Vasculitis in Children and Adolescents
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REVIEW ARTICLE
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Vasculitis in Children and Adolescents Clinical Presentation, Etiopathogenesis, and Treatment Kjell Tullus and Stephen D. Marks Department of Paediatric Nephrology, Great Ormond Street Hospital for Children NHS Trust, Great Ormond Street, London, UK
Contents Abstract . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 375 1. Kawasaki Disease . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 376 ¨ nlein Purpura . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 376 2. Henoch-Scho 3. Less Common Forms of Vasculitis. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 377 3.1 Anti-Neutrophil Cytoplasmic Autoantibody (ANCA)-Associated Vasculitis. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 377 3.2 Polyarteritis Nodosa. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 378 3.3 Takayasu Arteritis . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 378 3.4 Systemic Lupus Erythematosus. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 378 4. Conclusions . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 379
Abstract
The childhood vasculitides are a group of diseases affecting small to large blood vessels. The two most common conditions are Henoch-Scho¨nlein purpura (HSP) and Kawasaki disease (KD). HSP is diagnosed on the basis of typical clinical findings and is mostly a self-limiting disease. KD is the most common vasculitis in infants and the development of coronary artery aneurysms is the major complication. Early treatment with intravenous immunoglobulin and aspirin (acetylsalicylic acid) is required to minimize this risk. All other vasculitides are very rare in children and include anti-neutrophil cytoplasmic autoantibody (ANCA)-associated vasculitis, polyarteritis nodosa and Takayasu disease. The etiology of all these vasculitic diseases is generally unknown and, therefore, no specific treatment is available. The more rare vasculitides are generally much more severe, o
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