Skip to main content

Advertisement

SpringerLink
Log in

Wegener granulomatosis in childhood and adolescence

  • Review
  • Published:
European Journal of Pediatrics Aims and scope Submit manuscript

Abstract

Wegener granulomatosis (WG) is a necrotising granulomatous small vessel vasculitis with a clinical predilection for involvement of the upper airways, lungs and kidneys, which occurs at all ages. The aetiology of WG, like other primary systemic vasculitides, remains unknown. Existing evidence suggests an autoimmune inflammatory process, characterised by early lesions with neutrophils and endothelial cells as active participants and involvement of antineutrophil cytoplasmatic antibodies (ANCA) directed against proteinase-3 (PR3). However, other concomitant factors like infections and environmental factors also appear to be necessary for the development of WG. In addition, multiple genetic factors seem to be involved in disease susceptibility. Whereas the first publications on WG in childhood were based on case reports, some studies in recent years allow to compare clinical findings, disease course, morbidity and mortality rates for childhood and adult onset patients. Whereas most aspects of WG are similar at all ages, some features appear to be significantly different. WG in childhood is more frequently complicated by subglottic stenosis and nasal deformity while treatment-related morbidity and malignancies are less common compared to adults. Introduction of combined treatment with cyclophosphamide and glucocorticoids resulted in a dramatic improvement of patient outcome; however, commonly occurring disease relapses and the risk of chronic organ damage at all ages make long-term follow-up of all patients and the establishment of new therapeutic regimens necessary.

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

Access this article

Log in via an institution

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

Instant access to the full article PDF.

Institutional subscriptions

Fig. 1

Similar content being viewed by others

Abbreviations

ANCA :

antineutrophil cytoplasmatic antibodies

c-ANCA :

cytoplasmatic pattern of ANCA

MPO :

myeloperoxidase

PR3 :

proteinase-3

WG :

Wegener granulomatosis

References

  1. Aasarød K, Bostad L, Hammerstrøm J, Jørstad S, Iversen BM (2001) Renal histopathology and clinical course in 94 patients with Wegener’s granulomatosis. Nephrol Dial Transplant 16: 953–960

    Article  PubMed  Google Scholar 

  2. Athreya BH (1995) Vasculitis in children. Pediatr Clin North Am 42: 1239–1261

    Google Scholar 

  3. Bacon PA, Carruthers D (2001) New therapeutic aspects: haemopoietic stem cell transplantation. Best Pract Res Clin Rheumatol 15: 299–313

    Article  CAS  PubMed  Google Scholar 

  4. Backman A, Grahne B, Holopainen E, Leisti J, Paavolainen M (1979) Wegener’s granulomatosis in childhood. A clinical report based on 3 cases. Int J Pediatr Otorhinolaryngol 1: 145–149

    Article  CAS  PubMed  Google Scholar 

  5. Baliga R, Chang CH, Bidani AK, Perrin EV, Fleischmann LE (1978) A case of generalized Wegener’s granulomatosis in childhood: successful therapy with cyclophosphamide. Pediatrics 61: 286–290

    CAS  PubMed  Google Scholar 

  6. Beggs AD, Hain SF (2002) F-18FDG-positron emission tomographic scanning and Wegener’s granulomatosis. Clin Nuc Med 27: 705–706

    Article  Google Scholar 

  7. Belostotsky VM, Shah V, Dillon MJ (2002) Clinical features in 17 pediatric patients with Wegener granulomatosis. Pediatr Nephrol 17: 754–761

    Article  PubMed  Google Scholar 

  8. Brouwer E, Huitema MG, Klok PA, Cohen Tervaert JW, Weening JJ, Kallenberg CGM (1993) Anti-meloperoxidase associated proliferative glomerulonephritis: an animal model. J Exp Med 177: 905–914

