eMedicine Specialties > Pediatrics: General Medicine > Pulmonology

Wegener Granulomatosis: Multimedia

Author: Rudolph P Valentini, MD, Director of Dialysis Services, Associate Professor, Department of Pediatrics, Division of Pediatric Nephrology, Wayne State University; Vice Chief of Staff, Children's Hospital of Michigan
Coauthor(s): Debbie S Toder, MD, Director of Cystic Fibrosis Center, Department of Pediatrics, Division of Pulmonary Medicine, Assistant Professor, Wayne State University and Children's Hospital of Michigan
Contributor Information and Disclosures

Updated: Mar 5, 2009

Multimedia

Shown is a chest radiograph of an 11-year-old gir...Media file 1: Shown is a chest radiograph of an 11-year-old girl who presented with an upper respiratory tract infection, myalgias, and arthralgias for 1 month followed by an abrupt presentation with pallor, hemoptysis, and hypertension. Her bilateral fluffy infiltrates are suggestive of a pulmonary hemorrhage. She had an antineutrophil cytoplasmic autoantibodies (ANCA)–positive pauci-immune necrotizing and crescentic glomerulonephritis (GN) associated with her pulmonary hemorrhage. Supportive therapy consisted of mechanical ventilation and hemodialysis along with immunosuppressive therapy. Her anti–glomerular basement membrane antibody test result was negative. Nearly 2 years later, she had a serum creatinine of 0.7 mg/dL and no residual pulmonary disease.
Shown is a chest radiograph of an 11-year-old gir...

Shown is a chest radiograph of an 11-year-old girl who presented with an upper respiratory tract infection, myalgias, and arthralgias for 1 month followed by an abrupt presentation with pallor, hemoptysis, and hypertension. Her bilateral fluffy infiltrates are suggestive of a pulmonary hemorrhage. She had an antineutrophil cytoplasmic autoantibodies (ANCA)–positive pauci-immune necrotizing and crescentic glomerulonephritis (GN) associated with her pulmonary hemorrhage. Supportive therapy consisted of mechanical ventilation and hemodialysis along with immunosuppressive therapy. Her anti–glomerular basement membrane antibody test result was negative. Nearly 2 years later, she had a serum creatinine of 0.7 mg/dL and no residual pulmonary disease.

An 11-year-old girl presented with an upper respi...Media file 2: An 11-year-old girl presented with an upper respiratory tract infection, myalgias, and arthralgias for 1 month followed by an abrupt presentation with pallor, hemoptysis, and hypertension. She had an antineutrophil cytoplasmic autoantibodies (ANCA)–positive pauci-immune necrotizing and crescentic glomerulonephritis (GN) associated with her pulmonary hemorrhage. A follow-up chest radiograph obtained several days later shows a complete resolution of her pulmonary infiltrates. This rapid resolution is more consistent with hemorrhage than with pneumonia.
An 11-year-old girl presented with an upper respi...

An 11-year-old girl presented with an upper respiratory tract infection, myalgias, and arthralgias for 1 month followed by an abrupt presentation with pallor, hemoptysis, and hypertension. She had an antineutrophil cytoplasmic autoantibodies (ANCA)–positive pauci-immune necrotizing and crescentic glomerulonephritis (GN) associated with her pulmonary hemorrhage. A follow-up chest radiograph obtained several days later shows a complete resolution of her pulmonary infiltrates. This rapid resolution is more consistent with hemorrhage than with pneumonia.

A renal biopsy specimen from a 13-year-old girl w...Media file 3: A renal biopsy specimen from a 13-year-old girl with antineutrophil cytoplasmic antibody (cANCA)–positive pulmonary renal syndrome. Seven weeks after presenting with sinusitis, she presented with an acute abdomen, pulmonary hemorrhage, and acute renal failure (creatinine 4.9 mg/dL). This biopsy specimen shows a necrotizing and crescentic glomerulonephritis (Silver stain).
A renal biopsy specimen from a 13-year-old girl w...

A renal biopsy specimen from a 13-year-old girl with antineutrophil cytoplasmic antibody (cANCA)–positive pulmonary renal syndrome. Seven weeks after presenting with sinusitis, she presented with an acute abdomen, pulmonary hemorrhage, and acute renal failure (creatinine 4.9 mg/dL). This biopsy specimen shows a necrotizing and crescentic glomerulonephritis (Silver stain).

