Henoch-Schönlein purpura in northern Spain: clinical spectrum of the disease in 417 patients from a single center.
Henoch-Schönlein purpura in northern Spain: clinical spectrum of the disease in 417 patients from a single center.
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DOI：
10.1097/md.0000000000000019
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发表时间：
2014-03
期刊：
影响因子：
1.6
通讯作者：
Blanco R
中科院分区：
文献类型：
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作者：
Calvo-Río V;Loricera J;Mata C;Martín L;Ortiz-Sanjuán F;Alvarez L;González-Vela MC;González-Lamuño D;Rueda-Gotor J;Fernández-Llaca H;González-López MA;Armesto S;Peiró E;Arias M;González-Gay MA;Blanco R
过敏性紫癜（HSP）患者的临床特征和预后的严重程度在以前的系列报道中差异很大，可能是由于选择偏倚。为了使用单个中心诊断的大量广泛的患者系列来建立所有年龄组的HSP的实际临床谱，我们对根据Michel等人提出的标准分类为患有HSP的417例患者进行了回顾性分析。在417例患者中，240例为男性，177例为女性，疾病诊断时的中位年龄为7.5岁（四分位距[IQR]，5.3-20.1岁）。四分之三的患者为20岁或以下的儿童或年轻人（n = 315），四分之一为成人（n = 102）。最常见的促发事件为既往感染（38%），通常为上呼吸道感染和/或血管炎发作前不久的药物摄入（18.5%）。发病时最常见的临床表现为皮肤损害（55.9%）、肾病（24%）、胃肠道受累（13.7%）、关节症状（9.1%）和发热（6.2%）。皮肤受累以紫癜性皮肤损害为主，其次为胃肠道（64.5%）、关节（63.1%）和肾脏（41.2%）。主要实验室检查结果为白细胞增多（36.7%）、贫血（8.9%）和血清伊加水平升高（31.7%）。最常用的治疗是皮质类固醇（35%）、非甾体类抗肿瘤药物（14%）和细胞毒性药物（5%）。中位随访12个月（IQR，2-38个月）后，在大多数病例中观察到完全恢复（n = 346; 83.2%），而仅在32例（7.7%）病例中观察到持续性（通常为轻度）肾病。在近三分之一的患者中观察到复发（n = 133; 31.9%）。总之，虽然HSP是影响儿童和青少年的典型血管炎，但在成人中并不少见。预后在大多数情况下是有利的，这在很大程度上取决于肾脏受累。
The severity of clinical features and the outcomes in previous series of patients reported with Henoch-Schönlein purpura (HSP) vary greatly, probably due to selection bias. To establish the actual clinical spectrum of HSP in all age groups using an unselected and wide series of patients diagnosed at a single center, we performed a retrospective review of 417 patients classified as having HSP according to the criteria proposed by Michel et al. Of 417 patients, 240 were male and 177 female, with a median age at the time of disease diagnosis of 7.5 years (interquartile range [IQR], 5.3–20.1 yr). Three-quarters of the patients were children or young people aged 20 years or younger (n = 315), and one-quarter were adults (n = 102). The most frequent precipitating events were a previous infection (38%), usually an upper respiratory tract infection, and/or drug intake (18.5%) shortly before the onset of the vasculitis. At disease onset the most common manifestations were skin lesions (55.9%), nephropathy (24%), gastrointestinal involvement (13.7%), joint symptoms (9.1%), and fever (6.2%). Cutaneous involvement occurring in all patients, mainly purpuric skin lesion, was the most common manifestation when the vasculitis was fully established, followed by gastrointestinal (64.5%), joint (63.1%), and renal involvement (41.2%). The main laboratory findings were leukocytosis (36.7%), anemia (8.9%), and increased serum IgA levels (31.7%). The most frequent therapies used were corticosteroids (35%), nonsteroidal antiinflammatory drugs (14%), and cytotoxic agents (5%). After a median follow-up of 12 months (IQR, 2–38 mo), complete recovery was observed in most cases (n = 346; 83.2%), while persistent, usually mild, nephropathy was observed in only 32 (7.7%) cases. Relapses were observed in almost a third of patients (n = 133; 31.9%). In conclusion, although HSP is a typical vasculitis affecting children and young people, it is not uncommon in adults. The prognosis is favorable in most cases, depending largely on renal involvement.