    CAS  PubMed  Google Scholar 

  9. Brouwer E, Huitema MG, Mulder AHL, Heeringa P, van Goor H, Cohen Terveart JW, Weening JJ, Kallenberg CG (1994) Neutrophil activation in vitro and in vivo in Wegener’s granulomatosis. Kidney Int 45: 1120–1131

    CAS  PubMed  Google Scholar 

  10. Charles L, Falk R, Jennette J (1992) Reactivity of antineutrophil cytoplasmic antibodies with mononuclear phagocytes. J Leuk Biol 51: 65–68

    CAS  Google Scholar 

  11. Cohen Tervaert JW, van der Woude FJ, Fauci AS, Ambrus JL, Velosa J, Keane WF, Meijer S, van der Giessen M, van der Hem GK, The TH (1989) Association between active Wegener’s granulomatosis and anticytoplasmic antibodies. Arch Intern Med 149: 2461–2465

    Article  PubMed  Google Scholar 

  12. Cohen Tervaert JW, Huitema MG, Hené RJ, Sluiter WJ, The TH, van der Hem GK, Kallenberg CGM (1990) Prevention of relapses in Wegener’s granulomatosis by treatment based on antineutrophil cytoplasmic antibody titre. Lancet 336: 709–711

    Article  PubMed  Google Scholar 

  13. Davenport A (1992) False-positive perinuclear and cytoplasmic antibody results leading to misdiagnosis of Wegener’s granulomatosis and/or microscopic polyarteritis. Clin Nephrol 37: 124–130

    CAS  PubMed  Google Scholar 

  14. Davies DJ, Moran JE, Niall JF, Ryan GB (1982) Segmental necrotizing glomerulonephritis with antineutrophil antibody: possible arbovirus aetiology ? BMJ 285: 606

    CAS  PubMed  Google Scholar 

  15. De Groot K, Reinhold-Keller E, Tatsis E, Paulsen J, Heller M, Nölle B, Gross WL (1996) Therapy for the maintenance of remission in sixty-five patients with generalized Wegener’s granulomatosis. Methotrexate versus trimethoprim/sulphamethoxazole. Arthritis Rheum 39: 2052–2061

    Google Scholar 

  16. DeRemee RA, McDonald TJ, Weiland LH (1985) Wegener’s granulomatosis: observations on treatment with antimicrobial agents. Mayo Clin Proc 60: 27–32

    CAS  PubMed  Google Scholar 

  17. Dijstelbloem HM, Scheepers RH, Oost WW, Stegeman CA, van der Pol WL, Sluiter WJ, Kallenberg CG, van de Winkel JG, Cohen Tervaert JW (1999) Fc gamma receptor polymorphisms in Wegener’s granulomatosis: risk factors for disease relapse. Arthritis Rheum 42: 1823–1827

    Article  CAS  PubMed  Google Scholar 

  18. Dillon MJ, Ansell BA (1995) Vasculitis in children and adolescents. Rheum Dis Clin North Am 21: 1115–1136

    CAS  PubMed  Google Scholar 

  19. Elmedhem A, Adu D, Savage COS (2003) Relapse rate and outcome of ANCA-associated small vessel vasculitis after transplantation. Nephrol Dial Transplant 18: 1001–1004

    Article  PubMed  Google Scholar 

  20. Esnault VL, Audrain MA, Sesboue R (1997) Alpha-1-antitrypsin phenotyping in ANCA-associated diseases: one of several arguments for protease/antiprotease imbalance in systemic vasculitis. Exp Clin Immunogenet 14: 206–213

    CAS  PubMed  Google Scholar 

  21. Falk RJ, Terrell RS, Charles LA, Jennette JC (1990) Anti-neutrophil cytoplasmic autoantibodies induce neutrophils to degranulate and produce oxygen radicals in vitro. Proc Natl Acad Sci USA 87: 4115–4119

    CAS  PubMed  Google Scholar 

  22. Fauci AS, Katz P, Haynes BF, Wolff SM (1979) Cyclophosphamide therapy of severe systemic necrotizing vasculitis. N Engl J Med 301: 235–238

