eMedicine Specialties > Rheumatology > Vasculitis

Wegener Granulomatosis: Multimedia

Author: Patricia J Papadopoulos, MD, Fellow, Department of Rheumatology, Walter Reed Army Medical Center, Washington, DC
Coauthor(s): Robert John O'Brian, MD, Staff Rheumatologist, National Naval Medical Center
Contributor Information and Disclosures

Updated: Aug 4, 2009

Multimedia

Saddle nose deformity in a 26-year-old man with W...Media file 1: Saddle nose deformity in a 26-year-old man with Wegener granulomatosis. Image courtesy of P. Papadopoulos, MD.
Saddle nose deformity in a 26-year-old man with W...

Saddle nose deformity in a 26-year-old man with Wegener granulomatosis. Image courtesy of P. Papadopoulos, MD.

Lung biopsy specimen from a patient with Wegener ...Media file 2: Lung biopsy specimen from a patient with Wegener granulomatosis showing evidence of vasculitis and inflammation (high-power view). Image courtesy of Z. Xu, MD.
Lung biopsy specimen from a patient with Wegener ...

Lung biopsy specimen from a patient with Wegener granulomatosis showing evidence of vasculitis and inflammation (high-power view). Image courtesy of Z. Xu, MD.

Lung biopsy specimen from a patient with Wegener ...Media file 3: Lung biopsy specimen from a patient with Wegener granulomatosis showing evidence of vasculitis and inflammation (high-power view). Image courtesy of Z. Xu, MD.
Lung biopsy specimen from a patient with Wegener ...

Lung biopsy specimen from a patient with Wegener granulomatosis showing evidence of vasculitis and inflammation (high-power view). Image courtesy of Z. Xu, MD.

Focal glomerulonephritis with crescent formation ...Media file 4: Focal glomerulonephritis with crescent formation on renal biopsy specimen, characteristic of Wegener granulomatosis.
Focal glomerulonephritis with crescent formation ...

Focal glomerulonephritis with crescent formation on renal biopsy specimen, characteristic of Wegener granulomatosis.

C-ANCA immunofluorescence pattern. Staining for a...Media file 5: C-ANCA immunofluorescence pattern. Staining for antineutrophil cytoplasmic antibody by indirect immunofluorescence shows heavy cytoplasmic staining, whereas nuclei are nonreactive (see Images 5-6). Image courtesy of K. Orr, MD.
C-ANCA immunofluorescence pattern. Staining for a...

C-ANCA immunofluorescence pattern. Staining for antineutrophil cytoplasmic antibody by indirect immunofluorescence shows heavy cytoplasmic staining, whereas nuclei are nonreactive (see Images 5-6). Image courtesy of K. Orr, MD.

P-ANCA immunofluorescence pattern. Perinuclear an...Media file 6: P-ANCA immunofluorescence pattern. Perinuclear antineutrophil cytoplasmic antibody staining pattern by indirect immunofluorescence shows perinuclear staining, whereas cytoplasm is nonreactive (see Image 4 and Image 6). Image courtesy of K. Orr, MD.
P-ANCA immunofluorescence pattern. Perinuclear an...

P-ANCA immunofluorescence pattern. Perinuclear antineutrophil cytoplasmic antibody staining pattern by indirect immunofluorescence shows perinuclear staining, whereas cytoplasm is nonreactive (see Image 4 and Image 6). Image courtesy of K. Orr, MD.

Goodpasture syndrome: Linear deposition of immuno...Media file 7: Goodpasture syndrome: Linear deposition of immunoglobulin G and C3 are observed on a renal biopsy specimen from a patient with Goodpasture syndrome. Immunofluorescence staining. Image courtesy of K. Orr, MD.
Goodpasture syndrome: Linear deposition of immuno...

Goodpasture syndrome: Linear deposition of immunoglobulin G and C3 are observed on a renal biopsy specimen from a patient with Goodpasture syndrome. Immunofluorescence staining. Image courtesy of K. Orr, MD.

Extensive thickening of the maxillary sinuses in ...Media file 8: Extensive thickening of the maxillary sinuses in a patient with Wegener granulomatosis. The patient also had intermittent epistaxis.
Extensive thickening of the maxillary sinuses in ...

Extensive thickening of the maxillary sinuses in a patient with Wegener granulomatosis. The patient also had intermittent epistaxis.

Wegener granulomatosis. Bilateral nodules observe...Media file 9: Wegener granulomatosis. Bilateral nodules observed on a plain chest radiograph in a patient with hemoptysis and hematuria. Image courtesy of G. Eschun, MD.
Wegener granulomatosis. Bilateral nodules observe...