More on Wegener Granulomatosis

Overview: Wegener Granulomatosis
Differential Diagnoses & Workup: Wegener Granulomatosis
Treatment & Medication: Wegener Granulomatosis
Follow-up: Wegener Granulomatosis
Multimedia: Wegener Granulomatosis
References

References

  1. Jennette JC, Falk RJ, Andrassy K, et al. Nomenclature of systemic vasculitides. Proposal of an international consensus conference. Arthritis Rheum. Feb 1994;37(2):187-92. [Medline].

  2. Breda L, Nozzi M, De Sanctis S, Chiarelli F. Laboratory Tests in the Diagnosis and Follow-Up of Pediatric Rheumatic Diseases: An Update. Semin Arthritis Rheum. Feb 24 2009;[Medline].

  3. Fauci AS, Haynes BF, Katz P, Wolff SM. Wegener's granulomatosis: prospective clinical and therapeutic experience with 85 patients for 21 years. Ann Intern Med. Jan 1983;98(1):76-85. [Medline].

  4. Hogan SL, Satterly KK, Dooley MA, Nachman PH, Jennette JC, Falk RJ. Silica exposure in anti-neutrophil cytoplasmic autoantibody-associated glomerulonephritis and lupus nephritis. J Am Soc Nephrol. Jan 2001;12(1):134-42. [Medline].

  5. Schlieben DJ, Korbet SM, Kimura RE, Schwartz MM, Lewis EJ. Pulmonary-renal syndrome in a newborn with placental transmission of ANCAs. Am J Kidney Dis. Apr 2005;45(4):758-61. [Medline].

  6. Cotch MF, Hoffman GS, Yerg DE, Kaufman GI, Targonski P, Kaslow RA. The epidemiology of Wegener's granulomatosis. Estimates of the five-year period prevalence, annual mortality, and geographic disease distribution from population-based data sources. Arthritis Rheum. Jan 1996;39(1):87-92. [Medline].

  7. US Renal Data System. Annual Data Report. In: Atlas of End-Stage Renal Disease in the United States. Vol 2. Bethesda, MD: National Institutes of Health, National Institute of Diabetes and Digestive and Kidney Diseases; 2008.

  8. North American Pediatric Renal Trials and Collaborative Studies 2007. Annual Report. Dialysis Access Data. NAPRTCS. Available at https://web.emmes.com/study/ped/. Accessed February 12, 2009.

  9. Hoffman GS, Kerr GS, Leavitt RY, et al. Wegener granulomatosis: an analysis of 158 patients. Ann Intern Med. Mar 15 1992;116(6):488-98. [Medline].

  10. Rottem M, Fauci AS, Hallahan CW, et al. Wegener granulomatosis in children and adolescents: clinical presentation and outcome. J Pediatr. Jan 1993;122(1):26-31. [Medline].

  11. Ellis EN, Wood EG, Berry P. Spectrum of disease associated with anti-neutrophil cytoplasmic autoantibodies in pediatric patients. J Pediatr. Jan 1995;126(1):40-3. [Medline].

  12. Valentini RP, Smoyer WE, Sedman AB, Kershaw DB, Gregory MJ, Bunchman TE. Outcome of antineutrophil cytoplasmic autoantibodies-positive glomerulonephritis and vasculitis in children: a single-center experience. J Pediatr. Feb 1998;132(2):325-8. [Medline].

  13. Hattori M, Kurayama H, Koitabashi Y. Antineutrophil cytoplasmic autoantibody-associated glomerulonephritis in children. J Am Soc Nephrol. Jul 2001;12(7):1493-500. [Medline].

  14. Luqmani RA, Bacon PA, Moots RJ, et al. Birmingham Vasculitis Activity Score (BVAS) in systemic necrotizing vasculitis. QJM. Nov 1994;87(11):671-8. [Medline].

  15. Sneller MC. Rituximab and Wegener's granulomatosis: are B cells a target in vasculitis treatment?. Arthritis Rheum. Jan 2005;52(1):1-5. [Medline].

