儿童期系统性血管炎

血管炎在组织学上定义为血管壁存在纤维素样坏死和炎症，按照主要受累血管的大小可分为大血管炎如大动脉炎；中血管炎如结节性多动脉炎，川崎病；小血管炎如过敏性紫癜，抗中性粒细胞胞浆抗体相关性血管炎等。儿童系统性血管炎包括一组异质性疾病，病因不清，发病机制、组织学、临床表现及预后各不相同。川崎病，过敏性紫癜主要见于儿童期，近年来对其病因、发病机制、治疗和预后进行了大量研究；而小血管炎中韦格纳肉芽肿病、显微镜下多血管炎和变应性肉芽肿性血管炎(Churg-Strauss综合征)可发生于任何年龄，儿童少见，临床资料有限、不为多数儿科医生昕认识、而早期诊断，选择恰当的治疗方法可避免或延缓不可逆损害的发生，使病人的长期生存率得到改善。     

儿童期血管炎

全身性血管炎综合征在儿童期罕见，它是与血管炎性变有关的许多不同疾病的主要特征。最近人们开始注意到循环免疫复合物、细胞免疫、抗中性白细胞胞浆抗体（ＡＮＣＡ）和内皮细胞抗体在发病机制中的作用。儿童的血管炎均可使肾脏受累。早期用免疫抑制剂治疗可使病人的长期生存率得以改善。     

儿童期血管炎

全身性血管炎综合征在儿童期罕见，它是与血管炎性变有关的许多不同疾病的主要特征。最近人们开始注意到循环免疫复合物、细胞免疫、抗中性白细胞胞浆抗体和内皮细胞抗体在发病机制中的作用。儿童的血管炎均可使肾脏受累。早期用免疫抑制剂治疗可使病人的长期生存率得以改善。     

川崎病病因的研究进展

川崎病是一种多发于婴幼儿的急性热性发疹性疾病，主要危害是冠状动脉扩张和冠状动脉瘤的形成，已经成为儿童后天性心脏病的的首要病因。但是目前其病因和发病机制仍不清楚。许多研究表明川崎病可能是在一定的遗传易感性基础上，由一种或多种广泛存在的病原体感染并导致自身免疫系统异常激活而引起的临床综合征。一系列的细菌病毒和川崎病有相关性，并且以抗原或超抗原介导了机体的免疫激活。流行病学特征表明遗传因素对于川崎病的发病和预后也有重要影响。     

从过敏性紫癜到IgA血管炎——发病机制新认识

过敏性紫癜为儿童期最常见的系统性小血管炎,早期被认为是过敏性疾病,随着免疫病理技术的发展,将其定义为IgA血管炎,累及肾脏的称为IgA血管炎肾炎(IgAVN)或紫癜性肾炎.其发病机制存在两种假说,其一为类似于IgA肾病(IgAN)的四重打击学说,其二为围绕血管炎症和黏膜免疫为主的新多重打击学说,认为IgAVN是一种双重...     

肺炎支原体感染与血管炎

肺炎支原体感染是我国儿童呼吸病学领域近年来关注的热点之一,可引起血管炎等肺外表现,但其致病机制尚不清楚。近年在儿童川崎病、过敏性紫癜等以血管炎为主要特征的疾病中发现伴随肺炎支原体感染,其病情可在抗支原体感染治疗后缓解,提示肺炎支原体与血管炎的密切联系。现从肺炎支原体所致血管炎的可能作用机制及临床研究进行阐述,旨在更好地...     

皮肤黏膜淋巴结综合征8例分析

皮肤黏膜淋巴结综合征（称MCLS），是一种全身性血管炎，主要在婴儿和儿童发病。现将我院1984～2004年收治的8例报告如下。     

儿童IgA血管炎肾损伤监测与早期生物标记物

IgA血管炎(IgA vasculitis,IgAV)是儿童期最常见的小血管炎,约40%~50%患儿可累及肾脏,其中部分可进展至慢性肾衰竭,最终导致终末期肾病,严重影响患儿及其家庭生活质量。目前肾活检病理检查仍是判断IgAV肾损伤程度的金标准,然而因其创伤性大、不易动态监测等,迫切需要探究无创、更易获得的检测指标。近年来,随着蛋白组学及分子基因学进步,探究尿液及血清标本中儿童IgAV肾损害的生物标志物日益受到关注。故现将相关研究作一综述。     

冠状动脉血流储备测定在儿科的临床应用

冠状动脉血流储备是衡量心肌功能的影像学检查之一，近年来被陆续运用于评价川崎病、冠状动脉再植术后以及心肌肥厚等儿童期疾病的冠状动脉病变及功能。随着无创影像技术的进展，冠状动脉血流储备必将成为诊断儿童冠状动脉疾病的重要手段之一。     

系统性血管炎早期识别

系统性血管炎是一组以血管炎为共同病理改变、多器官受累的疾病。可累及全身多器官系统的各级血管,临床表现多样,且缺乏特异性。总结儿童常见系统性血管炎的临床表现、诊断标准,旨在提高儿科医师对疾病的认识,早期发现及合理诊治,以改善预后。     

Vasculitis in children

The term vasculitis refers to inflammation in the blood vessel walls. The vasculitic conditions which affect children are a varied group of diseases many of which carry a potentially significant morbidity and mortality. This review describes the classification, diagnosis and management of the main primary systemic paediatric vasculitides. The conditions described include Henoch-Schönlein purpura, Kawasaki disease, Takayasu's arteritis, polyarteritis nodosa, and the ANCA associated vasculitic (AAV) conditions Wegener's granulomatosis, microscopic polyangiitis and Churg-Strauss syndrome.     

