Henoch-Schonlein purpura in children from northwestern Spain -: A 20-year epidemiologic and clinical study
Henoch-Schonlein purpura in children from northwestern Spain -: A 20-year epidemiologic and clinical study
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DOI：
10.1097/00005792-200109000-00001
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发表时间：
2001-09-01
期刊：
影响因子：
1.6
通讯作者：
González-Gay, MA
中科院分区：
文献类型：
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作者：
Calviño, MC;Llorca, J;González-Gay, MA
过敏性紫癜（HSP）是一种全身性血管炎，其特征为与任何潜在凝血功能障碍、胃肠道表现、关节炎和肾脏受累无关的紫癜性皮肤病变（25，51）。多形核白细胞浸润小血管和白细胞破裂是这种血管炎的典型病理表现。此外，组织的免疫荧光染色通常显示小血管壁和肾小球中存在免疫球蛋白A（伊加）主导的免疫沉积物（21，30）。过敏性紫癜主要是一种儿童疾病：它是儿童最常见的血管炎类型，在成人中不常见（26）。Heberden（27）首次描述了这种综合征，他报告了一名儿童的关节疼痛和疼痛性皮下水肿、腹痛、呕吐、血便和血尿以及腿部皮肤上的“血点”。然而，Schönlein和Henoch的名字最常用作综合征的名称。最初，Schönlein（52）使用术语“风湿性紫癜”描述了儿童关节痛和紫癜性皮肤病变之间的关系。几年后，他的学生Henoch（28岁）描述了一种紫癜、严重腹绞痛和黑便综合征。几年后，Henoch将肾炎称为该综合征的并发症（29）。Stone最近指出（56），在过去几年中，关于儿童HSP的临床和流行病学研究相对较少（50）。值得注意的是，HSP的长期发病率和死亡率主要归因于肾脏受累。在一些研究中，HSP在儿童中的报告占进入终末期肾衰竭患者的5%-15%（10，37）。这些研究中的大多数是在参考中心参加的一系列选定的肾功能不全患者人群中进行的。然而，两个研究系列（33，55）提示儿童HSP肾炎预后良好，这比大多数基于更多选择的患者组的已发表系列更乐观。为了进一步研究这种综合征，我们研究了在西班牙西北部一个特定人群的单一参考医院诊断的100名儿童中HSP的发病率、临床谱和预后，时间为20年。特别关注的是这些患者的结局，特别是与永久性肾损伤发展有关的风险因素。
Henoch-Schönlein purpura (HSP) is a systemic vasculitis characterized by purpuric skin lesions unrelated to any underlying coagulopathy, gastrointestinal manifestations, arthritis, and renal involvement (25, 51). Infiltration of small blood vessels with polymorphonuclear leukocytes and the presence of leukocytoclasia are typical pathologic findings in this vasculitis. In addition, immunofluorescence staining of tissues usually discloses the presence of immunoglobulin A (IgA)-dominant immune deposits in the wall of the small vessels and in the renal glomeruli (21, 30). Henoch-Schönlein purpura is mainly a childhood disease: it is the most common type of vasculitis in children and an infrequent condition in adults (26). The first description of this syndrome was given by Heberden (27), who reported a child with joint pain and painful subcutaneous edema, abdominal pain, vomiting, bloody stools and urine, and “bloody points” over the skin of his legs. However, the names of Schönlein and Henoch are most commonly used as the designation of the syndrome. Initially, Schönlein (52) described the association between arthralgia and purpuric cutaneous lesions in a child using the term “purpura rheumatica.” Some years later, his pupil Henoch (28) described a syndrome of purpura, severe abdominal colic, and melena. A few years later, Henoch referred to nephritis as a complication of this syndrome (29).As recently pointed out by Stone (56), there has been a relative paucity of clinical and epidemiologic studies on HSP in children over the past few years (50). Of note, the long-term morbidity and mortality of HSP are predominantly attributable to renal involvement. In some studies HSP in children has been reported to account for 5%–15% of patients entering end-stage renal failure (10, 37). Most of these studies were done on series of selected patient populations with kidney dysfunction attended in reference centers. Two unselected series (33, 55), however, suggested a good prognosis for HSP nephritis in children—a more optimistic outcome than most published series based on more selected groups of patients. To investigate this syndrome further, we examined the incidence, clinical spectrum, and prognosis of HSP in an unselected population of children diagnosed at the single reference hospital for a defined population in northwestern Spain over a 20-year period. Special interest was focused on the outcome of these patients and, in particular, on the risk factors implicated in the development of permanent renal damage.