Wegener granulomatosis. Bilateral nodules observed on a plain chest radiograph in a patient with hemoptysis and hematuria. Image courtesy of G. Eschun, MD.

This 42-year-old man presented with hemoptysis, w...Media file 10: This 42-year-old man presented with hemoptysis, weight loss, and night sweats. He was diagnosed with the limited form of Wegener granulomatosis.
This 42-year-old man presented with hemoptysis, w...

This 42-year-old man presented with hemoptysis, weight loss, and night sweats. He was diagnosed with the limited form of Wegener granulomatosis.

Bilateral cavitating nodules in a patient with We...Media file 11: Bilateral cavitating nodules in a patient with Wegener granulomatosis.
Bilateral cavitating nodules in a patient with We...

Bilateral cavitating nodules in a patient with Wegener granulomatosis.

Wegener granulomatosis. This patient presented wi...Media file 12: Wegener granulomatosis. This patient presented with massive hemoptysis. No nodules are identified on the chest radiograph, although a subsequent CT scan showed several noncavitating nodules.
Wegener granulomatosis. This patient presented wi...

Wegener granulomatosis. This patient presented with massive hemoptysis. No nodules are identified on the chest radiograph, although a subsequent CT scan showed several noncavitating nodules.

Diffuse alveolar hemorrhage in a 21-year-old man ...Media file 13: Diffuse alveolar hemorrhage in a 21-year-old man with Wegener granulomatosis, part 1. Image courtesy of the US Government.
Diffuse alveolar hemorrhage in a 21-year-old man ...

Diffuse alveolar hemorrhage in a 21-year-old man with Wegener granulomatosis, part 1. Image courtesy of the US Government.

Diffuse alveolar hemorrhage in a 21-year-old man ...Media file 14: Diffuse alveolar hemorrhage in a 21-year-old man with Wegener granulomatosis, part 2. Image courtesy of the US Government.
Diffuse alveolar hemorrhage in a 21-year-old man ...

Diffuse alveolar hemorrhage in a 21-year-old man with Wegener granulomatosis, part 2. Image courtesy of the US Government.

More on Wegener Granulomatosis

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

References

  1. 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].

  2. Wegener's Granulomatosis Etanercept Trial (WGET) Research Group. Limited versus severe Wegener's granulomatosis: baseline patient data on patients in the Wegener's granulomatosis etanercept trial. Arthritis Rheum. August 2003;48(8):2299-2309. [Medline].

  3. Kallenberg CGM. Pathogenesis of PR3-ANCA associated vasculitis. J Autoimmun. February-March 2008;30:29-36. [Medline].

  4. Finkielman JD, Lee AS, Hummel AM, et al. ANCA are detectable in nearly all patients with active severe Wegener's Granulomatosis. Am J Med. July 2007;120:643.e9-14. [Medline].

  5. Kallenberg CGM, Heeringa P, Stegeman CA. Mechanisms of disease: pathogenesis and treatment of ANCA-associated vasculitis. Nat Clin Pract Rheumatol. December 2006;2:661-670. [Medline].

  6. Moosig F, Lamprecht P, Gross WL. Wegener's Granulomatosis: the current view. Clin Rev Allergy Immunol. October 2008;35(1-2):19-21. [Medline].

  7. Spagnolo P, Richeldi L, DuBois RM. Environmental triggers and susceptibility factors in idiopathic granulomatous diseases. Semin Respir Crit Care Med. December 2008;29:610-619. [Medline].

  8. Stegeman CA, Tervaert JW, Sluiter WJ, Manson WL, de Jong PE, Kallenberg CG. Association of chronic nasal carriage of Staphylococcus aureus and higher relapse rates in Wegener granulomatosis. Ann Intern Med. January 1994;120(1):12-17. [Medline].

  9. Phillip R, Luqmani R. Mortality in systemic vasculitis: a systematic review. Clin Exp Rheumatol. September-October 2008;26:S94-S104. [Medline].

  10. Watts RA, Lane SE, Koldingsnes W, et al. Epidemiology of vasculitis in Europe. Ann Rheum Dis. December 2001;60(12):1156-7. [Medline].

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

  12. Manganelli P, Fietta P, Carotti M, Pesci A, Salaffi F. Respiratory system involvement in systemic vasculitis. Clin Exp Rheumatol. March-April 2006;24:S48-S59. [Medline].

  13. Hinze CH, Colbert RA. B-cell depletion in Wegener's Granulomatosis. Clin Rev Allergy Immunol. June 2008;34:372-379. [Medline].

