Abstract
Vasculitis is defined as inflammation of the blood vessels and can result in stenosis or aneurysm, which may in turn lead to occlusion or rupture of the vessel compromising tissue perfusion. The manifestations of these diseases depend on the size and site of the vessels effected. Vasculitis can be secondary to numerous inflammatory and infectious diseases but this review will concentrate on the systemic primary vasculitides and aims to discuss the presentations and approaches to management of a number of these conditions.
Similar content being viewed by others
References
Gardner-Medwin JM, Dolezalova P, Cummins C, Southwood TR. Incidence of Henoch-Schonlein purpura, Kawasaki disease, and rare vasculitides in children of different ethnic origins. Lancet. 2002;360:1197–202.
Burns JC, Glode MP. Kawasaki syndrome. Lancet. 2004;364:533–44.
Piram M, Mahr A. Epidemiology of immunoglobulin A vasculitis (Henoch-Schonlein): current state of knowledge. Curr Opin Rheumatol. 2013;25:171–8.
Hunder GG, Arend WP, Bloch DA, et al. The American college of rheumatology 1990 criteria for the classification of vasculitis. Introduction. Arthritis Rheum. 1990;33:1065–7.
Ozen S, Ruperto N, Dillon MJ, et al. EULAR/PReS endorsed consensus criteria for the classification of childhood vasculitides. Ann Rheum Dis. 2006;65:936–41.
Ozen S, Pistorio A, Iusan SM, et al. EULAR/PRINTO/PRES criteria for Henoch-Schonlein purpura, childhood polyarteritis nodosa, childhood Wegener granulomatosis and childhood Takayasu arteritis: Ankara 2008. Part II: Final classification criteria. Ann Rheum Dis. 2010;69:798–806.
Jennette JC, Falk RJ, Bacon PA, et al. 2012 revised international Chapel Hill consensus conference nomenclature of vasculitides. Arthritis Rheum. 2013;65:1–11.
Rao JK, Allen NB, Pincus T. Limitations of the 1990 American college of rheumatology classification criteria in the diagnosis of vasculitis. Ann Intern Med. 1998;129:345–52.
Wilkinson NM, Page J, Uribe AG, Espinosa V, Cabral DA. 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;34:224–6.
Gross WL, Trabandt A, Reinhold-Keller E. Diagnosis and evaluation of vasculitis. Rheumatology (Oxford). 2000;39:245–52.
D’Cruz D, Direskeneli H, Khamashta M, Hughes GR. Lymphocyte activation markers and von Willebrand factor antigen in Wegener’s granulomatosis: potential markers for disease activity. J Rheumatol. 1999;26:103–9.
Pohl M. Henoch-Schonlein purpura nephritis. Pediatr Nephrol. 2015;30:245–52.
Ronkainen J, Koskimies O, Ala-Houhala M, et al. Early prednisone therapy in Henoch-Schonlein purpura: a randomized, double-blind, placebo-controlled trial. J Pediatr. 2006;149:241–7.
Haroon M. Should children with Henoch-Schonlein purpura and abdominal pain be treated with steroids? Arch Dis Child. 2005;90:1196–8.
Weiss PF, Klink AJ, Localio R, et al. Corticosteroids may improve clinical outcomes during hospitalization for Henoch-Schonlein purpura. Pediatrics. 2010;126:674–81.
Dudley J, Smith G, Llewelyn-Edwards A, Bayliss K, Pike K, Tizard J. Randomised, double-blind, placebo-controlled trial to determine whether steroids reduce the incidence and severity of nephropathy in Henoch-Schonlein Purpura (HSP). Arch Dis Child. 2013;98:756–63.
Eleftheriou D, Batu ED, Ozen S, Brogan PA. Vasculitis in children. Nephrol Dial Transplant. 2015;30:i94–103.
Calvo-Rio V, Loricera J, Mata C, et al. Henoch-Schonlein purpura in northern Spain: clinical spectrum of the disease in 417 patients from a single center. Medicine (Baltimore). 2014;93:106–13.
Prais D, Amir J, Nussinovitch M. Recurrent Henoch-Schonlein purpura in children. J Clin Rheumatol. 2007;13:25–8.
Narchi H. Risk of long term renal impairment and duration of follow up recommended for Henoch-Schonlein purpura with normal or minimal urinary findings: a systematic review. Arch Dis Child. 2005;90:916–20.