    CAS  PubMed  Google Scholar 

  23. Gencik M, Muller S, Borgmann S, Fricke H (2000) Proteinase 3 gene polymorphisms and Wegener’s granulomatosis. Kidney Int 58: 2473–2477

    Article  CAS  PubMed  Google Scholar 

  24. Guillevin L, Pagnoux C (2003) Indications of plasma exchanges for systemic vasculitides. Therap Apher Dial 7: 155–160

    Article  PubMed  Google Scholar 

  25. Guillevin L, Cordier JF, Lhote F, Cohen P, Jarrousse B, Royer I, Lesavre P, Jacquot C, Bindi P, Bielefeld P, Desson JF, Détrée F, Dubois A, Hachulla E, Hoen B, Jacomy D, Seigneuric C, Lauque D, Stern M, Longy-Boursier M (1997) A prospective, multicenter, randomised trial comparing steroids and pulse cyclophosphamide versus steroids and oral cyclophosphamide in the treatment of generalized Wegener’s granulomatosis. Arthritis Rheum 40: 2187–2198

    Google Scholar 

  26. Haas JP, Metzler M, Ruder H, Waldherr R, Boswald M, Rupprecht T (2002) An unusual manifestation of Wegener’s granulomatosis in a 4-year-old girl. Pediatr Neurol 27: 71–74

    Article  PubMed  Google Scholar 

  27. Harper L, Savage COS (2000) Pathogenesis of ANCA-associated systemic vasculitis. J Pathol 190: 349–359

    Article  CAS  PubMed  Google Scholar 

  28. Haubitz M, Schellong S, Göbel U, Schurek HJ, Schaumann D, Koch KM, Brunkhorst R (1998) Intravenous pulse administration of cyclophosphamide versus daily oral treatment in patients with antineutrophil cytoplasmatic antibody-associated vasculitis and renal involvement. A prospective, randomised study. Arthritis Rheum 41: 1835–1844

    Article  CAS  PubMed  Google Scholar 

  29. Haynes BF, Fishman ML, Fauci AS, Wolff SM (1977) The ocular manifestations of Wegener’s granulomatosis. Fifteen years experience and review of the literature. Am J Med 63: 131–141

    CAS  PubMed  Google Scholar 

  30. Hoffman GS (1997) Treatment of Wegener’s granulomatosis: time to change the standard of care? Arthritis Rheum 40: 2099–2104

    Google Scholar 

  31. Hoffman GS, Kerr GS, Leavitt RY, Hallahan CW, Lebovics RS, Travis WD, Rottem M, Fauci AS (1992) Wegner granulomatosis: an analysis of 158 patients. Ann Int Med 116: 488–498

    CAS  PubMed  Google Scholar 

  32. Hoffman GS, Thomas-Golbanov CK, Chan J, Akst LM, Eliachar I (2003) Treatment of subglottic stenosis, due to Wegener’s granulomatosis, with intralesional corticosteroids and dilation. J Rheumatol 30: 1017–1021

    PubMed  Google Scholar 

  33. Hollander D, Manning RT (1967) The use of alkylating agents in the treatment of Wegener’s granulomatosis. Ann Intern Med 67: 393–398

    CAS  PubMed  Google Scholar 

  34. Israel HL (1988) Sulfamethoxazole-trimethoprim therapy for Wegener’s granulomatosis. Arch Intern Med 148: 2293–2295

    Article  CAS  PubMed  Google Scholar 

  35. Jayne DR, Gaskin G, Pusey CD, Lockwood CM (1995) ANCA and predicting relapse in systemic vasculitis. QJM 88: 127–133

    CAS  PubMed  Google Scholar 

  36. Jennette JC, Falk RJ, Andrassy K, Bacon PA, Churg J, Gross WL, Hagen C, Hoffman GS, Hunder GG, Kallenberg CGM, McCluskey RT, Sinico RA, Rees AJ, van Es LA, Waldherr R, Wiik A (1994) Nomenclature of systemic vasculitides – Proposal of an International Consensus Conference. Arthritis Rheum 37: 187–192

