Pediatric Nephrology

, Volume 21, Issue 1, pp 46–53

Childhood microscopic polyangiitis associated with MPO-ANCA

  • Amira Peco-Antic
  • Branka Bonaci-Nikolic
  • Gordana Basta-Jovanovic
  • Mirjana Kostic
  • Jasmina Markovic-Lipkovski
  • Milos Nikolic
  • Brankica Spasojevic
Original Article


We reviewed the clinical, histological and serological parameters of microscopic polyangiitis (MPA) associated with antineutrophil cytoplasmic antibodies (ANCA) specific to myeloperoxidase (MPO). Six girls and one boy aged 12.0±2.6 years (7–15 years) met the following inclusion criteria: (1) clinical manifestations of systemic small vessel involvement; (2) histological demonstration of pauci-immune necrotizing glomerulonephritis; and (3) serological findings of increased concentration of MPO-ANCA by ELISA test. The main clinical manifestations were: influenza-like symptoms (100%), hematuria/proteinuria (100%), purpura (100%), pulmonary-renal syndrome (57%), acute renal failure (ARF) (29%), ischemic cerebral insults (29%), and necrotizing vasculitis of the skin (29%). All patients underwent renal biopsy examined by immunohistochemistry with expression of alpha-smooth muscle actin (alpha SMA) in glomerular and interstitial spaces. Patients were followed from 6 months to 5.5 years (35.4± 23.2 months). None of the patients died. Two of seven children who had ARF progressed to end stage renal disease; one developed chronic renal failure, and four normalized renal function. ARF and central nervous system involvement at presentation were parameters of poor renal outcome. A high score of fibro-cellular glomerular crescents was associated with worse prognosis. Early treatment enables a favorable prognosis of MPO-ANCA-associated MPA in children.


