Abstract
The term “systemic vasculitis” encompasses a diverse set of diseases linked by the presence of blood-vessel inflammation that are often associated with critical complications. These diseases are uncommon in childhood and are frequently subjected to a delayed diagnosis. Although the diagnosis and treatment may be similar for adult and childhood systemic vasculitides, the prevalence and classification vary according to the age group under investigation. For example, Kawasaki disease affects children while it is rarely encountered in adults. In 2006, the European League Against Rheumatism (EULAR) and the Pediatric Rheumatology European Society (PReS) proposed a classification system for childhood vasculitis adopting the system devised in the Chapel Hill Consensus Conference in 1993, which categorizes vasculitides according to the predominant size of the involved blood vessels into small, medium and large vessel diseases. Currently, medical imaging has a pivotal role in the diagnosis of vasculitis given recent developments in the imaging of blood vessels. For example, early diagnosis of coronary artery aneurysms, a serious complication of Kawasaki disease, is now possible by magnetic resonance imaging (MRI) of the heart and multidetector computed tomography (MDCT); positron emission tomography/CT (PET/CT) helps to assess active vascular inflammation in Takayasu arteritis. Our review offers a unique approach using the integration of the proposed classification criteria for common systemic childhood vasculitides with their most frequent imaging findings, along with differential diagnoses and an algorithm for diagnosis based on common findings. It should help radiologists and clinicians reach an early diagnosis, therefore facilitating the ultimate goal of proper management of affected children.
Similar content being viewed by others
References
Wilfong EM, Seo P (2013) Vasculitis in the intensive care unit. Best Pract Res Clin Rheumatol 27:95–106
Luca N (2013) Vasculitis and thrombophlebitis, article on Medscape. http://emedicine.medscape.com/article/1008239. Updated 11 Feb 2013. Accessed 20 Nov 2013
Waller R, Ahmed A, Patel I et al (2013) Update on the classification of vasculitis. Best Pract Res Clin Rheumatol 27:3–17
Brunner J, Feldman B, Tyrrell P et al (2010) Takayasu arteritis in children and adolescents. Rheumatology 49:1806–1814
Jennette JC, Falk RJ, Andrassy K et al (1994) Nomenclature of systemic vasculitides: proposal of an international consensus conference. Arthritis Rheum 37:187–192
Jennette JC, Falk RJ, Bacon PA et al (2013) 2012 revised International Chapel Hill consensus conference nomenclature of vasculitides. Arthritis Rheum 65:1–11
Spira D, Kötter I, Ernemann U et al (2010) Imaging of primary and secondary inflammatory diseases involving large and medium-sized vessels and their potential mimics: a multitechnique approach. Vascular and Interventional Radiology. AJR Am J Roentgenol 194:848–856
Yim D, Curtis N, Cheung M et al (2013) An update on Kawasaki disease: epidemiology, aetiology and pathogenesis. J Paediatr Child Health 49:704–708
Ozen S, Ruperto N, Dillon M et al (2006) EULAR/PReS endorsed consensus criteria for the classification of childhood vasculitis. Ann Rheum Dis 65:936–941
Ozen S, Pistorio A, Iusan S et al (2010) EULAR/PRINTO/PRES criteria for Henoch-Schonlein purpura, childhood polyarteritis nodosa, childhood wegner granulomatosis and childhood Takayasu arteritis: Ankara 2008. Part II: final classification criteria. Ann Rheum Dis 69:798–806
Watts R, Lane S, Scott DG (2005) What is known about the epidemiology of vasculitides? Best Pract Res Clin Rheumatol 19:191–207
