Skip to main content

Advertisement

Log in

Imaging of systemic vasculitis in childhood

  • Review
  • Published:
Pediatric Radiology Aims and scope Submit manuscript

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.

This is a preview of subscription content, log in via an institution to check access.

Access this article

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Fig. 1
Fig. 2
Fig. 3
Fig. 4
Fig. 5
Fig. 6
Fig. 7
Fig. 8
Fig. 9
Fig. 10
Fig. 11
Fig. 12
Fig. 13
Fig. 14
Fig. 15
Fig. 16
Fig. 17
Fig. 18
Fig. 19
Fig. 20
Fig. 21
Fig. 22

Similar content being viewed by others

References

  1. Wilfong EM, Seo P (2013) Vasculitis in the intensive care unit. Best Pract Res Clin Rheumatol 27:95–106

    Article  PubMed  Google Scholar 

  2. Luca N (2013) Vasculitis and thrombophlebitis, article on Medscape. http://emedicine.medscape.com/article/1008239. Updated 11 Feb 2013. Accessed 20 Nov 2013

  3. Waller R, Ahmed A, Patel I et al (2013) Update on the classification of vasculitis. Best Pract Res Clin Rheumatol 27:3–17

    Article  PubMed  Google Scholar 

  4. Brunner J, Feldman B, Tyrrell P et al (2010) Takayasu arteritis in children and adolescents. Rheumatology 49:1806–1814

    Article  PubMed  Google Scholar 

  5. Jennette JC, Falk RJ, Andrassy K et al (1994) Nomenclature of systemic vasculitides: proposal of an international consensus conference. Arthritis Rheum 37:187–192

    Article  CAS  PubMed  Google Scholar 

  6. Jennette JC, Falk RJ, Bacon PA et al (2013) 2012 revised International Chapel Hill consensus conference nomenclature of vasculitides. Arthritis Rheum 65:1–11

    Article  CAS  PubMed  Google Scholar 

  7. 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

    Article  PubMed  Google Scholar 

  8. 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

    Article  PubMed  Google Scholar 

  9. 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

    Article  PubMed Central  CAS  PubMed  Google Scholar 

  10. 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

    Article  PubMed  Google Scholar 

  11. Watts R, Lane S, Scott DG (2005) What is known about the epidemiology of vasculitides? Best Pract Res Clin Rheumatol 19:191–207

    Article  PubMed  Google Scholar 

  12. Cakar N, Yalcinkaya F, Duzova A et al (2008) Takayasu arteritis in children. J Rheumatol 35:913–919

    PubMed  Google Scholar 

  13. 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

    Article  CAS  PubMed  Google Scholar 

  14. 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

    Article  CAS  PubMed  Google Scholar 

  15. Nastri MV, Baptista LP, Baroni RH et al (2004) Gadolinium-enhanced three-dimensional MR angiography of Takayasu arteritis. Radiographics 24:773–786

  16. Alibaz-Oner F, Aydin SZ, Direskeneli H (2013) Advances in the diagnosis, assessment and outcome of Takayasu arteritis. Clin Rheumatol 32:541–546

    Article  PubMed  Google Scholar 

  17. Hausleiter J, Meyer T, Hermann F et al (2009) Estimated radiation dose associated with Cardiac CT angiography. JAMA 301:500–507

    Article  CAS  PubMed  Google Scholar 

  18. 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

    Article  PubMed  Google Scholar 

  19. 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

    CAS  PubMed  Google Scholar 

  20. 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

    CAS  PubMed  Google Scholar 

  21. 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

    Article  PubMed  Google Scholar 

  22. 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

    Article  PubMed  Google Scholar 

  23. 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

    Article  PubMed  Google Scholar 

  24. 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

  25. 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

    Article  PubMed  Google Scholar 

  26. 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

    Article  Google Scholar 

  27. Eleftheriou D, Dillon M, Brogan P (2009) Advances in childhood vasculitis. Curr Opin Rheumatol 21:411–418

    Article  PubMed  Google Scholar 

  28. Schmidt WA (2004) Use of imaging studies in the diagnosis of vasculitis. Curr Rheumatol Rep 6:203–211

    Article  PubMed  Google Scholar 

  29. 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

    PubMed  Google Scholar 

  30. Levine D, Akikusa J, Manson D et al (2007) Chest CT findings in pediatric Wegener’s granulomatosis. Pediatr Radiol 37:57–62