  16. [Best Evidence] Jayne DR, Gaskin G, Rasmussen N, Abramowicz D, Ferrario F, Guillevin L. Randomized trial of plasma exchange or high-dosage methylprednisolone as adjunctive therapy for severe renal vasculitis. J Am Soc Nephrol. Jul 2007;18(7):2180-8. [Medline].

  17. Keogh KA, Ytterberg SR, Fervenza FC, Carlson KA, Schroeder DR, Specks U. Rituximab for refractory Wegener's granulomatosis: report of a prospective, open-label pilot trial. Am J Respir Crit Care Med. Jan 15 2006;173(2):180-7. [Medline].

  18. Nowack R, Gobel U, Klooker P, Hergesell O, Andrassy K, van der Woude FJ. Mycophenolate mofetil for maintenance therapy of Wegener's granulomatosis and microscopic polyangiitis: a pilot study in 11 patients with renal involvement. J Am Soc Nephrol. Sep 1999;10(9):1965-71. [Medline].

  19. Joy MS, Hogan SL, Jennette JC, Falk RJ, Nachman PH. A pilot study using mycophenolate mofetil in relapsing or resistant ANCA small vessel vasculitis. Nephrol Dial Transplant. Dec 2005;20(12):2725-32. [Medline].

  20. Stegeman CA, Tervaert JW, de Jong PE, Kallenberg CG. Trimethoprim-sulfamethoxazole (co-trimoxazole) for the prevention of relapses of Wegener's granulomatosis. Dutch Co-Trimoxazole Wegener Study Group. N Engl J Med. Jul 4 1996;335(1):16-20. [Medline].

  21. Wegener's Granulomatosis Etanercept Trial (WGET) Research Group. Etanercept plus standard therapy for Wegener's granulomatosis. N Engl J Med. Jan 27 2005;352(4):351-61. [Medline].

  22. Patel AM, Lehman TJ. Rituximab for severe refractory pediatric Wegener granulomatosis. J Clin Rheumatol. Oct 2008;14(5):278-80. [Medline].

  23. Gottlieb BS, Miller LC, Ilowite NT. Methotrexate treatment of Wegener granulomatosis in children. J Pediatr. Oct 1996;129(4):604-7. [Medline].

  24. Hoffman GS, Leavitt RY, Kerr GS, Fauci AS. The treatment of Wegener's granulomatosis with glucocorticoids and methotrexate. Arthritis Rheum. Nov 1992;35(11):1322-9. [Medline].

  25. Hogan SL, Nachman PH, Wilkman AS, Jennette JC, Falk RJ. Prognostic markers in patients with antineutrophil cytoplasmic autoantibody-associated microscopic polyangiitis and glomerulonephritis. J Am Soc Nephrol. Jan 1996;7(1):23-32. [Medline].

  26. Jagiello P, Aries P, Arning L, et al. The PTPN22 620W allele is a risk factor for Wegener's granulomatosis. Arthritis Rheum. Dec 2005;52(12):4039-43. [Medline].

  27. Jayne D, Rasmussen N, Andrassy K, et al. A randomized trial of maintenance therapy for vasculitis associated with antineutrophil cytoplasmic autoantibodies. N Engl J Med. Jul 3 2003;349(1):36-44. [Medline].

  28. Kist-van Holthe JE, Ho PL, Stablein D, Harmon WE, Baum MA. Outcome of renal transplantation for Wilms' tumor and Denys-Drash syndrome: a report of the North American Pediatric Renal Transplant Cooperative Study. Pediatr Transplant. Jun 2005;9(3):305-10. [Medline].

  29. Lamprecht P, Voswinkel J, Lilienthal T, et al. Effectiveness of TNF-alpha blockade with infliximab in refractory Wegener's granulomatosis. Rheumatology (Oxford). Nov 2002;41(11):1303-7. [Medline].

  30. Lynch JP 3rd, Hoffman GS. Wegener's granulomatosis: controversies and current concepts. Compr Ther. Sep 1998;24(9):421-40. [Medline].

  31. Pediatric end-stage renal disease. USRDS. United States Renal Data System. Am J Kidney Dis. Aug 1997;30(2 Suppl 1):S128-44. [Medline].