  14. Al-Hakeem DA, Fedele S, Carlos R, Porter S. Extranodal NK/T-cell lymphoma, nasal type. Oral Oncol. January 2007;43(1):4-14. [Medline].

  15. Leavitt RY, Fauci AS, Bloch DA, et al. The American College of Rheumatology 1990 criteria for the classification of Wegener's granulomatosis. Arthritis Rheum. August 1990;33(8):1101-1107. [Medline].

  16. Renaudineau Y, Le Meur Y. Renal involvement in Wegener's Granulomatosis. Clinic Rev Allerg Immunol. October 2008;35:22-29. [Medline].

  17. Polychronopoulos VS, Prakash UBS, Golbin JM, Edell ES, Specks U. Airway involvement in Wegener's Granulomatosis. Rheum Dis Clin N Am. November 2007;33:755-775. [Medline].

  18. Mukhtyar C, Guillevin L, Cid MC, et al. EULAR recommendations for the management of primary small and medium vessel vasculitis. Ann Rheum Dis. March 2009;68:310-317. [Medline].

  19. Frankel SK, Cosgrove GP, Fischer A. Update in the diagnosis and management of pulmonary vasculitis. Chest. February 2006;129(2):452-65. [Medline].

  20. DeGroot K, Harper L, Jayne DRW, et al. Pulse versus daily oral cyclophosphamide for induction of remission in antineutrophil cytoplasmic antibody-associated vasculitis: a randomized trial. Ann Intern Med. May 2009;150:670-680. [Medline].

  21. [Best Evidence] DeGroot K, Rasmussen N, Bacon PA, et al. Randomized trial of cyclophosphamide versus methotrexate for induction of remission in early systemic antineutrophil cytoplasmic antibody-associated vasculitis. Arthritis Rheum. August 2005;52:2461-2469. [Medline].

  22. Pusey CD, Rees AJ, Evans DJ, Peters DK, Lockwood CM. Plasma exchange in focal necrotizing glomerulonephritis without anti-GBM antibodies. Kidney Int. October 1991;40(4):757-763. [Medline].

  23. [Best Evidence] Jayne DR, Gaskin G, Rasmussen N, et al. Randomized trial of plasma exchange or high-dosage methylprednisolone as adjunctive therapy for severe renal vasculitis. J Am Soc Nephrol. July 2007;18(7):2180-2188. [Medline].

  24. Klemmer PJ, Chalermskulrat W, Reif MS, Hogan SL, Henke DC, Falk RJ. Plasmapheresis therapy for diffuse alveolar hemorrhage in patients with small-vessel vasculitis. Am J Kidney Dis. December 2003;42(6):1149-1153. [Medline].

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

  26. Sanders JS, Slot MC, Stegeman CA. Maintenance therapy for vasculitis associated with antineutrophil cytoplasmic autoantibodies. N Engl J Med. November 2003;349(21):2072-2073. [Medline].

  27. Slot MC, Tervaert JW, Boomsma MM, Stegeman CA. Positive classic antineutrophil cytoplasmic antibody (C-ANCA) titer at switch to azathioprine therapy associated with relapse in proteinase 3-related vasculitis. Arthritis Rheum. April 2004;51(2):269-273. [Medline].

  28. Pagnoux C, Mahr A, Hamidou MA, et al. Azathioprine or methotrexate maintenance for ANCA-associated vasculitis. N Engl J Med. December 2008;359:2790-2803. [Medline].

  29. Metzler C, Miehle N, Manger K, et al. Elevated relapse rate under oral methotrexate versus leflunomide for maintenance of remission in Wegener's granulomatosis. Rheumatology (Oxford). July 2007;46(7):1087-1091. [Medline].

  30. 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].

  31. Bosch X, Guilabert A, Espinosa G, Mirapeix E. Treatment of antineutrophil cytoplasmic antibody-associated vasculitis: a systematic review. JAMA. August 2007;298:655-669. [Medline].

  32. Martinez V, Cohen P, Pagnoux C, et al. Intravenous immunoglobulins for relapses of systemic vasculitides associated with antineutrophil cytoplasmic autoantibodies: results of a multicenter, prospective, open-label study of twenty-two patients. Arthritis Rheum. January 2008;58(1):308-371. [Medline].

  33. 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. December 2005;20(12):2725-2732. [Medline].

  34. Koukoulaki M, Jayne DR. Mycophenolate mofetil in anti-neutrophil cytoplasm antibodies-associated systemic vasculitis. Nephron Clin Pract. 2006;102(3-4):c100-7. [Medline].

