Pediatric Vasculitis: Classification and Clinical Approach
To learn about the spectrum of vasculitis in children
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Ezgi Deniz Batu and Seza Özen
Learning Objectives
1. To learn about the spectrum of vasculitis in children 2. To understand the classification of vasculitis 3. To recognize the clinical features that should alert a physician to a suspect of vasculitis
Case Vignette
A 10-year-old boy presented to a local hospital with fever, abdominal pain, and purple rash on his legs and buttocks. There was a history of upper respiratory tract infection about 2 weeks ago. He described nausea, but there was no weight loss, vomiting, dysuria, nor bloody stools. Routine blood investigations were within normal limits except for leukocytosis and elevation of acute-phase reactants (erythrocyte sedimentation rate (ESR) and C-reactive protein (CRP)). He received oral antibiotics and intravenous hydration. Five days later, he presented to our outpatient clinic with complaints of intense abdominal pain and hematuria. On physical examina-
E.D. Batu, MD • S. Özen, MD (*) Department of Pediatric Rheumatology, Hacettepe University Faculty of Medicine, Sihhiye, Ankara 06410, Turkey e-mail: [email protected]; sezaozen@ hacettepe.edu.tr
tion, he had palpable purpura on his legs (Fig. 33.1) and tenderness in his abdomen. Laboratory investigations demonstrated leukocytosis with elevation of ESR and CRP. Platelet counts and liver and renal function tests were normal except for hypoalbuminemia. A stool benzidine test for occult blood was positive. He also had hematuria and nephrotic range proteinuria. An abdominal ultrasound revealed minimal diffuse increase in renal parenchymal echogenicity and an increase in wall thickness of intestinal segments. Renal
Fig. 33.1 Palpable purpura over the left leg of a 10-year-old boy with IgAV/HSP
© Springer Science+Business Media Singapore 2017 S. Sawhney, A. Aggarwal (eds.), Pediatric Rheumatology, DOI 10.1007/978-981-10-1750-6_33
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E.D. Batu and S. Özen
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biopsy showed immunoglobulin A (IgA) nephropathy with mesangial proliferation, endocapillary hypercellularity, segmental sclerosis, and cellular crescents in 57 % of glomeruli. He was treated with steroids and an immunosuppressive agent.
This patient is a typical example of a child with severe IgA vasculitis (IgAV; formerly HenochSchönlein purpura, HSP) involving the skin, gastrointestinal tract, and kidneys. He also has the common features of vasculitis: constitutional symptoms, skin features, organ involvement of the affected vessels (gastrointestinal small vessels in this case), and high acute-phase reactants.
Introduction The systemic vasculitides are heterogeneous, uncommon systemic diseases characterized by blood vessel inflammation that may cause tissue ischemia and injury as a result of vascular stenosis, occlusion, aneurysm, or rupture [1, 2]. Childhood vasculitis is a challenging group of multisystem conditions that often require integrated care from different subspecialties such as rheumatology, nephrology, cardiology, neurology, gastroenterology, and dermatology. This chapter explores the epidemiology and classification of p
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