Cakar N, Yalcinkaya F, Duzova A et al (2008) Takayasu arteritis in children. J Rheumatol 35:913–919
Kumar S, Radhakrishnan S, Phadke RV et al (1997) Takayasu’s arteritis: evaluation with three-dimensional time-of-flight MR angiography. Eur Radiol 7:44–50
Yamada I, Nakagawa T, Himeno Y et al (2000) Takayasu arteritis: diagnosis with breath-hold contrast-enhanced three-dimensional MR angiography. J Magn Reson Imaging 11:481–487
Nastri MV, Baptista LP, Baroni RH et al (2004) Gadolinium-enhanced three-dimensional MR angiography of Takayasu arteritis. Radiographics 24:773–786
Alibaz-Oner F, Aydin SZ, Direskeneli H (2013) Advances in the diagnosis, assessment and outcome of Takayasu arteritis. Clin Rheumatol 32:541–546
Hausleiter J, Meyer T, Hermann F et al (2009) Estimated radiation dose associated with Cardiac CT angiography. JAMA 301:500–507
Apfaltrer P, Hanna EL, Schoepf UJ et al (2012) Radiation dose and image quality at high-pitch CT angiography of the aorta: intraindividual and interindividual comparisons with conventional CT angiography. AJR Am J Roentgenol 199:1402–1409
Karapolat I, Kalfa M, Keser G et al (2013) Comparison of F18-FDG PET/CT findings with current clinical disease status in patients with Takayasu’s arteritis. Clin Exp Rheumatol 31:S15–S21
Cheng Y, Lv N, Wang Z et al (2013) 18-FDG-PET in assessing disease activity in Takayasu arteritis: a meta-analysis. Clin Exp Rheumatol 31:S22–S27
Newburger J, Takahashi M, Gerber MA et al (2004) Diagnosis, treatment and long-term management of Kawasaki disease: a statement for health professionals from the Committee on Rheumatic Fever, Endocarditis and Kawasaki Disease, Council on Cardiovascular Disease in the Young, American Heart Association. Circulation 110:2747–2771
Dominguez S, Anderson M, El-Adawy M et al (2012) Preventing coronary artery abnormalities: a need for earlier diagnosis and treatment of Kawasaki disease. Pediatr Infect Dis J 31:1217–1220
Mavrogeni S, Papadopoulos G, Karanasios E et al (2008) How to image Kawasaki disease: a validation of different imaging techniques. Int J Cardiol 124:27–31
Mavrogeni S, Bratis K, Karanasios E et al (2011) CMR evaluation of cardiac involvement during the convalescence of Kawasaki disease. J Am Coll Cardiol Img 4(10):1140–1141
Yu Y, Sun K, Wang R et al (2011) Comparison study of echocardiography and dual-source CT in diagnosis of coronary artery aneurysm due to Kawasaki disease: coronary artery disease. Echocardiography 28:1025–1034
Duan Y, Wang X, Cheng Z et al (2012) Application of prospective ECG-triggered dual-source CT coronary angiography for infants and children with coronary artery aneurysms due to Kawasaki disease. Br J Radiol 85:1190–1197
Eleftheriou D, Dillon M, Brogan P (2009) Advances in childhood vasculitis. Curr Opin Rheumatol 21:411–418
Schmidt WA (2004) Use of imaging studies in the diagnosis of vasculitis. Curr Rheumatol Rep 6:203–211
Mujagic S, Sarihodzic S, Huseinagic H et al (2011) Wegner’s granulomatosis of the paransal sinuses with orbital and central nervous system involvement-diagnostic imaging. Acta Neurol Belg 111:241–244
Levine D, Akikusa J, Manson D et al (2007) Chest CT findings in pediatric Wegener’s granulomatosis. Pediatr Radiol 37:57–62
Ananthakrishnan L, Sharma N, Kanne J (2009) Wegner’s granulomatosis in the chest: high-resolution CT findings. AJR Am J Roentgenol 192:676–682
Connolly B, Manson D, Eberhard A et al (1996) CT appearance of pulmonary vasculitis in children. AJR Am J Roentgenol 167:901–904
Polychronopoulos V, Prakash U, Golbin J et al (2007) Airway involvement in Wegener’s granulomatosis. Rheum Dis Clin N Am 33:755–775
Summers R, Aggarwal N, Sneller M et al (2002) CT virtual bronchoscopy of the central airways in patients with Wegener’s granulomatosis. Chest 121:242–250
