Pediatric Nephrology

, Volume 28, Issue 2, pp 191–196 | Cite as

Renovascular hypertension—is it fibromuscular dysplasia or Takayasu arteritis



Renovascular hypertension (RVH) can be caused by many different diseases, with the most common being fibromuscular dysplasia (FMD) and Takayasu arteritis (TA). A strikingly different diagnostic pattern is seen in children with RVH from different parts of the world. In Europe and North America, these children are mainly diagnosed as having FMD while in Asia and South Africa they will most often get a diagnosis of TA. When comparing the clinical diagnosis for FMD and TA, it becomes obvious that there is a great deal of overlap between the definitions of these two conditions. Different ways to come to the most accurate diagnosis using imaging of the blood vessel wall and positron emission tomography (PET) will be discussed. How an accurate diagnosis should influence the treatment of the children with these conditions will also be addressed.


Renovascular hypertension Midaortic syndrome Fibromuscular dysplasia Takayasu arteritis Positron emission tomography (PET) 


  1. 1.
    Tullus K, Brennan E, Hamilton G, Lord R, McLaren CA, Marks SD, Roebuck DJ (2008) Renovascular hypertension in children. Lancet 371(9622):1453–1463PubMedCrossRefGoogle Scholar
  2. 2.
    Deal JE, Snell MF, Barratt TM, Dillon MJ (1992) Renovascular disease in childhood. J Pediatr 121(3):378–384PubMedCrossRefGoogle Scholar
  3. 3.
    McTaggart SJ, Gulati S, Walker RG, Powell HR, Jones CL (2000) Evaluation and long-term outcome of pediatric renovascular hypertension. Pediatr Nephrol 14(10–11):1022–1029PubMedCrossRefGoogle Scholar
  4. 4.
    Shroff R, Roebuck DJ, Gordon I, Davies R, Stephens S, Marks S, Chan M, Barkovics M, McLaren CA, Shah V, Dillon MJ, Tullus KJ (2006) Angioplasty for renovascular hypertension in children: 20-year experience. Pediatrics 118(1):268–275PubMedCrossRefGoogle Scholar
  5. 5.
    Tummolo A, Marks SD, Stadermann M, Roebuck DJ, McLaren CA, Hamilton G, Dillon MJ, Tullus KG (2009) Mid-aortic syndrome: long-term outcome of 36 children. Pediatr Nephrol 24(11):2225–2232PubMedCrossRefGoogle Scholar
  6. 6.
    Bayazit AK, Yalcinkaya F, Cakar N, Duzova A, Bircan Z, Bakkaloglu A, Canpolat N, Kara N, Sirin A, Ekin M, Oner A, Akman S, Mir S, Baskin E, Poyrazoglu HM, Noyan A, Akil I, Bakkaloglu S, Soylu A (2007) Reno-vascular hypertension in childhood: a nationwide survey. Pediatr Nephrol 22(9):1327–1333PubMedCrossRefGoogle Scholar
  7. 7.
    D'Souza SJ, Tsai WS, Silver MM, Chait P, Benson LN, Silverman E, Hébert D, Balfe JW (1998) Diagnosis and management of stenotic aorto-arteriopathy in childhood. J Pediatr 132(6):1016–1022PubMedCrossRefGoogle Scholar
  8. 8.
    Daniels SR, Loggie JM, McEnery PT, Towbin RB (1987) Clinical spectrum of intrinsic renovascular hypertension in children. Pediatrics 80(5):698–704PubMedGoogle Scholar
  9. 9.
    Kumar P, Arora P, Kher V, Rai PK, Gulati S, Baijal SS, Kumar S (1996) Malignant hypertension in children in India. Nephrol Dial Transplant 11(7):1261–1266PubMedCrossRefGoogle Scholar
  10. 10.
    Stadermann MB, Montini G, Hamilton G, Roebuck DJ, McLaren CA, Dillon MJ, Marks SD, Tullus K (2010) Results of surgical treatment for renovascular hypertension in children: 30-year single-centre experience. Nephrol Dial Transplant 25(3):807–813PubMedCrossRefGoogle Scholar
  11. 11.
    Stanley JC, Zelenock GB, Messina LM, Wakefield TW (1995) Pediatric renovascular hypertension: a thirty-year experience of operative treatment. J Vasc Surg 21(2):212–226PubMedCrossRefGoogle Scholar
  12. 12.
    Stanley JC, Criado E, Upchurch GR Jr, Brophy PD, Cho KJ, Rectenwald JE, Michigan Pediatric Renovascular Group, Kershaw DB, Williams DM, Berguer, Henke PK, Wakefield TW (2006) Pediatric renovascular hypertension: 132 primary and 30 secondary operations in 97 children. J Vasc Surg 44(6):1219–1228PubMedCrossRefGoogle Scholar
