlein purpura with nephritis in adults: adverse prognostic indicators in a UK population
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DOI：
10.1093/qjmed/hcl034
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发表时间：
2006-04-01
影响因子：
13.3
通讯作者：
Ballardie, F
中科院分区：
文献类型：
--
作者：
Shrestha, S;Sumingan, N;Ballardie, F
背景资料：过敏性紫癜肾炎（HSN）在成人可能会导致严重的器官损伤，但其罕见性已导致缺乏data.Aim：评估成人HSN patients.Design：回顾性分析的临床结果和危险因素。方法：37例成人HSN参加区域血管炎诊所1974年至2004年进行了评估。对于列入，肾活检显示占主导地位的系膜伊加免疫沉积物是必需的，再加上至少两个：紫癜性皮疹，关节痛，abdominal pain.Results：10例（27%）进展为终末期肾功能衰竭（ESRF）。肾衰竭发生率在前十年最高，5年生存率为72%，10年生存率为68%，最终审查时为46%。ESRF的风险因素包括：随访期间蛋白尿>= 1 g/天（RR 83.8，p = 0.0006）;就诊时高血压（RR = 53.3，p = 0.0045）和随访期间（RR = 5.9，p = 0.05）;就诊时肾损害（RR 8.0，p = 0.0015）;年龄< 30岁（RR 7.6，p = 0.02）;男性（RR = 6.0，p = 0.05）。活检经常显示新月体，大多数影响< 50%的肾小球;它们的存在预示着ESRF，间质纤维化和肾小管萎缩也是如此。相反，肾缓解率也很高（43%）。32%的患者使用了细胞毒性药物，对结局无明显影响。影响经典肾外系统的复发很常见，但与肾功能下降无关。高比例的患者（41%）也遭受了血管炎性器官损伤以外的经典systems.Discussion：HSN在成人是一种严重的复发性疾病，经常在小血管ANCA阳性vasculitides引起肾功能衰竭。该系列的预后和风险与其他国家不同，包括ESRF的风险高于以前的系列。不同的群体要么发展ESRF，要么汇款。缺乏明确的益处表明，皮质类固醇应该保留给严重疾病的患者，而细胞毒性药物可能不适合肾衰竭高危患者。
Background: Henoch Schonlein purpura with nephritis (HSN) in adults may cause severe organ injury, but its rarity has contributed to a lack of data.Aim: To evaluate clinical outcomes and risk factors in adult HSN patients.Design: Retrospective analysis.Methods: Thirty-seven patients with adult HSN attending the Regional Vasculitis Clinic between 1974 and 2004 were assessed. For inclusion, a renal biopsy showing predominant mesangial IgA immune deposits was required, plus at least two of: purpuric rash, arthralgia, abdominal pain.Results: Ten patients (27%) progressed to end-stage renal failure (ESRF). Renal failure rates were highest in the first decade, with survival rate 72% at 5 years, 68% at 10 years and 46% at final review. Risk factors for ESRF were: proteinuria >= 1 g/day during follow-up (RR 83.8, p = 0.0006); hypertension at presentation (RR = 53.3, p = 0.0045) and during follow-up (RR = 5.9, p = 0.05); renal impairment at presentation (RR 8.0, p = 0.0015); age < 30 years (RR 7.6, p = 0.02); and male sex (RR = 6.0, p = 0.05). Biopsies frequently showed crescents, mostly affecting < 50% of glomeruli; their presence predicted ESRF, as did interstitial fibrosis and tubular atrophy. Renal remission, in contrast, was also high (43%). Cytotoxics were used in 32%, with no clear effect on outcome. Relapses affecting the classical extra-renal systems were common, but were not associated with declines in renal function. A high proportion of patients (41%) also suffered vasculitic organ injuries outside the classical systems.Discussion: HSN in adults is a serious relapsing disease, causing renal failure as frequently as in small-vessel ANCA-positive vasculitides. Prognosis and risks differed in this series from those in other countries, including a higher risk of ESRF than in previous series. Distinct groups developed either ESRF, or remitted. The absence of clear benefit suggests that corticosteroids should be reserved for patients with serious disease, and that cytotoxics may not be merited for those at high risk of renal failure.