McCabe C, Jones Q, Nikolopoulou A et al (2011) Pulmonary-renal syndrome: an update for respiratory physician. Respir Med 105:1413–1421
Masi A, Hunder G, Lie J et al (1990) The American College of Rheumatology 1990 criteria for the classification of Churg-Strauss syndrome (allergic granulomatosis and angiitis). Arthritis Rheum 33:1094–1100
Castaner E, Alguersuari A, Gallardo X et al (2010) When to suspect pulmonary vasculitis: radiologic and clinical clues. Radiographics 30:33–53
Saulsbury FT (2001) Henoch-Schonlein purpura. Curr Opin Rheumatol 13:35–40
Rizzi R, Bruno S, Dammacco R (1997) Behcet disease. Int J Clin Lab Res 27:225–232
Kokturk A (2012) Clinical and pathological manifestations with differential diagnosis in Behçet’s disease. Pathol Res Int 2012, 69039010. doi:10.1155/2012/690390
Cellucci T, Benseler SM (2010) Diagnosing central nervous system vasculitis in children. Curr Opin Pediatr 22:731–738
Kanekar S, Devgun PA (2014) Pattern approach to focal high white matter signal on magnetic resonance imaging. Radiol Clin N Am 52:241–261
Lee Y, Kim J, Kim E et al (2009) Tumor-mimicking primary angiitis of the central nervous system: initial and follow-up MR features. Neuroradiology 51:651–659
Gowdie P, Twilt M, Benseler S (2012) Primary and secondary central nervous system vasculitis. J Child Neurol 27:1448–1459
Benseler SM, deVeber G, Hawkins C et al (2005) Angiography-negative primary central nervous system vasculitis in children: a newly recognized inflammatory central nervous system disease. Arthritis Rheum 52:2159–2167
Cosottini M, Canovetti S, Pesaresi I et al (2013) 3-T magnetic resonance angiography in primary angiitis of the central nervous system. J Comput Assist Tomogr 37:493–498
White ML, Zhang Y (2010) Primary angiitis of the central nervous system: apparent diffusion coefficient lesion analysis. Clin Imaging 34:1–6
Fugate J, Claassen D, Cloft H et al (2010) Posterior reversible encephalopathy syndrome: associated clinical and radiological findings. Mayo Clin Proc 85:427–432
Chang HK, Choi YJ, Baek SK et al (2001) Osteonecrosis and bone infarction in association with Behcet’s disease: report of two cases. Clin Exp Rheumatol 19:S51–S54
Hirahara K, Kano Y, Asano Y et al (2013) Osteonecrosis of the femoral head in a patient with Henoch-Schönlein purpura and drug-induced hypersensitivity syndrome treated with corticosteroids. Acta Derm Venereol 93:85–86
Marten K, Schnyder P, Schirg E et al (2005) Pattern-based differential diagnosis in pulmonary vasculitis using volumetric CT. AJR Am J Roentgenol 184:720–733
Engeler C, Tashjian J, Trenkner S et al (1993) Ground glass opacity of lung parenchyma: a guide to analysis with high-resolution CT. AJR Am J Roentgenol 160:249–251
Miller WT Jr, Shah RM (2005) Isolated diffuse ground-glass opacity in thoracic CT: causes and clinical presentations. AJR Am J Roentgenol 184:613–622
Acknowledgments
This work was presented as a scientific poster at the 2014 Society of Pediatric Radiology meeting in Washington, D.C., U.S.A.
Conflicts of interest
None
Author information
Authors and Affiliations
Corresponding author
Additional information
CME activity
This article has been selected as the CME activity for the current month. Please visit the SPR Web site at www.pedrad.org on the Education page and follow the instructions to complete this CME activity.
Rights and permissions
About this article
Cite this article
Soliman, M., Laxer, R., Manson, D. et al. Imaging of systemic vasculitis in childhood. Pediatr Radiol 45, 1110–1125 (2015). https://doi.org/10.1007/s00247-015-3339-3
Received:
Revised:
Accepted:
Published:
Issue Date:
DOI: https://doi.org/10.1007/s00247-015-3339-3