Acute renal failure Alpha SMA ANCA Childhood Microscopic polyangiitis 


  1. 1.
    Jennette JC, Falk RJ (1997) Small-vessel vasculitis. N Engl J Med 337:1512–1523PubMedCrossRefGoogle Scholar
  2. 2.
    Jayne DR, Rasmussen N (1997) Treatment of antineutrophil cytoplasm autoantibody-associated systemic vasculitis: initiatives of the European Community Systemic Vasculitis Clinical Trials Study Group. Mayo Clin Proc 72:737–747PubMedCrossRefGoogle Scholar
  3. 3.
    Hoffman GS, Specks U (1998) Antineutrophil cytoplasmic antibodies. Arthritis Rheum 41:1521–1537PubMedCrossRefGoogle Scholar
  4. 4.
    Wiik A (2002) Rational use of ANCA in the diagnosis of vasculitis. Rheumatology 41:481–483PubMedCrossRefGoogle Scholar
  5. 5.
    Jennette JC (2003) Nephrology forum: rapidly progressive crescentic glomerulonephritis. Kidney Int 63:1164–1177PubMedCrossRefGoogle Scholar
  6. 6.
    Guillevin L, Durand-Gasselin B, Cevalos R, Gayraud M, Lhote F, Callard P, Amouroux J, Casassus P, Jarrousse B (1999) Microscopic polyangiitis: clinical and laboratory findings in eighty-five patients. Arthritis Rheum 42:421–430PubMedCrossRefGoogle Scholar
  7. 7.
    Hagen EC, Daha MR, Hermans J, Andrassy K, Csernok E, Gaskin G, Lesevre P, Ludemann J, Rasmussen N, Sinco RA, Wiik A, van der Woude FJ (1998) Diagnostic value of standardized assays for anti-neutrophil cytoplasmic antibodies in idiopathic systemic vasculitis. EC/BCR Project for ANCA Assay Standardization. Kidney Int 53:743–753PubMedCrossRefGoogle Scholar
  8. 8.
    Schmitt WH, van der Woude FJ (2004) Clinical application of antineutrophil cytoplasmic antibody testing. Curr Opin Rheumatol 16:9–17PubMedCrossRefGoogle Scholar
  9. 9.
    Mahr A, Guillevin L, Poissonnet M, Ayme S (2004) Prevalence of polyarteritis nodosa, microscopic polyangiitis, Wegener’s granulomatosis and Churg Strauss syndrome in a French urban multiethnic population in 2000: a capture-recapture estimate. Arthritis Rheum 51:92–99PubMedCrossRefGoogle Scholar
  10. 10.
    Dillon MJ (1999) Vasculitis. In: Barratt TM, Avner ED, Harmon WE (eds) Pediatric nephrology, 4th edn. Lippincott, Baltimore, pp 779–792Google Scholar
  11. 11.
    Falcini F (2004) Vascular and connective tissue diseases in the paediatric world. Lupus 13:77–84PubMedCrossRefGoogle Scholar
  12. 12.
    Luqmani RA, Bacon PA, Moots RJ, Janssen BA, Pall A, Emery P, Savage C, Adu D (1994) Birmingham vasculitis activity score (BVAS) in systemic necrotizing vasculitis. QJM 87:671–678PubMedGoogle Scholar
  13. 13.
    Besbas N, Ozen S, Saatci U, Topaloğlu R, Tinaztepe K, Bakkaloglu A (2000) Renal involvement in polyarteritis nodosa: evaluation of 26 Turkish children. Pediatr Nephrol 14:325–327PubMedCrossRefGoogle Scholar
  14. 14.
    The Fourth Report on the Diagnosis, Evaluation, and Treatment of High Blood Pressure in Children and Adolescents (2004) National High Blood Pressure Education Program Working Group on High Blood Pressure in Children and Adolescents. Pediatrics 114:555–576CrossRefGoogle Scholar
  15. 15.
    Liu Y (2004) Epithelial to mesenchymal transition in renal fibrogenesis: pathologic significance, molecular mechanism, and therapeutic intervention. J Am Soc Nephrol 15:1–12PubMedCrossRefGoogle Scholar
  16. 16.
    Kiernan JA (1990) Fixation. Histological histochemical methods. Pergamon Press, Oxford, pp 10–32Google Scholar
  17. 17.
    Brouet JC, Clauvel JP, Danon F, Klein M, Seligmann M (1974) Biologic and clinical significance of cryoglobulins. A report of 86 cases. Am J Med 57:775–788PubMedCrossRefGoogle Scholar
  18. 18.
    Pomper MG, Miller TJ, Stone JH, Tidmore WC, Hellmann DB (1999) CNS vasculitis in autoimmune disease: MR imaging findings and correlation with angiography. AJNR Am J Neuroradiol 20:75–85PubMedGoogle Scholar
  19. 19.
    Savage CO, Winearls CG, Evans DJ, Rees AJ, Lockwood CM (1985) Microscopic polyarteritis: presentation, pathology and prognosis. Q J Med 56:467–483PubMedGoogle Scholar
  20. 20.
    Reinhold-Keller E, Herlyn K, Wagner-Bastmeyer R, Gutfleisch J, Peter HH, Raspe HH, Gross WL (2002) No difference in the incidences of vasculitides between north and south Germany: first results of the German vasculitis register. Rheumatology 41:540–549PubMedCrossRefGoogle Scholar