    CAS  PubMed  Google Scholar 

  37. Kallenberg CGM, Rarok A, Stegeman CA, Limburg PC (2002) New insights into the pathogenesis of antineutrophil cytoplasmic autoantibody-associated vasculitis. Autoimmunity Rev 1: 61–66

    Article  CAS  Google Scholar 

  38. Kaplan AA (1999) Therapeutic apheresis for renal disorders. Ther Apheresis 3: 25–30

    Article  CAS  Google Scholar 

  39. Kerr GS, Fleisher TA, Hallahan CW, Leavitt RY, Fauci AS, Hoffman GS (1993) Limited prognostic value of changes in antineutrophil cytoplasmatic antibody titer in patients with Wegener’s granulomatosis. Arthritis Rheum 36: 365–371

    Google Scholar 

  40. Klinger H (1931) Grenzformen der periarteritis Nodosa. Frankfurt Z Pathol 42: 455–480

    Google Scholar 

  41. Koldingsnes W, Nossent JC (2003) Baseline features and initial treatment as predictors of remission and relapse in Wegener granulomatosis. J Rheumatol 30: 80–88

    PubMed  Google Scholar 

  42. Langford CA (1998) The diagnostic utility of c-ANCA in Wegener’s granulomatosis. Clev Clin J Med 65: 135–140

    CAS  Google Scholar 

  43. Langford CA, Hoffman GS (1999) Wegener’s granulomatosis. Thorax 54: 629–637

    CAS  PubMed  Google Scholar 

  44. Langford CA, Sneller MC, Hallahan CW, Hoffman GS, Kammerer WA, Talar-Williams C, Fauci A, Lebovics RS (1996) Clinical features and therapeutic management of subglottic stenosis in patients with Wegener’s granulomatosis. Arthritis Rheum 39: 1754–1760

    Google Scholar 

  45. Langford CA, Talar-Williams C, Sneller MC (2000) Use of methotrexate and glucocorticois in the treatment of Wegener’s granulomatosis. Long-term renal outcome in patients with glomerulonephritis. Arthritis Rheum 43: 1836–1840

    Google Scholar 

  46. Langford CA, Talar-Williams C, Barron KS, Sneller MC (2003) Use of a cylophosphamide-induction Methotrexate-maintenance regimen for the treatment of Wegener’s granulomatosis: extended follow-up and rate of relapse. Am J Med 114: 463–469

    Article  CAS  PubMed  Google Scholar 

  47. Leawitt RY, Fauci AS, Bloch DA, Michel BA, Hunder GG, Arend WP, Calabrese LH, Fries JF, Lie JT, Lightfoot RW Jr, Masi AT, McShane DJ, Mills JA, Stevens MB, Wallace SL, Zvaifler NJ (1990) The American College of Rheumatology 1990 criteria for the classification of Wegener’s granulomatosis. Arthritis Rheum 33: 1101–1107

    CAS  PubMed  Google Scholar 

  48. Lee KS, Kim TS, Fujimoto K, Moriya H, Watanabe H, Tateishi U, Ashizawa K, Johkoh T, Kim EA, Kwon OJ (2003) Thoracic manifestation of Wegener’s granulomatosis: CT findings in 30 patients. Eur Radiol 13: 43–51

    Article  PubMed  Google Scholar 

  49. Levine SM, Stone JH (2001) New approaches to treatment in systemic vasculitis: biological therapies. Best Pract Res Clin Rheumatol 15: 315–333

    Article  CAS  PubMed  Google Scholar 

  50. Lie JT and the Members and Consultants of the American College of Rheumatology Subcommittee on Classification of Vasculitis (1990) Illustrated histopathologic classification criteria for selected vasculitis syndromes. Arthritis Rheum 33: 1074–1087

