Wegener's granulomatosis: A rare, chronic and multisystem disease

Surgeon

Volumn 4, Issue 1, 2006, Pages 45-52

  Khan, Anwar Moin ^a,b   Elahi, F ^a   Hashmi, S R ^c   Mahida, K H ^d   Ingrams, D R ^a  

^a ROYAL GWENT HOSPITAL   (United Kingdom)

^b University Hospital of Wales ^*  (United Kingdom)

^c UNIVERSITY HOSPITAL OF WALES   (United Kingdom)

^d ZIAUDDIN MEDICAL UNIVERSITY   (Pakistan)

Author keywords

Antineutrophil Cytoplasmic antibody; Autoimmune; Multisystem; Wegener's granulomatosis

Indexed keywords

AZATHIOPRINE; CORTICOSTEROID; CYCLOPHOSPHAMIDE; GLUCOCORTICOID; IMMUNOSUPPRESSIVE AGENT; METHYLPREDNISOLONE; NEUTROPHIL CYTOPLASMIC ANTIBODY; STEROID; SULFAMETHOXAZOLE; TRIMETHOPRIM;

CENTRAL NERVOUS SYSTEM DISEASE; CHRONIC DISEASE; CLINICAL FEATURE; COMPUTER ASSISTED TOMOGRAPHY; CONNECTIVE TISSUE DISEASE; DIFFERENTIAL DIAGNOSIS; DOSE RESPONSE; DRUG MEGADOSE; DRUG RESPONSE; DRUG SUBSTITUTION; EAR DISEASE; EARLY DIAGNOSIS; ERYTHROCYTE SEDIMENTATION RATE; EYE DISEASE; HEAD AND NECK DISEASE; HEMORRHAGIC CYSTITIS; HISTOPATHOLOGY; HUMAN; IMMUNOSUPPRESSIVE TREATMENT; INFECTION; LARYNX DISORDER; LOW DRUG DOSE; LUNG DISEASE; MALE STERILITY; MORBIDITY; MORTALITY; MOUTH DISEASE; NASAL BIOPSY; NEOPLASM; NOSE DISEASE; OROPHARYNX; PARANASAL SINUS; PATHOPHYSIOLOGY; PROGNOSIS; RARE DISEASE; RECURRENCE RISK; REVIEW; RISK ASSESSMENT; RISK REDUCTION; SKIN DISEASE; STATISTICAL SIGNIFICANCE; SURVIVAL RATE; SURVIVAL TIME; THORAX RADIOGRAPHY; TREATMENT OUTCOME; TREATMENT RESPONSE; URINALYSIS; WEGENER GRANULOMATOSIS;

EID: 32044436089     PISSN: 1479666X     EISSN: None     Source Type: Journal    
DOI: 10.1016/S1479-666X(06)80021-1     Document Type: Review

References (78)

