Division of Rheumatology, Hospital Universitario Marqués de Valdecilla, IFIMAV, Santander, Spain.
Clin Exp Rheumatol. 2014 May-Jun;32(3 Suppl 82):S34-40. Epub 2014 Feb 11.
Hypersensitivity vasculitis (HV) and Henoch-Schönlein purpura (HSP) are the most common entities included within the category of cutaneous vasculitis (CV). Palpable purpura and histological changes characterised by the presence of leukocytoclastic vasculitis are common in both conditions. Therefore, considerable overlap between them is often seen. It is especially true when the CV occurs in adults. To further establish clinical differences between these two conditions, in the present study we assessed the main clinical differences between HV and HSP in a wide and unselected series of adults with CV from a defined population.
We reviewed the clinical records of 297 consecutive adults (age >20 years) seen at a single centre between January 1975 and December 2012 that were classified as having HSP or HV according to the criteria proposed by Michel et al. (J Rheumatol 1992; 19: 721-8).
Based on the inclusion criteria, 102 adult patients (71 men/31 women) were classified as HSP and 195 (104 men/91 women) as HV. The mean age was similar in both groups (55.8±16.5 years in HSP and 56.8±18.3 years in HV). Precipitating events, usually an upper respiratory tract infection and/or drug intake, were more frequently observed in HV. Both at the beginning of the disease and when the CV was established clinical manifestations were more frequent in patients with HSP than in those with HV. It was the case for gastrointestinal (57.4% vs. 6.8%; p<0.001), joint (51.5% vs. 36.6%; p=0.01) and renal involvement (86.3% vs. 18.3%; p<0.001). Corticosteroid (56.7% vs. 22%; p<0.001) and cytotoxic drug (19.4% vs. 3.2%; p<0.001) use was also more common in patients with HSP. After a median follow-up of 15.5 (interquartile range- IQR; 3-37) months in HSP and 4 (IQR; 2-12) months in HV, the outcome was better in HV than in HSP. In this regard, complete recovery (72.6% vs. 85.4%; p=0.01) was more commonly observed in HV while residual renal involvement (15.3% vs. 4.2%; p<0.001) was more common in HSP. The disease relapsed in 35.3% of patients with HSP and in 24.4% with HV (p=0.07).
Our results confirm the claim that these two diseases presenting with similar cutaneous involvement are certainly two separate entities with greater systemic involvement and less favourable outcome in HSP.
过敏性血管炎 (HV) 和亨诺克-舍恩莱因紫癜 (HSP) 是最常见的皮肤血管炎 (CV) 类别中的实体。可触及性紫癜和以白细胞碎裂性血管炎存在为特征的组织学变化在这两种情况下都很常见。因此，它们之间经常存在相当大的重叠。当 CV 发生在成年人中时尤其如此。为了进一步确定这两种情况之间的临床差异，在本研究中，我们评估了来自特定人群的广泛且未经选择的成年 CV 患者中 HV 和 HSP 之间的主要临床差异。
我们回顾了 1975 年 1 月至 2012 年 12 月期间在一个单一中心就诊的 297 例连续成年患者（年龄>20 岁）的临床记录，这些患者根据 Michel 等人提出的标准被归类为 HSP 或 HV（J Rheumatol 1992；19：721-8）。
根据纳入标准，102 例成年患者（71 例男性/31 例女性）被归类为 HSP，195 例（104 例男性/91 例女性）被归类为 HV。两组的平均年龄相似（HSP 为 55.8±16.5 岁，HV 为 56.8±18.3 岁）。HV 中通常以上呼吸道感染和/或药物摄入为诱因的诱发事件更为常见。在疾病开始时和 CV 确立时，HSP 患者的临床表现比 HV 患者更为频繁。胃肠道受累（57.4%对 6.8%；p<0.001）、关节受累（51.5%对 36.6%；p=0.01）和肾脏受累（86.3%对 18.3%；p<0.001）均如此。HSP 患者更常使用皮质类固醇（56.7%对 22%；p<0.001）和细胞毒性药物（19.4%对 3.2%；p<0.001）。在 HSP 中为 15.5 个月（中位数；四分位距 IQR；3-37），在 HV 中为 4 个月（IQR；2-12）的中位随访后，HV 的预后优于 HSP。在这方面，HV 中更常见完全缓解（72.6%对 85.4%；p=0.01），而 HSP 中更常见残余肾脏受累（15.3%对 4.2%；p<0.001）。HSP 中有 35.3%的患者疾病复发，HV 中有 24.4%的患者疾病复发（p=0.07）。
我们的结果证实了这两种疾病具有相似的皮肤受累，但实际上是两种不同的实体，HSP 具有更大的系统性受累和更不利的预后。