  35. Stone JH, Uhlfelder ML, Hellmann DB, Crook S, Bedocs NM, Hoffman GS. Etanercept combined with conventional treatment in Wegener's granulomatosis: a six-month open-label trial to evaluate safety. Arthritis Rheum. May 2001;44(5):1149-1154. [Medline].

  36. 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].

  37. Booth A, Harper L, Hammad T, Bacon P, Griffith M, Levy J, et al. Prospective study of TNFalpha blockade with infliximab in anti-neutrophil cytoplasmic antibody-associated systemic vasculitis. J Am Soc Nephrol. Mar 2004;15(3):717-21. [Medline].

  38. Lee RW, D'Cruz DP. Novel therapies for anti-neutrophil cytoplasmic antibody-associated vasculitis. Drugs. 2008;68:747-770. [Medline].

  39. Sangle SR, Hughes GR, D'Cruz DP. Infliximab in patients with systemic vasculitis that is difficult to treat: poor outcome and significant adverse effects. Ann Rheum Dis. April 2007;66(4):564-565. [Medline].

  40. Stasi R, Stipa E, Del Poeta G, Amadori S, Newland AC, Provan D. Long-term observation of patients with anti-neutrophil cytoplasmic antibody-associated vasculitis treated with rituximab. Rheumatology (Oxford). November 2006;45(11):1432-1436. [Medline].

  41. 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. January 2006;173(2):180-187. [Medline].

  42. Aries PM, Hellmich B, Voswinkel J, et al. Lack of efficacy of rituximab in Wegener's granulomatosis with refractory granulomatous manifestations. Ann Rheum Dis. July 2006;65(7):853-858. [Medline].

  43. Seo P, Specks U, Keogh KA. Efficacy of rituximab in limited Wegener's granulomatosis with refractory granulomatous manifestations. J Rheumatol. October 2008;35(10):2017-2023. [Medline].

  44. Birck R, Warnatz K, Lorenz HM, et al. 15-Deoxyspergualin in patients with refractory ANCA-associated systemic vasculitis: a six-month open-label trial to evaluate safety and efficacy. J Am Soc Nephrol. February 2003;14(2):440-447. [Medline].

  45. Schmitt WH, Birck R, Heinzel PA, et al. Prolonged treatment of refractory Wegener's granulomatosis with 15-deoxyspergualin: an open study in seven patients. Nephrol Dial Transplant. June 2005;20(6):1083-1092. [Medline].

  46. Schmitt WH, Hagen EC, Neumann I, et al. Treatment of refractory Wegener's granulomatosis with antithymocyte globulin (ATG): an open study in 15 patients. Kidney Int. April 2004;65(4):1440-1448. [Medline].

  47. Walsh M, Chaudhry A, Jayne D. Long-term follow-up of relapsing/refractory anti-neutrophil cytoplasm antibody associated vasculitis treated with the lymphocyte depleting antibody alemtuzumab (CAMPATH-1H). Ann Rheum Dis. September 2008;67(9):1322-1327. [Medline].

  48. Seo P, Min Y-I, Holbrook JT, et al. Damage caused by Wegener's Granulomatosis and its treatment: prospective data from the Wegener's Granulomatosis Etanercept Trial (WGET). Arthritis Rheum. July 2005;52:2168-2178. [Medline].

  49. Mukhtyar C, Flossmann O, Hellmich B, et al. Outcomes from studies of antineutrophil cytoplasm antibody associated vasculitis: a systematic review by the European League Against Rheumatism systemic vasculitis task force. Ann Rheum Dis. July 2008;67:1004-1010. [Medline].

  50. Haroun MK, Stone JH, Nair R, Racusen L, Hellmann DB, Eustace JA. Correlation of percentage of normal glomeruli with renal outcome in Wegener's granulomatosis. Am J Nephrol. September-December 2002;22(5-6):497-503. [Medline].

  51. Hauer HA, Bajema IM, Van Houwelingen HC, et al. Determinants of outcome in ANCA-associated glomerulonephritis: a prospective clinico-histopathological analysis of 96 patients. Kidney Int. November 2002;62(5):1732-1742. [Medline].

  52. Brusselle CG. Pulmonary-renal syndromes. Acta Clin Belg. March-April 2007;62:88-96. [Medline].

  53. Danda D, Mathew AJ, Mathew J. Wegener's granulomatosis -- a rare presentation. Clin Rheumatol. February 2008;27:273-275. [Medline].