    CAS  PubMed  Google Scholar 

  51. Mahr A, Girad T, Agher R, Guillevin L (2001) Analysis of factors predictive of survival based on 49 patients with systemic Wegener’s granulomatosis and prospective follow-up. Rheumatology 40: 492–498

    Article  CAS  PubMed  Google Scholar 

  52. Matic G, Michelsen A, Hofmann D, Winkler R, Tiess M, Schneidewind JM, Müller W, Ramlow W (2001) Three cases of c-ANCA-positive vasculitis treated with immunoadsoption: possible benefit in early treatment. Ther Apheresis 5: 68–72

    Article  CAS  Google Scholar 

  53. McHugh K, Manson D, Eberhard BA, Shore A, Laxer RM (1991) Wegener’s granulomatosis in childhood. Pediatr Radiol 21: 552–555

    CAS  PubMed  Google Scholar 

  54. Moorthy AV, Chesney RW, Segar WE, Groshong T (1977) Wegener granulomatosis in childhood: prolonged survival following cytotoxic therapy. J Pediatr 91: 616–618

    CAS  PubMed  Google Scholar 

  55. Nespoli L, Duse M, Vitiello MA, Perinotto G, Fiocca R, Giannetti A, Colombo A (1979) A rapid unfavorable outcome of Wegener’s granulomatosis in early childhood. Eur J Pediatr 131: 277–282

    CAS  PubMed  Google Scholar 

  56. Neumann G, Benz-Bohm G, Rister M (1984) Wegener’s granulomatosis in childhood. Review of the literature and case report. Pediatr Radiol 14: 267–271

    CAS  PubMed  Google Scholar 

  57. Niles JL, McCluskey RT, Ahmad MF, Arnaout MA (1989) Wegener’s granulomatosis autoantigen is a novel neutrophil serine proteinase. Blood 74: 1888–1893

    CAS  PubMed  Google Scholar 

  58. Nölle B, Specks U, Ludermann J, Rohrbach MS, DeRemee RA, Gross WL (1989) Anticytoplasmic autoantibodies: their immunodignostic value in Wegener’s granulomatosis. Ann Intern Med 111: 28–40

    Google Scholar 

  59. Patry YC, Trewick DC, Gregoire M, Audrian MA, Moreau AM, Muller JY, Meflah K, Esnault VL (2001) Rats injected with syngeneic rat apoptotic neutrophils develop antineutrophil cytoplasmic antibodies. J Am Soc Nephrol 12: 1764–1768

    CAS  PubMed  Google Scholar 

  60. Phillips MJ (1998) Stenting therapy for stenosing airway diseases. Respirology 3: 215–219

    CAS  PubMed  Google Scholar 

  61. Radford DJ, Savage COS, Nash GB (2000) Treatment of rolling neutrophils with antineutrophil cytoplasmic antibodies causes conversion to firm integrin-mediazed adhesion. Arthritis Rheum 43: 1337–1345

    Google Scholar 

  62. Rao JK, Weinberger M, Oddone EZ, Allen NB, Landsman P, Feussner JR (1995) The role of antineutrophil cytoplasmic antibody (c-ANCA) testing in the diagnosis of Wegener’s granulomatosis. A literature review and meta-analysis. Ann Intern Med 123: 925–932

    Google Scholar 

  63. Reinhold-Keller E, Beuge N, Latza U, de Groot K, Rudert H, Nölle B, Heller M, Gross WL (2000) An interdisciplinary approach to the care of patients with Wegener’s granulomatosis. Long-term outcome in 155 patients. Arthritis Rheum 43: 1021–1032

    CAS  PubMed  Google Scholar 

  64. Reinhold-Keller E, Herlyn K, Wagner-Bastmeyer R, Gutfleisch J, Peter HH, Raspe HH, Gross WL (2002) Effect of Wegener’s granulomatosis on work disability, need for medical care, and quality of life in patients younger than 40 years at diagnosis. Arthritis Rheum 47: 320–325