  21. 21.
    Watts RA, Gonzalez-Gay MA, Lane SE, Garcia-Porrua C, Bentham G, Scott DG (2001) Geoepidemiology of systemic vasculitis: comparison of the incidence in two regions of Europe. Ann Rheum Dis 60:170–172PubMedCentralPubMedCrossRefGoogle Scholar
  22. 22.
    Gonzalez Gay MA, Garcia-Porrua C (1999) Systemic vasculitis in adults in northwestern Spain, 1988–1997. Clinical and epidemiologic aspects. Medicine (Baltimore) 78:292–308Google Scholar
  23. 23.
    El-Reshaid K, Kapoor M, El-Reshaid W, Madda JP, Varro J (1997) The spectrum of renal disease associated with microscopic polyangiitis and classic polyarteritis nodosa in Kuwait. Nephrol Dial Transplant 12:1874–1882PubMedCrossRefGoogle Scholar
  24. 24.
    Hattori M, Kurayama H, Koitabashi Y; Japanese Society for Pediatric Nephrology (2001) Antineutrophil cytoplasmic autoantibody-associated glomerulonephritis in children. J Am Soc Nephrol 12:1493–1500PubMedGoogle Scholar
  25. 25.
    Yu F, Zhao MH, Huang JP, Yao Y, Zou WZ, Zhang YK, Wang HY (2003) Clinical and pathological features of anti-neutrophil cytoplasm antibody associated systemic vasculitis in children. Zhonghua Er Ke Za Zhi 41:831–834PubMedGoogle Scholar
  26. 26.
    Tidman M, Olander R, Svalander C, Danielsson D (1998) Patients hospitalized because of small vessel vasculitides with renal involvement in the period 1975–95: organ involvement, antineutrophil cytoplasmic antibodies patterns, seasonal attack rates and fluctuation of annual frequencies. J Intern Med 244:133–141PubMedCrossRefGoogle Scholar
  27. 27.
    Falk RJ, Hogan S, Carey TS, Jennette JC (1990) Clinical course of anti-neutrophil cytoplasmic autoantibody-associated glomerulonephritis and systemic vasculitis. The Glomerular Disease Collaborative Network. Ann Intern Med 113:656–663PubMedCrossRefGoogle Scholar
  28. 28.
    Somer T, Finegold SM (1995) Vasculitides associated with infections, immunisation and antimicrobial drugs. Clin Infect Dis 20:1010–1036PubMedCrossRefGoogle Scholar
  29. 29.
    Hellmich B, Ehren M, Lindstaedt M, Meyer M, Pfohl M, Schatz H (2001) Anti-MPO-ANCA-positive microscopic polyangiitis following subacute bacterial endocarditis. Clin Rheumatol 20:441–443PubMedCrossRefGoogle Scholar
  30. 30.
    Chou TN, Hsu TS, Chen RM, Lin LI, Tsay GJ (2000) Parvovirus B19 infection associated with the production of anti-neutrophil cytoplasmic antibody (ANCA) and anticardiolipin antibody (aCL). Lupus 9:551–554PubMedCrossRefGoogle Scholar
  31. 31.
    Song JH, Lee KJ, Lee SW, Han Jy, Kim MJ (2001) Fatal pulmonary-renal syndrome manifested with immune complex crescentic glomerulonephritis in a patient with MPO-ANCA seropositivity. Yonsei Med J 42:425–430PubMedGoogle Scholar
  32. 32.
    Merkel PA (1998) Drugs associated with vasculitis. Curr Opin Rheumatol 10:45–50PubMedCrossRefGoogle Scholar
  33. 33.
    Bakkaloglu A, Ozen S, Baskin E, Besbas N, Gur-Guven A, Kasapcopur O, Tinaztepe K (2001) The significance of antineutrophil cytoplasmic antibody in microscopic polyangiitis and classic polyarteritis nodosa. Arch Dis Child 85:427–430PubMedCentralPubMedCrossRefGoogle Scholar
  34. 34.
    Blanco Filho F, Ernesto LC, Rosa MA, Stuginsky LA, Ziochevsky ER, Blanco F (2001) Rapidly progressive antineutrophil cytoplasm antibodies associated with pulmonary-renal syndrome in 10-year-old girl. Sao Paulo Med J 119:29–32PubMedCrossRefGoogle Scholar
  35. 35.
    Bosch X, Mirapeiz E, Font J, Borrellas X, Rodriguez R, Lopez-Soto A, Ingelmo M, Rovert L (1991) Prognostic implication of anti-neutrophil cytoplasmic autoantibodies with myeloperoxidase specificity in anti-glomerular basement membrane disease. Clin Nephrol 36:107–113PubMedGoogle Scholar
  36. 36.
    Rydel JJ, Rodby R (1998) An 18-year-old man with Goodpasture’s syndrome and ANCA-negative central nervous system vasculitis. Am J Kidney Dis 2:345–349CrossRefGoogle Scholar
  37. 37.
    Jennette JC, Falk RJ, Andrassy K, Bacon PA, Churg J, Gross WL, Hagen EC, Hoffman GS, Hunder GG, Kallenberg CG et al (1994) Nomenclature of systemic vasculitides. Proposal of an international consensus conference. Arthritis Rheum 37:187–192PubMedCrossRefGoogle Scholar