  32. Sneller MC, Fauci AS. Pathogenesis of vasculitis syndromes. Med Clin North Am. Jan 1997;81(1):221-42. [Medline].

  33. Taketomo CK, Hodding JH, Kraus DM. Pediatric Dosage Handbook. 4th ed. Hudson, Ohio: Lexi-comp Inc; 1997.

Further Reading

Keywords

Wegener granulomatosis, WG, Wegener's granulomatosis, ANCA disease, antineutrophil cytoplasmic autoantibodies disease, ANCA-associated vasculitis, ANCA-associated glomerulonephritis, ANCA-associated GN, antineutrophil cytoplasmic autoantibodies–associated vasculitis, antineutrophil cytoplasmic autoantibodies–associated glomerulonephritis, antineutrophil cytoplasmic autoantibodies–associated GN, pulmonary renal syndrome, necrotizing granulomatous lesions of the upper or lower respiratory tract, generalized necrotizing vasculitis involving both arteries and veins, focal necrotizing glomerulonephritis, microscopic polyangiitis, Churg-Strauss syndrome, microscopic polyangiitis, MPA, end-stage renal disease, ESRD, sinusitis, otitis media, subglottic stenosis, pulmonary infiltrates, pulmonary nodules, hemoptysis, hematuria, proteinuria, arthralgia, arthritis, rhinorrhea, epistaxis, pharyngitis, congestive heart failure, CHF

Contributor Information and Disclosures

Author

Rudolph P Valentini, MD, Director of Dialysis Services, Associate Professor, Department of Pediatrics, Division of Pediatric Nephrology, Wayne State University; Vice Chief of Staff, Children's Hospital of Michigan
Rudolph P Valentini, MD is a member of the following medical societies: American Academy of Pediatrics, American Society of Nephrology, and American Society of Pediatric Nephrology
Disclosure: Nothing to disclose.

Coauthor(s)

Debbie S Toder, MD, Director of Cystic Fibrosis Center, Department of Pediatrics, Division of Pulmonary Medicine, Assistant Professor, Wayne State University and Children's Hospital of Michigan
Debbie S Toder, MD is a member of the following medical societies: American Academy of Pediatrics and American Thoracic Society
Disclosure: Nothing to disclose.

Medical Editor

Girish D Sharma, MD, Associate Professor, Department of Pediatrics, Rush University Medical Center, Rush Children's Hospital; Director of Pediatric Pulmonary Section and Rush Cystic Fibrosis Center
Girish D Sharma, MD is a member of the following medical societies: American Academy of Pediatrics, American College of Chest Physicians, American Thoracic Society, and Royal College of Physicians of Ireland
Disclosure: Nothing to disclose.

Pharmacy Editor

Mary L Windle, PharmD, Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy, Pharmacy Editor, eMedicine
Disclosure: Pfizer Inc Stock Investment from financial planner; Avanir Pharma Stock Investment from financial planner ; WebMD Salary and stock Employment and investment from financial planner

Managing Editor

Heidi Connolly, MD, Associate Professor of Pediatrics and Psychiatry, University of Rochester; Director, Pediatric Sleep Medicine Services, Strong Sleep Disorders Center
Heidi Connolly, MD is a member of the following medical societies: American Academy of Pediatrics, American Thoracic Society, and Society of Critical Care Medicine
Disclosure: Nothing to disclose.

CME Editor

Mary E Cataletto, MD, Associate Director, Division of Pediatric Pulmonology, Winthrop University Hospital; Professor of Clinical Pediatrics, State University of New York at Stony Brook; Director of Children's Sleep Services, Winthrop University Hospital
Mary E Cataletto, MD is a member of the following medical societies: American Academy of Pediatrics and American College of Chest Physicians
Disclosure: Shering Plough Pharmaceuticals Honoraria Consulting

Chief Editor

Michael R Bye, MD, Professor of Clinical Pediatrics, Division of Pulmonary Medicine, Columbia University College of Physicians and Surgeons; Attending Physician, Pediatric Pulmonary Medicine, Morgan Stanley Children's Hospital of New York Presbyterian, Columbia University Medical Center
Michael R Bye, MD is a member of the following medical societies: American Academy of Pediatrics, American College of Chest Physicians, and American Thoracic Society
Disclosure: Merck Honoraria Speaking and teaching

 
 
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