    Google Scholar 

  65. Reinhold-Keller E, Fink COE, Herlyn K, Gross WL, de Groot K (2002) High rate of renal relapse in 71 patients with Wegener’s granulomatosis under maintenance of remission with low-dose methotrexate. Arthritis Rheum 47: 326–332

    Google Scholar 

  66. Reumaux D, Vossebeld PJ, Roos D, Verhoeven AJ (1995) Effect of tumor necrosis factor-induced integrin activation on Fc gamma receptor II-mediated signal transduction: relevance for activation of neutrophils by anti-proteinase 3 or anti-myeloperoxidase antibodies. Blood 86: 3189–3195

    CAS  PubMed  Google Scholar 

  67. Roberti I, Reisman L, Churg J (1993) Vasculitis in childhood. Pediatr Nephrol 7: 479–489

    CAS  PubMed  Google Scholar 

  68. Rottem M, Fauci AS, Hallahan CW, Kerr GS, Lebovics R, Leavitt RY, Hoffman GS (1993) Wegener granulomatosis in children and adolescents: clinical presentation and outcome. J Pediatr 122: 26–31

    CAS  PubMed  Google Scholar 

  69. Savage CO, Pottinger BE, Gaskin G, Pusey CD, Pearson JD (1992) Autoantibodies developing to myeloperoxidase and proteinase 3 in systemic vasculitis stimulate neutrophil cytotoxicity toward cultured endothelial cells. Am J Pathol 141: 335–342

    CAS  PubMed  Google Scholar 

  70. Savage COS, Harper L, Adu D (1997) Primary systemic vasculitis. Lancet 349: 553–558

    Article  CAS  PubMed  Google Scholar 

  71. Sedivá A, Kolárová I, Bartunková J (1998) Antineutrophil cytoplasmatic antibodies in children. Eur J Pediatr 157: 987–991

    Article  PubMed  Google Scholar 

  72. Segelmark M, Elzouki AN, Wieslander J, Eriksson S (1995) The PiZ gene of α1-antitrypsin as a determinant of outcome in PR3-ANCA-positive vasculitis. Kidney Int 48: 844–850

    CAS  PubMed  Google Scholar 

  73. Sneller MC, Hoffman GS, Talar-Williams C, Kerr GS, Hallahan CW, Fauci AS (1995) An analysis of forty-two Wegener’s granulomatosis patients treated with methotrexate and prednisone. Arthritis Rheum 38: 608–613

    CAS  PubMed  Google Scholar 

  74. Staepperts I, Van Laer C, Deschepper K, Van de Heyning P, Vermeire P (2000) Endoscopic management of severe subglottic stenosis in Wegener’s granulomatosis. Clin Rheumatol 19: 315–317

    Article  PubMed  Google Scholar 

  75. Stegeman CA, Cohen Tervaert JW, Sluiter WJ, Manson W, de Jong PE, Kallenberg CGM (1994) Association of chronic nasal carriage of Staphylococcus aureus and higher relapse rates in Wegener’s granulomatosis. Ann Intern Med 113: 12–17

    Google Scholar 

  76. Stegeman CA, Cohen Tervaert JW, de Jong PE, Kallenberg CGM (1996) Trimethoprim-sulfamethoxazole for the prevention of relapses of Wegener’s granulomatosis. N Engl J Med 335: 16–20

    Article  CAS  PubMed  Google Scholar 

  77. Stegmayr BG, Gothefors L, Malmer B, Müller-Wiefel DE, Nilsson K, Sundelin B (2000) Wegener granulomatosis in children and young adults. A case of ten patients. Pediatr Nephrol 14: 208–213

    CAS  Google Scholar 

  78. Stone JH, Uhlfelder ML, Hellmann DB, Crook S, Bedocs NM, Hoffman GS (2001) Etanercept combined with conventional treatment in Wegener’s granulomatosis: a six-month open-label trial to evaluate safety. Arthritis Rheum 44: 1149–1154