    Article  PubMed  Google Scholar 

  31. Ananthakrishnan L, Sharma N, Kanne J (2009) Wegner’s granulomatosis in the chest: high-resolution CT findings. AJR Am J Roentgenol 192:676–682

    Article  PubMed  Google Scholar 

  32. Connolly B, Manson D, Eberhard A et al (1996) CT appearance of pulmonary vasculitis in children. AJR Am J Roentgenol 167:901–904

    Article  CAS  PubMed  Google Scholar 

  33. Polychronopoulos V, Prakash U, Golbin J et al (2007) Airway involvement in Wegener’s granulomatosis. Rheum Dis Clin N Am 33:755–775

  34. 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

    Article  PubMed  Google Scholar 

  35. McCabe C, Jones Q, Nikolopoulou A et al (2011) Pulmonary-renal syndrome: an update for respiratory physician. Respir Med 105:1413–1421

    Article  PubMed  Google Scholar 

  36. 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

    Article  CAS  PubMed  Google Scholar 

  37. Castaner E, Alguersuari A, Gallardo X et al (2010) When to suspect pulmonary vasculitis: radiologic and clinical clues. Radiographics 30:33–53

    Article  PubMed  Google Scholar 

  38. Saulsbury FT (2001) Henoch-Schonlein purpura. Curr Opin Rheumatol 13:35–40

    Article  CAS  PubMed  Google Scholar 

  39. Rizzi R, Bruno S, Dammacco R (1997) Behcet disease. Int J Clin Lab Res 27:225–232

    Article  CAS  PubMed  Google Scholar 

  40. Kokturk A (2012) Clinical and pathological manifestations with differential diagnosis in Behçet’s disease. Pathol Res Int 2012, 69039010. doi:10.1155/2012/690390

    Article  Google Scholar 

  41. Cellucci T, Benseler SM (2010) Diagnosing central nervous system vasculitis in children. Curr Opin Pediatr 22:731–738

    Article  PubMed  Google Scholar 

  42. Kanekar S, Devgun PA (2014) Pattern approach to focal high white matter signal on magnetic resonance imaging. Radiol Clin N Am 52:241–261

    Article  PubMed  Google Scholar 

  43. 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

    Article  PubMed  Google Scholar 

  44. Gowdie P, Twilt M, Benseler S (2012) Primary and secondary central nervous system vasculitis. J Child Neurol 27:1448–1459

    Article  PubMed  Google Scholar 

  45. 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

    Article  PubMed  Google Scholar 

  46. 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

    Article  PubMed  Google Scholar 

  47. White ML, Zhang Y (2010) Primary angiitis of the central nervous system: apparent diffusion coefficient lesion analysis. Clin Imaging 34:1–6

    Article  PubMed  Google Scholar 

  48. Fugate J, Claassen D, Cloft H et al (2010) Posterior reversible encephalopathy syndrome: associated clinical and radiological findings. Mayo Clin Proc 85:427–432

    Article  PubMed Central  PubMed  Google Scholar 

  49. 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

    CAS  PubMed  Google Scholar 

  50. 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

    PubMed  Google Scholar 

  51. 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

    Article  PubMed  Google Scholar 

  52. 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

    Article  CAS  PubMed  Google Scholar 

  53. 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

    Article  PubMed  Google Scholar 

Download references

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

Authors

Corresponding author

Correspondence to Andrea S. Doria.

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

Reprints and permissions

About this article

Check for updates. Verify currency and authenticity via CrossMark

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

Download citation

  • Received:

  • Revised:

  • Accepted:

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s00247-015-3339-3

Keywords

Navigation