Vasculitis in children: Evaluation
- David Cabral, MBBS, FRCPC
David Cabral, MBBS, FRCPC
- Clinical Professor of Pediatrics, Head, Division of Rheumatology, Department of Pediatrics
- University of British Columbia
- Kimberly Morishita, MD, MHSc, FRCPC
Kimberly Morishita, MD, MHSc, FRCPC
- Clinical Assistant Professor of Pediatrics
- Division of Rheumatology, Department of Pediatrics
- University of British Columbia
Vasculitis is defined as the presence of inflammation in a blood vessel that may occur as a primary process or secondary to an underlying disease. Clinical symptoms vary widely depending upon the type and location of the vessels involved and the extent of inflammation . Because of the multisystem nature of these disorders, many pediatric subspecialties, including rheumatology, dermatology, nephrology, pulmonology, and cardiology, may be involved in the diagnostic workup and ongoing care of a child with vasculitis.
Although most types of primary vasculitis in childhood are rare, with an estimated incidence of 20.4 per 100,000 children below 17 years of age, prompt recognition and treatment of these disorders are important as these can be severe and life-threatening conditions without appropriate management.
The approach to evaluating a child with suspected vasculitis is reviewed here. The classification and overview of the management of childhood vasculitis are discussed separately. (See "Vasculitis in children: Classification and incidence" and "Vasculitis in children: Management overview".)
The diagnosis of primary vasculitis is challenging because the clinical presentation of childhood vasculitis varies widely depending upon the type and location of the vessels involved, the extent of inflammation, and subsequent vessel wall damage with associated hemodynamic changes (table 1) .
Many patients ultimately diagnosed with a specific vasculitis will have presented initially with a combination of fever or other constitutional symptoms, skin lesions, and laboratory evidence of inflammation. These early findings are neither specific nor sensitive for vasculitis and may be found in other conditions including common infections. In patients with such nonspecific systemic symptoms, vasculitis should be strongly considered if symptoms do not resolve as would be expected in a "self-limited" infectious illness. In addition to systemic symptoms, the presence of multiorgan involvement should heighten one's suspicion of vasculitis.To continue reading this article, you must log in with your personal, hospital, or group practice subscription. For more information on subscription options, click below on the option that best describes you:
- Dedeoglu F, Sundel RP. Vasculitis in children. Rheum Dis Clin North Am 2007; 33:555.
- Ozen S. Problems in classifying vasculitis in children. Pediatr Nephrol 2005; 20:1214.
- Athreya BH. Vasculitis in children. Pediatr Clin North Am 1995; 42:1239.
- Roane DW, Griger DR. An approach to diagnosis and initial management of systemic vasculitis. Am Fam Physician 1999; 60:1421.
- Jennette JC, Falk RJ, Andrassy K, et al. Nomenclature of systemic vasculitides. Proposal of an international consensus conference. Arthritis Rheum 1994; 37:187.
- Jennette JC, Falk RJ. Small-vessel vasculitis. N Engl J Med 1997; 337:1512.
- Bowyer S, Roettcher P. Pediatric rheumatology clinic populations in the United States: results of a 3 year survey. Pediatric Rheumatology Database Research Group. J Rheumatol 1996; 23:1968.
- Malleson PN, Fung MY, Rosenberg AM. The incidence of pediatric rheumatic diseases: results from the Canadian Pediatric Rheumatology Association Disease Registry. J Rheumatol 1996; 23:1981.
- Wilkinson NM, Page J, Uribe AG, et al. Establishment of a pilot pediatric registry for chronic vasculitis is both essential and feasible: a Childhood Arthritis and Rheumatology Alliance (CARRA) survey. J Rheumatol 2007; 34:224.
- Bowyer SL, Ragsdale CG, Sullivan DB. Factor VIII related antigen and childhood rheumatic diseases. J Rheumatol 1989; 16:1093.
- Akikusa JD, Schneider R, Harvey EA, et al. Clinical features and outcome of pediatric Wegener's granulomatosis. Arthritis Rheum 2007; 57:837.
- Belostotsky VM, Shah V, Dillon MJ. Clinical features in 17 paediatric patients with Wegener granulomatosis. Pediatr Nephrol 2002; 17:754.
- Gross WL, Trabandt A, Reinhold-Keller E. Diagnosis and evaluation of vasculitis. Rheumatology (Oxford) 2000; 39:245.
- Hagen EC, Daha MR, Hermans J, et al. Diagnostic value of standardized assays for anti-neutrophil cytoplasmic antibodies in idiopathic systemic vasculitis. EC/BCR Project for ANCA Assay Standardization. Kidney Int 1998; 53:743.
- Hoffman GS, Specks U. Antineutrophil cytoplasmic antibodies. Arthritis Rheum 1998; 41:1521.
- Xin G, Zhao MH, Wang HY. Detection rate and antigenic specificities of antineutrophil cytoplasmic antibodies in chinese patients with clinically suspected vasculitis. Clin Diagn Lab Immunol 2004; 11:559.
- Katayama H, Shimizu T, Tanaka Y, et al. Three-dimensional magnetic resonance angiography of vascular lesions in children. Heart Vessels 2000; 15:1.
- Noone DG, Twilt M, Hayes WN, et al. The new histopathologic classification of ANCA-associated GN and its association with renal outcomes in childhood. Clin J Am Soc Nephrol 2014; 9:1684.
- CLINICAL PRESENTATION
- DIFFERENTIATING PRIMARY VASCULITIS FROM OTHER DISORDERS
- Clinical correlation with vessel size
- General considerations
- Initial evaluation
- - History
- - Physical examination
- - Laboratory evaluation
- Further evaluation
- - Additional laboratory testing
- - Organ-specific imaging and functional studies
- - Tissue biopsy
- SOCIETY GUIDELINE LINKS