  38. 38.
    Peňas PF, Porras JI, Fraga J, Bernis C, Sarriá C, Daudén E (1996) Microscopic polyangiitis. A systemic vasculitis with a positive P-ANCA. Br J Dermatol 134:542–547CrossRefGoogle Scholar
  39. 39.
    Ozen S, Anton J, Arisoy N, Bakkaloglu A, Besbas N, Brogan P, Garcia-Consuegra J, Dolezalova P, Dressler F, Duzova A, Ferriani VP, Hilario MO, Ibanez-Rubio M, Kasapcopur O, Kuis W, Lehman TJ, Nemcova D, Nielsen S, Oliveira SK, Schikler K, Sztajnbok F, Terreri T, Zulian F, Woo P (2004) Juvenile polyarteritis: results of a multicenter survey of 110 children. J Pediatr 145:517–522PubMedCrossRefGoogle Scholar
  40. 40.
    Kyndt X, Reumaux D, Bridoux F, Tribout B, Bataille P, Hachulla E, Hatron PY, Douthilleul P, Vanhille P (1999) Serial measurements of antineutrophil cytoplasmic autoantibodies in patients with systemic vasculitis. Am J Med 106:527–533PubMedCrossRefGoogle Scholar
  41. 41.
    Bonaci-Nikolic B, Andrejevic S, Bukilica M, Nikolic MM (2002) Clinical and prognostic value of antineutrophil cytoplasmic antibodies in Wegener’s granulomatosis and microscopic polyangiitis: comment on the article by Russell et al. Arthritis Rheum 46:278–280PubMedCrossRefGoogle Scholar
  42. 42.
    Schultz DR, Diego JM (2000) Antineutrophil cytoplasmic antibodies (ANCA) and systemic vasculitis: update of assays, immunopathogenesis, controversies, and report of a novel de novo ANCA-associated vasculitis after kidney transplantation. Semin Arthritis Rheum 29:267–285PubMedCrossRefGoogle Scholar
  43. 43.
    Locke IC, Leaker B, Cambridge G (1999) A comparison of the characteristics of circulating anti-myeloperoxidase autoantibodies in vasculitis with those in non-vasculitic conditions. Clin Exp Immunol 115:369–376PubMedCentralPubMedCrossRefGoogle Scholar
  44. 44.
    Baskin E, Bakkaloglu A, Besbas N, Hascelik G, Saatci U, Gök F, Ozen S (2002) Ceruloplasmin levels in antineutrophil cytoplasmic antibody-positive patients. Pediatr Nephrol 17:917–919PubMedCrossRefGoogle Scholar
  45. 45.
    Popa ER, Franssen CF, Limburg PC, Huitema MG, Kallenberg CG, Tervaert JW (2002) In vitro cytokine production and proliferation of T cells from patients with anti-proteinase 3- and antimyeloperoxidase-associated vasculitis, in response to proteinase 3 and myeloperoxidase. Arthritis Rheum 46:1894–904PubMedCrossRefGoogle Scholar
  46. 46.
    Arranz O, Ara J, Rodriguez R, Saurina A, Mirapiex E, Darnell A (2000) Serum levels of soluble interleukin-2 receptor in patients with ANCA-associated vasculitis. J Nephrol 13:59–64PubMedGoogle Scholar
  47. 47.
    Hauer HA, Bajema IM, van Houwelingen HC, Ferrario F, Noël LH, Waldherr R, Jayne DR, Rasmussen N, Bruijn JA, Hagen EC; European Vasculitis Study group (EUVAS) (2002) Determinants of outcome in ANCA-associated glomerulonephritis: a prospective clinico-histopathological analysis of 96 patients. Kidney Int 62:1732–1742PubMedCrossRefGoogle Scholar
  48. 48.
    Kamesh L, Harper L, Savage CO (2002) ANCA-positive vasculitis. J Am Soc Nephrol 13:1953–1960PubMedCrossRefGoogle Scholar
  49. 49.
    Haas M, Eustace JA (2004) Immune complex deposits in ANCA-associated crescentic glomerulonephritis: a study of 126 cases. Kidney Int 65:2145–2152PubMedCrossRefGoogle Scholar
  50. 50.
    Reumaux D, Duthilleul P, Ross D (2004) Pathogenesis of diseases associated with antineutrophil cytoplasm autoantibodies. Human Immunol 65:1–12CrossRefGoogle Scholar

Copyright information

© IPNA 2005

Authors and Affiliations

  • Amira Peco-Antic
    • 1
  • Branka Bonaci-Nikolic
    • 2
  • Gordana Basta-Jovanovic
    • 2
  • Mirjana Kostic
    • 1
  • Jasmina Markovic-Lipkovski
    • 3
  • Milos Nikolic
    • 4
  • Brankica Spasojevic
    • 1
  1. 1.Department of NephrologyUniversity Children’s HospitalBelgradeSerbia
  2. 2.Institute of Allergy and Clinical ImmunologyClinical Centre of SerbiaBelgradeSerbia
  3. 3.Institute of PathologySchool of Medicine, University of BelgradeBelgradeSerbia
  4. 4.Clinical Centre of SerbiaInstitute of DermatologyBelgradeSerbia

Personalised recommendations