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Purpose of Review: To examine recent advances in the pathophysiology and therapy of pediatric vasculitis., Recent Findings: The past 2 years have been marked by significant progress in extending novel techniques to the investigation of the two most common pediatric vasculitis syndromes, Henoch-Schonlein purpura and Kawasaki disease. Study of other vasculitides, such as Wegener granulomatosis, Churg-Strauss syndrome, and microscopic polyangiitis, is impeded by the small number of pediatric patients. Nonetheless, national and international registries are beginning to provide the foundation for generation of testable hypotheses regarding pathogenesis and optimal treatment. Thus, recent data from the study of children suggest that disorders in the control of inflammation, such as those that underlie familial Mediterranean fever and other autoinflammatory diseases, may predispose to vasculitis. Improved knowledge of mechanisms of disease, in turn, should pave the way for more targeted, effective, and tolerable therapies for children with systemic vasculitis., Summary: International collaboration to study rare disorders such as pediatric vasculitis are demonstrating disorders of inflammatory regulation that predispose to these diseases and may point toward new treatment approaches.

Purpose of Review: This review provides the readers with an update on the clinical approach of a patient seeking treatment with cutaneous vasculitis. It outlines the work-up for assessing patients with cutaneous vasculitis and discusses the essential features of the main conditions included within this category., Recent Findings: Recent works on genetic and infectious factors implicated in the pathogenesis of Henoch-Schonlein purpura are discussed. Special attention is given to the prognosis and response to treatment. Also, recent reports on cutaneous vasculitis secondary to connective tissue diseases are reviewed., Summary: With this review, the reader will be able to establish the steps to be followed in the clinical approach to a patient seeking treatment with cutaneous vasculitis.

Purpose of Review: The purpose of this review is to provide an update on the new developments in pediatric vasculitis., Recent Findings: Because most childhood vasculitides are rare, few large systematic studies have been done. Studies of Henoch-Schönlein purpura have focused on pathogenesis and outcome. Genetic associations and molecular changes occurring during Henoch-Schönlein purpura, including cytokines, and endothelial and nitric oxide metabolism are discussed. Risk factors for renal involvement and poor renal outcome are described. Uncontrolled series of treatment protocols for severe Henoch-Schönlein purpura nephritis are mentioned. Several studies have focused on the pathogenesis of other primary vasculitides, especially polyarteritis nodosa. Series describing the clinical manifestations of childhood vasculitis and case reports of uncommon manifestations of vasculitis in children are presented. The efficacy of new therapies, including the use of thalidomide and biologic modifiers, has been shown in individual childhood cases; however, there are no controlled studies of these agents., Summary: Besides studies of Henoch-Schönlein purpura, advances in pediatric vasculitis are few as a result of the rarity of most vasculitides in childhood. Multicenter collaboration is necessary to substantially increase the scientific base of investigating and treating childhood vasculitis., (Copyright 2004 Lippincott Williams & Wilkins)

Purpose of Review: The purpose of this review is to educate the reader about the one of the most common vasculitides of childhood: Henoch-Schonlein purpura. Although the disease has been described for over a century, the etiology has yet to be discovered. Adult-onset Henoch-Schonlein purpura is unusual, but through case studies, this review examines some of the common manifestations of this presentation of disease. Long-term outcome studies are still defining how this disease affects a small but significant percentage of patients afflicted with Henoch-Schonlein purpura., Recent Findings: This article will summarize recent work in molecular biology and genetics evaluating predisposing factors in the development of Henoch-Schonlein purpura. Further illustrations of the various complications of Henoch-Schonlein purpura will be reviewed. Recent long-term studies of outcomes of patients with renal disease from Henoch-Schonlein purpura will be summarized., Summary: After reading this review, the clinician will be able to elucidate the manifestations of Henoch-Schonlein purpura, determine appropriate treatment of the disease, and, most importantly, give the patient information about long-term consequences of Henoch-Schonlein purpura.

Henoch-Schönlein purpura (HSP) is the most common vasculitis syndrome of childhood. It is also known as anaphylactoid purpura, leukocytoclastic vasculitis, allergic vasculitis, and rarely, as rheumatoid purpura. It is generally a benign, self-limited disorder that follows an intercurrent illness, usually of the upper-respiratory tract. The classic triad of clinical symptoms and signs includes purpuric rash, abdominal cramping, and hematuria, but the spectrum of the clinical expression of HSP may vary from only minimal petechial rash to severe gastrointestinal, renal, neurologic, pulmonary, and joint disease. Most children have self-limited disease, and on long-term follow-up, systemic involvement or serious sequelae are not frequent. The current lack of knowledge about the factors underlying both the etiology and pathogenesis and the extent of clinical expression of HSP is illustrated in the recent literature. In addition, there is no agreement regarding the role of corticosteroids in the clinical management of HSP, and this subject has not received adequate attention during the past year. The recent clinical literature emphasizes the distinction between HSP and other hypersensitivity vasculitides and provides several in-depth reviews and multiple case reports illustrating the expanding clinical spectrum of the disorder. Exciting developments regarding immunologic aspects of HSP have been published and are summarized. The utility of antineutrophil cytoplasmic antibody as it applies to HSP remains to be elucidated.