  54. Hellmich B, Lamprecht P, Gross WL. Advances in the therapy of Wegener's granulomatosis. Curr Opin Rheumatol. Jan 2006;18(1):25-32. [Medline].

  55. Jayne D. Review article: Progress of treatment in ANCA-associated vasculitis. Nephrology (Carlton). February 2009;14(1):42-48. [Medline].

  56. Lane SE, Watts R, Scott DGI. Epidemiology of systemic vasculitis. Curr Rheumatol Rep. August 2005;7:270-275. [Medline].

  57. Ponniah I, Shaheen A, Shankar KA, Kumaran MG. Wegener's granulomatosis: the current understanding. Oral Surg Oral Med Oral Pathol Oral Radiol Endod. Sep 2005;100(3):265-70. [Medline].

  58. Seo P. Wegener's granulomatosis: managing more than inflammation. Curr Opin Rheum. January 2008;20:10-16. [Medline].

  59. Stone JH, Hoffman GS. Wegener's granulomatosis. In: Hochberg MC, Silman AJ, Smolen JS, Weinblatt ME, Weisman MH, eds. Rheumatology. Vol 2. 4th ed. Philadelphia, PA: Mosby Elsevier; 2008:1533-1544/Chap 146.

  60. Watts RA, Scott DGI. Classification and epidemiology of the vasculitides. Baillieres Clin Rheumatol. May 1997;11(2):191-217. [Medline].

  61. Weeda LW, Coffey SA. Wegener's Granulomatosis. Oral Maxillofac Surg Clin North Am. November 2008;20:643-649. [Medline].

Keywords

Wegener granulomatosis, WG, Wegener's granulomatosis, Wegener's disease, Wegener disease, systemic vasculitis, systemic necrotizing angiitis, necrotizing glomerulonephritis, necrotizing granulomatous inflammation of the respiratory tract, rapidly progressive glomerulonephritis, ANCA-associated vasculitis, ANCA, AAV, PR3-ANCA, alveolar capillaritis, diffuse alveolar hemorrhage, pauci-immune complex, glomerulonephritis

Contributor Information and Disclosures

Author

Patricia J Papadopoulos, MD, Fellow, Department of Rheumatology, Walter Reed Army Medical Center, Washington, DC
Patricia J Papadopoulos, MD is a member of the following medical societies: Alpha Omega Alpha, American College of Physicians-American Society of Internal Medicine, and American College of Rheumatology
Disclosure: Nothing to disclose.

Coauthor(s)

Robert John O'Brian, MD, Staff Rheumatologist, National Naval Medical Center
Robert John O'Brian, MD is a member of the following medical societies: American College of Rheumatology
Disclosure: Nothing to disclose.

Medical Editor

Robert E Wolf, MD, PhD, Professor Emeritus, Department of Medicine, Louisiana State University Health Sciences Center at Shreveport; Chief, Rheumatology Section, Medical Service, Overton Brooks Veterans Administration Medical Center of Shreveport
Robert E Wolf, MD, PhD is a member of the following medical societies: American College of Rheumatology, Arthritis Foundation, and Society for Leukocyte Biology
Disclosure: Nothing to disclose.

Pharmacy Editor

Francisco Talavera, PharmD, PhD, Senior Pharmacy Editor, eMedicine
Disclosure: eMedicine Salary Employment

Managing Editor

Elliot Goldberg, MD, Dean of the Western Pennsylvania Clinical Campus, Professor, Department of Medicine, Temple University School of Medicine
Elliot Goldberg, MD is a member of the following medical societies: Alpha Omega Alpha, American College of Physicians, and American College of Rheumatology
Disclosure: Nothing to disclose.

CME Editor

Alex J Mechaber, MD, FACP, Associate Dean for Undergraduate Medical Education, Associate Professor of Medicine, University of Miami Miller School of Medicine
Alex J Mechaber, MD, FACP is a member of the following medical societies: Alpha Omega Alpha, American College of Physicians-American Society of Internal Medicine, and Society of General Internal Medicine
Disclosure: Nothing to disclose.

Chief Editor

Herbert S Diamond, MD, Professor of Medicine, Temple University School of Medicine; Chairman Emeritus, Department of Internal Medicine, Western Pennsylvania Hospital
Herbert S Diamond, MD is a member of the following medical societies: Alpha Omega Alpha, American College of Physicians, American College of Rheumatology, American Medical Association, and Phi Beta Kappa
Disclosure: medifocus Honoraria Review panel membership; health dialogs Honoraria Consulting; West Penn Allegheny Health System None Board membership

 
 
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