    Article  CAS  PubMed  Google Scholar 

  79. Stone JH and the Wegener’s Granulomatosis Etanercept Trial Research Group (2003) Limited versus severe Wegener’s granulomatosis. Baseline data on patients in the Wegener’s granulomatosis etanercept trial. Arthritis Rheum 48: 2299–2309

    Article  PubMed  Google Scholar 

  80. Travis WD, Hoffman GS, Leavitt RY, Pass HI, Fauci AS (1991) Surgical pathology of the lung in Wegener’s granulomatosis. Review of 87 open lung biopsies from 67 patients. Am J Surg Pathol 15: 315–333

    CAS  PubMed  Google Scholar 

  81. Tyndall A, Passweg J, Gratwohl A (2001) Haemopoietic stem cell transplantation in the treatment of severe autoimmune diseases 2000. Ann Rheum Dis 60: 702–707

    Article  CAS  PubMed  Google Scholar 

  82. Valentini RP, Smøyer WE, Sedman AB, Kershaw DB, Gregory MJ, Bunchman TE (1998) Outcome of antineutrophil cytoplasmic autoantibodies-positive glomerulonephritis and vasculitis in children: a single-center experience J Pediatr 132: 325–328

    Google Scholar 

  83. Van der Woude FJ, Rasmussen N, Lobatto S, Wiik A, Permin H, van Es LA, van der Giessen M, van der Hem GK, The TH (1985) Autoantibodies against neutrophils and monocytes: tool for diagnosis and marker of disease activity in Wegener’s granulomatosis. Lancet 1: 425–429

    PubMed  Google Scholar 

  84. Von Scheven E, Lee C, Berg BO (1998) Pediatric Wegener’s granulomatosis complicated by central nervous system vasculitis. Pediatr Neurol 19: 317–319

    Article  PubMed  Google Scholar 

  85. Walton EW (1958) Giant cell granuloma of the respiratory tract (Wegener’s granulomatosis). BMJ 2: 265–270

    Google Scholar 

  86. Wegener F (1936) Über generalisierte, septische Gefäßerkrankungen. Verh Dtsch Ges Pathol 29: 202–210

    Google Scholar 

  87. Wegener F (1939) Über eine eigenartige rhinogene Granulomatose mit besonderer Beteiligung des Arterien Systems und der Nieren. Beitr Pathol Anat Allg Pathol 36–68

  88. Weiss M, Crissman J (1984) Renal biopsy findings in Wegener’s granulomatosis: segmental necrotizing glomerulonephritis with glomerular thrombosis. Human Pathol 15: 943–956

    CAS  Google Scholar 

  89. Westman KWA, Bygren PG, Olsson H, Ranstam J, Wieslander J (1998) Relapse rate, renal survival, and cancer morbidity in patients with Wegener’s granulomatosis or microscopic polyangiitis with renal involvement. J Am Soc Nephrol 9: 842–852

    CAS  PubMed  Google Scholar 

Download references

Acknowledgements

The authors thank Prof. Dr. R. Waldherr, Institute of Pathology, Heidelberg, Germany, for the histopathology of kidney involvement.

Author information

Authors and Affiliations

  1. Department of Paediatrics, University of Münster, Albert-Schweitzer-Strasse 33, 48129 , Münster, Germany

    Michael Frosch & Dirk Foell

  2. Institute of Experimental Dermatology, University of Münster, Münster, Germany

    Dirk Foell

Authors
  1. Michael Frosch
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. Dirk Foell
    View author publications

    You can also search for this author in PubMed Google Scholar

Corresponding author

Correspondence to Michael Frosch.

Rights and permissions

Reprints and permissions

About this article

Cite this article

Frosch, M., Foell, D. Wegener granulomatosis in childhood and adolescence. Eur J Pediatr 163, 425–434 (2004). https://doi.org/10.1007/s00431-004-1464-3

Download citation

  • Received:

  • Revised:

  • Accepted:

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s00431-004-1464-3

Keywords

Search

Navigation