  13. 13.
    Tyagi S, Kaul UA, Satsangi DK, Arora R (1997) Percutaneous transluminal angioplasty for renovascular hypertension in children: initial and long-term results. Pediatrics 99(1):44–49PubMedCrossRefGoogle Scholar
  14. 14.
    Olin JW, Sealove BA (2011) Diagnosis, management, and future developments of fibromuscular dysplasia. J Vasc Surg 53(3):826–836PubMedCrossRefGoogle Scholar
  15. 15.
    Slovut DP, Olin JW (2004) Fibromuscular dysplasia. N Engl J Med 350(18):1862–1871PubMedCrossRefGoogle Scholar
  16. 16.
    Blondin D, Lanzman R, Schellhammer F, Oels M, Grotemeyer D, Baldus SE, Rump LC, Sandmann W, Voiculescu A (2010) Fibromuscular dysplasia in living renal donors: still a challenge to computed tomographic angiography. Eur J Radiol 75(1):67–71PubMedCrossRefGoogle Scholar
  17. 17.
    Cragg AH, Smith TP, Thompson BH, Maroney TP, Stanson AW, Shaw GT, Hunter DW, Cocharn ST (1989) Incidental fibromuscular dysplasia in potential renal donors: long-term clinical follow-up. Radiology 172(1):145–147PubMedGoogle Scholar
  18. 18.
    Neymark E, LaBerge JM, Hirose R, Melzer JS, Kerlan RK Jr, Wilson MW, Gordon RL (2000) Arteriographic detection of renovascular disease in potential renal donors: incidence and effect on donor surgery. Radiology 214(3):755–760PubMedGoogle Scholar
  19. 19.
    Harrison EG Jr, Hunt JC, Bernatz PE (1967) Morphology of fibromuscular dysplasia of the renal artery in renovascular hypertension. Am J Med 43(1):97–112PubMedCrossRefGoogle Scholar
  20. 20.
    Stanley JC, Fry WJ (1975) Renovascular hypertension secondary to arterial fibrodysplasia in adults: criteria for operation and results of surgical therapy. Arch Surg 110(8):922–928PubMedCrossRefGoogle Scholar
  21. 21.
    Stanley JC, Gewertz BL, Fry WJ (1976) Renal: systemic renin indices and renal vein renin ratios as prognostic indicators in remedial renovascular hypertension. J Surg Res 20(3):149–155PubMedCrossRefGoogle Scholar
  22. 22.
    Marks SD, Gullett AM, Brennan E, Tullus K, Jaureguiberry G, Klootwijk E, Stanescu HC, Kleta R, Woolf AS (2011) Renal FMD may not confer a familial hypertensive risk nor is it caused by ACTA2 mutations. Pediatr Nephrol 26(10):1857–1861PubMedCrossRefGoogle Scholar
  23. 23.
    Forsey J, Dhandayuthapani G, Hamilton M, Martin R, Tizard E, Ramanan A (2011) Takayasu arteritis: key clinical factors for early diagnosis. Arch Dis Child Educ Pract Ed 96(5):176–182PubMedCrossRefGoogle Scholar
  24. 24.
    Gulati A, Bagga A (2010) Large vessel vasculitis. Pediatr Nephrol 25(6):1037–1048PubMedCrossRefGoogle Scholar
  25. 25.
    Kerr GS, Hallahan CW, Giordano J, Leavitt RY, Fauci AS, Rottem M, Hoffman GS (1994) Takayasu arteritis. Ann Intern Med 120(11):919–929PubMedGoogle Scholar
  26. 26.
    Ishikawa K (1988) Diagnostic approach and proposed criteria for the clinical diagnosis of Takayasu's arteriopathy. J Am Coll Cardiol 12(4):964–972PubMedCrossRefGoogle Scholar
  27. 27.
    Arend WP, Michel BA, Bloch DA, Hunder GG, Calabrese LH, Edworthy SM, Fauci AS (1990) The American College of Rheumatology 1990 criteria for the classification of Takayasu arteritis. Arthritis Rheum 33(8):1129–1134PubMedCrossRefGoogle Scholar
  28. 28.
    Dillon MJ, Ozen S (2006) A new international classification of childhood vasculitis. Pediatr Nephrol 21(9):1219–1222PubMedCrossRefGoogle Scholar
  29. 29.
    Ozen S, Ruperto N, Dillon MJ, Bagga A, Barron K, Davin JC, Kawasaki T, Lindsley C, Petty RE, Prieur AM, Ravelli A, Woo P (2006) EULAR/PReS endorsed consensus criteria for the classification of childhood vasculitides. Ann Rheum Dis 65(7):936–941PubMedCrossRefGoogle Scholar
  30. 30.