Vasculitis in children

Vasculitis in children is uncommon and hardly any information is available from India. We, at PGIMER, Chandigarh, have diagnosed and followed many children with vasculitis of different types though not all, which occur in children. In this article, we have given an overview of the vasculitides that we have encountered alongwith a review of relevant literature. We have described 8 children with classical PAN and have highlighted a higher frequency of CNS involvement in our patients. Amongst the 10 BCPAN children, as many as 8 had peripheral gangrene which resulted in autoamputation in 7. Gangrene of such severity has not been previously reported in this condition. We have also included 30 children with HSP. Gastrointestinal involvement was noted in 86.7% of children and in one of these, it was severe encough to result in hypovolemic shock. Such severe bleeding is very rare. Two of our patients with HSP came late to us after having been operated for an acute abdomen elsewhere. Although renal involvement was seen less frequently than reported in the literature, the severity of involvement was greater (nephrotic range proteinuria in 62% and azotemia in 50%). We have only limited experience of Kawasaki Disease but it appears that children with this disorder are probably not being diagnosed in the acute stage in our country.     

Vasculitis in children

Systemic vasculitis is characterized by blood vessel inflammation which may lead to tissue injury from vascular stenosis, occlusion, aneurysm, and/or rupture. Apart from relatively common vasculitides such as Henoch–Schönlein Purpura (HSP) and Kawasaki disease (KD), most of the primary vasculitic syndromes are rare in childhood, but are associated with significant morbidity and mortality. Classification criteria for childhood vasculitis and a disease activity scoring tool have recently been proposed and validated. The cause of the majority of vasculitides is unknown, although it is likely that a complex interaction between environmental factors such as infections and inherited host responses trigger the disease and determine the vasculitis phenotype. Several genetic polymorphisms in vasculitis have now been described that may be relevant in terms of disease predisposition or development of disease complications. Treatment regimens continue to improve, with the use of different immunosuppressive medications and newer therapeutic approaches such as biologic agents. We provide an overview of paediatric vasculitides with emphasis on presenting features, current insights on aetiopathogenesis and treatment advances.     

Vasculitis

Inflammatory changes in blood vessels are a prominent feature of several diseases, which can be categorized by the size of the vessels, the nature of the inflammatory exudate, and the specific organs involved. The clinical and laboratory findings are variable and frequently nonspecific. Therapy ranges from essentially just observation to high-dose steroids combined with immunosuppressive agents, but treatment must be individualized. Early and correct diagnosis is thus important, but because there is considerable overlap between diseases, especially in the leukocytoclastic vasculitis group, one should never delay initiation of therapy while one is trying to finalize the diagnosis, particularly when the patient may be deteriorating rapidly.     

Vasculitis in children

Systemic vasculitis is characterized by blood vessel inflammation which may lead to tissue injury from vascular stenosis, occlusion, aneurysm, and/or rupture. Apart from relatively common vasculitides such as IgA Vasculitis (IgAV; previously referred to as Henoch-Sch?nlein Purpura [HSP]) and Kawasaki disease (KD), most of the primary vasculitic syndromes are rare in childhood, but are associated with significant morbidity and mortality. The cause of the majority of vasculitides is unknown, although it is likely that a complex interaction between environmental factors such as infections and inherited host responses trigger the disease and determine the vasculitis phenotype. Several genetic polymorphisms in vasculitis have now been described. Treatment regimens continue to improve, with the use of different immunosuppressive medications and newer therapeutic approaches such as biologic agents. Randomized control studies involving predominantly adults have recently recruited children with vasculitis too; but rare disease trial design is required for paediatric specific trials. The SHARE (Single-Hub Access for Pediatric Rheumatology in Europe) project has recently provided guidance on management of rare paediatric rheumatic diseases including the vasculitides. This article provides an overview of paediatric vasculitides with emphasis on presenting features, current insights on aetiopathogenesis and treatment advances.     

Vasculitis in children

This article provides a general overview of vasculitis, situations in which the diagnosis should be considered, diagnostic methods, and therapeutic considerations. Details and treatments unique to specific vasculitides are also reviewed.     

Vasculitis in children

Vasculitis is a group of rare diseases of unknown etiology, characterised by inflammation and necrosis of blood vessels, contributing to various clinical consequences. The variety of clinical symptoms and presence of symptoms from various syndromes often make the diagnosis difficult. Until now no causal treatment has been established. In order to achieve a remission of the disease, corticosteroids and immunosuppressive therapy are recommended.