Cabral DA, Uribe AG, Benseler S, et al. Classification, presentation, and initial treatment of Wegener’s granulomatosis in childhood. Arthritis Rheum. 2009;60:3413–24.
Eleftheriou D, Melo M, Marks SD, et al. Biologic therapy in primary systemic vasculitis of the young. Rheumatology (Oxford). 2009;48:978–86.
Bohm M, Gonzalez Fernandez MI, Ozen S, et al. Clinical features of childhood granulomatosis with polyangiitis (wegener’s granulomatosis). Pediatr Rheumatol Online J. 2014;12:18.
Sacri AS, Chambaraud T, Ranchin B, et al. Clinical characteristics and outcomes of childhood-onset ANCA-associated vasculitis: a French nationwide study. Nephrol Dial Transplant. 2015;30:i104–12.
Akikusa JD, Schneider R, Harvey EA, et al. Clinical features and outcome of pediatric Wegener’s granulomatosis. Arthritis Rheum. 2007;57:837–44.
Peco-Antic A, Bonaci-Nikolic B, Basta-Jovanovic G, et al. Childhood microscopic polyangiitis associated with MPO-ANCA. Pediatr Nephrol. 2006;21:46–53.
Brogan P, Eleftheriou D, Dillon M. Small vessel vasculitis. Pediatr Nephrol. 2010;25:1025–35.
Ozen S, Anton J, Arisoy N, et al. Juvenile polyarteritis: results of a multicenter survey of 110 children. J Pediatr. 2004;145:517–22.
Zwerina J, Eger G, Englbrecht M, Manger B, Schett G. Churg-Strauss syndrome in childhood: a systematic literature review and clinical comparison with adult patients. Semin Arthritis Rheum. 2009;39:108–15.
Vaglio A, Buzio C, Zwerina J. Eosinophilic granulomatosis with polyangiitis (Churg-Strauss): state of the art. Allergy. 2013;68:261–73.
Sasamoto M, Shigeta Y, Hisashi K, et al. Cutaneous polyarteritis nodosa associated with streptococcal infection in a child and a review of the literature. Nihon Rinsho Meneki Gakkai Kaishi. 1999;22:144–50.
Scott DG, Watts RA. Epidemiology and clinical features of systemic vasculitis. Clin Exp Nephrol. 2013;17:607–10.
Guillevin L, Lhote F, Cohen P, et al. Polyarteritis nodosa related to hepatitis B virus. A prospective study with long-term observation of 41 patients. Medicine (Baltimore). 1995;74:238–53.
Guillevin L, Lhote F, Amouroux J, Gherardi R, Callard P, Casassus P. Antineutrophil cytoplasmic antibodies, abnormal angiograms and pathological findings in polyarteritis nodosa and Churg-Strauss syndrome: indications for the classification of vasculitides of the polyarteritis Nodosa Group. Br J Rheumatol. 1996;35:958–64.
Cakar N, Yalcinkaya F, Duzova A, et al. Takayasu arteritis in children. J Rheumatol. 2008;35:913–9.
Brunner J, Feldman BM, Tyrrell PN, et al. Takayasu arteritis in children and adolescents. Rheumatology (Oxford). 2010;49:1806–14.
Watson L, Brogan P, Peart I, Landes C, Barnes N, Cleary G. Diagnosis and assessment of disease activity in takayasu arteritis: a childhood case illustrating the challenge. Case Rep Rheumatol. 2014;2014:603171.
Mavrogeni S, Dimitroulas T, Chatziioannou SN, Kitas G. The role of multimodality imaging in the evaluation of Takayasu arteritis. Semin Arthritis Rheum. 2013;42:401–12.
Morales E, Pineda C, Martinez-Lavin M. Takayasu’s arteritis in children. J Rheumatol. 1991;18:1081–4.
Acknowledgments
The authors would like to acknowledge help of Dr J Chaitow for providing the picture presented in Fig. 3.
Contributions
Both authors contributed to all aspects of the manuscript. DS-G will act as guarantor for this paper.
Conflict of Interest
None.
Source of Funding
None.
Author information
Authors and Affiliations
Corresponding author
Rights and permissions
About this article
Cite this article
Singh-Grewal, D., Durkan, A.M. Pediatric Vasculitis. Indian J Pediatr 83, 156–162 (2016). https://doi.org/10.1007/s12098-015-1876-2
Received:
Accepted:
Published:
Issue Date:
DOI: https://doi.org/10.1007/s12098-015-1876-2