    Ozen S, Pistorio A, Iusan SM, Bakkaloglu A, Herlin T, Brik R, Buoncompagni A, Lazar C, Bilge I, Uziel Y, Rigante D, Cantarini L, Hilario MO, Silva CA, Alegria M, Norambuena X, Belot A, Berkun Y, Estrella AI, Olivieri AN, Alpigiani MG, Rumba I, Sztajnbok F, Tambic-Bukovac L, Breda L, Al-Mayouf S, Mihaylova D, Chasnyk V, Sengler C, Klein-Gitelman M, Djeddi D, Nuno L, Pruunsild C, Brunner J, Kondi A, Pagava K, Pederzoli S, Martini A, Ruperto N, Paediatric Rheumatology International Trials Organisation (PRINTO) (2010) EULAR/PRINTO/PRES criteria for Henoch-Schönlein purpura, childhood polyarteritis nodosa, childhood Wegener granulomatosis and childhood Takayasu arteritis: Ankara 2008. Part II: Final classification criteria. Ann Rheum Dis 69(5):798–806PubMedCrossRefGoogle Scholar
  31. 31.
    Desai MY, Stone JH, Foo TK, Hellmann DB, Lima JA, Bluemke DA (2005) Delayed contrast-enhanced MRI of the aortic wall in Takayasu's arteritis: initial experience. AJR Am J Roentgenol 184(5):1427–1431PubMedGoogle Scholar
  32. 32.
    Blockmans D (2011) PET in vasculitis. Ann N Y Acad Sci 1228:64–70PubMedCrossRefGoogle Scholar
  33. 33.
    Arnaud L, Haroche J, Mathian A, Gorochov G, Amoura Z (2011) Pathogenesis of Takayasu's arteritis: a 2011 update. Autoimmun Rev 11(1):61–67PubMedCrossRefGoogle Scholar
  34. 34.
    Lee SG, Ryu JS, Kim HO, Oh JS, Kim YG, Lee CK, Yoo B (2009) Evaluation of disease activity using F-18 FDG PET-CT in patients with Takayasu arteritis. Clin Nucl Med 34(11):749–752PubMedCrossRefGoogle Scholar
  35. 35.
    Tanaka F, Kawakami A, Iwanaga N, Tamai M, Izumi Y, Aratake K, Arima K, Kamachi M, Nakamura H, Huang M, Ida H, Origuchi T, Eguchi K (2006) Infliximab is effective for Takayasu arteritis refractory to glucocorticoid and methotrexate. Intern Med 45(5):313–316PubMedCrossRefGoogle Scholar
  36. 36.
    Valsakumar AK, Valappil UC, Jorapur V, Garg N, Nityanand S, Sinha N (2003) Role of immunosuppressive therapy on clinical, immunological, and angiographic outcome in active Takayasu's arteritis. J Rheumatol 30(8):1793–1798PubMedGoogle Scholar
  37. 37.
    Shinjo SK, Pereira RM, Tizziani VA, Radu AS, Levy-Neto M (2007) Mycophenolate mofetil reduces disease activity and steroid dosage in Takayasu arteritis. Clin Rheumatol 26(11):1871–1875PubMedCrossRefGoogle Scholar
  38. 38.
    Hoffman GS, Merkel PA, Brasington RD, Lenschow DJ, Liang P (2004) Anti-tumor necrosis factor therapy in patients with difficult to treat Takayasu arteritis. Arthritis Rheum 50(7):2296–2304PubMedCrossRefGoogle Scholar
  39. 39.
    Ozen S, Duzova A, Bakkaloglu A, Bilginer Y, Cil BE, Demircin M, Davin JC, Bakkaloglu M (2007) Takayasu arteritis in children: preliminary experience with cyclophosphamide induction and corticosteroids followed by methotrexate. J Pediatr 150(1):72–76PubMedCrossRefGoogle Scholar
  40. 40.
    Srinivasan A, Krishnamurthy G, Fontalvo-Herazo L, Nijs E, Meyers K, Kaplan B, Cahill AM (2011) Spectrum of renal findings in pediatric fibromuscular dysplasia and neurofibromatosis type 1. Pediatr Radiol 41(3):308–316PubMedCrossRefGoogle Scholar
  41. 41.
    Courtel JV, Soto B, Niaudet P, Gagnadoux MF, Carteret M, Quignodon JF, Brunelle F (1998) Percutaneous transluminal angioplasty of renal artery stenosis in children. Pediatr Radiol 28(1):59–63PubMedCrossRefGoogle Scholar
  42. 42.
    McCulloch M, Andronikou S, Goddard E, Sinclair P, Lawrenson J, Mandelstam S, Beningfield SJ, Millar AJ (2003) Angiographic features of 26 children with Takayasu's arteritis. Pediatr Radiol 33(4):230–235PubMedGoogle Scholar
  43. 43.
    Hahn D, Thomson PD, Kala U, Beale PG, Levin SE (1998) A review of Takayasu's arteritis in children in Gauteng, South Africa. Pediatr Nephrol 12(8):668–675PubMedCrossRefGoogle Scholar

Copyright information

© IPNA 2012

Authors and Affiliations

  1. 1.Great Ormond St Hospital - Paediatric NephrologyLondonUK

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