1. Klinger H. Grenzformen der periarteritis nodosa. Z Pathol 1931;42:455-80.
2. Wegener F. Uber eine eigenartige rhinogene granulomatose mit besonderer beteiligung des arteriensysytems und der nieveu. Beitr Pathol. 1939;102:36-68.
3. Yi ES, Colby TV. Wegener's granulomatosis. Semin Diagn Pathol 2001;18:34-46.
4. Harari S, Paciocco G, Aramu S. Ear and nose involvement in systemic diseases. Monaldi Arch Chest Dis 2000;55:466-70.
5. Fauci AS, Haynes FB, Katz P, Wolff SM. Wegener's granulomatosis : prospective clinical and therapeutic experience with 85 patients for 21 years. Ann Intern Med 1983;98:76-85.
6. Karmody CS, Boston M. Wegener's granulomatosis: presentation as an otologic problem. Otolaryngology 1978;86:573-84.
7. Savage CO, Harper L, Cockwell P, Adu D, Howie AJ. ABC of arterial and vascular disease: vasculitis. Bmj 2000;320: 1325-28.
8. Jennings CR, Jones NS, Dugar J, Powell RJ, Lowe J. Wegener's granulomatosis-a review of diagnosis and treatment in 53 subjects. Rhinology 1998;36:188-91.
9. Hoffman GS, Kerr GS, Leavitt RY, Hallahan CW, Lebovics RS, Travis WD et al. Wegener granulornatosis: an analysis of 158 patients. Ann Intern Med 1992;116:488-98.
10. Stegmayr BG, Gothefors L, Malmer B, Muller Wiefel DE, Nilsson K, Sundelin B. Wegener granulomatosis in children and young adults. A case study of ten patients. Pediatr Nephrol 2000;14:208-13.
11. Luqmani RA, Bacon PA, Beaman M, Scott DG, Emery P, Lee SJ et al. Classical versus non-renal Wegener's granulomatosis. Q J Med 1994;87:161-67.
12. Gaskin G, Pusey CD. Systemic vasculitis. Oxford Textbook of Clinical Nephrology. Oxford: Oxford University Press, 1996.
13. Cotch MF, Hoffman GS, Yerg DE, Kaufman GI, Targonski P, Kaslow RA. The epidemiology of Wegener's granulornatosis. Estimates of the five-year period prevalence, annual mortality, and geographic disease distribution from population-based data sources. Arthritis Rheum 1996;39:87-92.
14. Katzman GL, Langford CA, Sneller MC, Koby M, Patronas NJ. Pituitary involvement by Wegener's granulornatosis: a report of two cases. AJNR Am J Neuroradiol 1999; 20(3): 519-23.
15. Scott DGI, Baon PA, Elliot PJ, Trobe CR, Wallington TB. Systemic Vasculitis in a district general hospital 1972-80. Clinical and laboratory features, classification and prognosis of 80 cases. Q J Med 1982;203:292-311.
16. Carruthers DM, Watts RA, Symmons DP, Scott DG. Wegener's granulomatosis--increased incidence or increased recognition? Br J Rheumatol 1996;35:142-45.
17. Takwoingi YM, Dempster JH. Wegener's granulomatosis: an analysis of 33 patients seen over a 10-year period. Clin Otolaryngol Allied Sci 2003;28:187-94.
18. Kallenberg CGM, Brouwer E, Weening JJ, Tervaert JW. Antineutrophil cytoplasmic antibodies: current diagnostic and pathophysiologic potential. Kidney Int. 1994;46:1-15.
19. George J, Levy Y, Kallenberg CG, Shoenfeld Y. Infections and Wegener's granulomatosis--a cause and effect relationship? Qjm 1997;90:367-73.
20. Davies DJ, Moran JE, Niall JF, Ryan GB. Segmental necrotising glomerulonephritis with antineutrophil antibody: possible arbovirus aetiology? BMJ 1982;285:606.
21. van der Woude FJ, Rasmussen N, Lobatto S, Wiik A, Permin H, van Es LA et al. Autoantibodies against neutrophils and monocytes: tool for diagnosis and marker of disease activity in Wegener's granulomatosis. Lancet 1985;1:425-29.
22. Kerr GS, Fleisher TA, Hallahan CW, Leavitt RY, Fauci AS, Hoffman GS. Limited prognostic value of changes in antineutrophil cytoplasmic antibody titer in patients with Wegener's granulomatosis. Arthritis Rheum 1993;36:365-71.
23. Jenne DE, Tschopp J LJ, Ludemann J, Utecht B, Gross WL. Wegener's autoantigen decoded. Nature 1990;346:520.
24. Jennette JC, Hoidal JR, Falk RJ. Specificity of anti-neutrophil cytoplasmic autoantibodies for proteinase 3. Blood 1990;75: 2263-64.
25. Niles JL, McCluskey RT, Ahmad MF, Amaout MA. Wegener's granulomatosis autoantigen is a novel neutrophil serine proteinase. Blood 1989;74:1888-93.
26. Falk RJ, Jennette JC. Anti-neutrophil cytoplasmic autoantibodies with specificity for myeloperoxidase in patients with systemic vasculitis and idiopathic necrotizing and crescentic glomerulonephritis. N Engl J Med 1988;318: 1651-57.
27. Savige J, Gillis D, Benson E, Davies D, Esnault V, Falk RJ et al. International Consensus Statement on Testing and Reporting of Antineutrophil Cytoplasmic Antibodies (ANCA). Am J Clin Pathol 1999;111:507-13.
28. Hagen EC. Development of solid-phase assays for the detection of anti-neutrophil cytoplasmic antibodies (ANCA) for clinical application: report of a large clinical evaluation study. Clin Exp Immunol 1995;101 (Suppl 1):29.
29. Adu D, Savage COS, Lockwood C et al. ANCA positive and ANCA negative microscopic polyarteritis. Clin Exp Immunol 1995;101 (Suppl 1):62.
30. Bindi P, Mougenot B, Mentre F, Noel LH, Peraldi MN, Vanhille P et al. Necrotizing crescentic glomerulonephritis without significant immune deposits: a clinical and serological study. Q J Med 1993;86:55-68.
31. Ludemann J, Utecht B, Gross WL. Detection and quantitation of anti-neutrophil cytoplasm antibodies in Wegener's granulornatosis by ELISA using affinity-purified antigen. J Immunol Methods 1988;114:167-74.
32. Tervaert JW, Van Der Woude FJ, Fauci AS, Ambrus JL, Velosa J, Keane WF et al. Association between active Wegener's Granulornatosis and anticytoplasmic antibodies. Arch Intern Med 1989;149:2461-65.
33. Klaassen RJL, Goldshmeding R, Dolman KM, Vlekke AB, Weigel HM, Eeftinck Schattenkerk JK et el. Anti neutrophil cytoplasmic autoantibodies in patients with symptomatic HIV infection, Clin Exp Immunol 1992;87:24-30.
34. Mege JL, Escallier JC, Bongrand P, et al. Antineutrophils cytoplasmic antibodies and infection. London: Plenum, 1992: 42.
35. Cheesman AD, Jani P. Cyst, granulomas and tumors of the jaws, nose and sinuses. In: Hibbert J, ed. Scott-Brown's Otolaryngology. Oxford: Butterworth-Heinemann, 1997: 23/21-23/40.
36. DeRemee RA. Extrapulmonary manifestations of Wegener's granulornatosis and other respiratory vasculitides. Semin Respir Med. 1988;9:403-08.
37. Specks U, DeRemee RA. Granulomatous, vasculitis. Wegener's granulomatosis and Churg-Strauss syndrome. Rheum Dis Clin North Am 1990;16:377-97.
38. Krafcik SS, Covin RB, Lynch JP 3rd , Sitrin RG. Wegener's granulornatosis in the elderly. Chest 1996;109:430-37.
39. Travis WD, Hoffman GS, Leavitt RY, Pass HI, Fauci AS. Surgical pathology of the lung in Wegener's granulomatosis. Review of 87 open lung biopsies from 67 patients. Am J Surg Pathol 1991;15:315-33.
40. Fauci AS, Wolff SM. Wegener's granulornatosis studies in 18 patients and a review of the literature. Medicine 1973;52: 535-61.
41. Bibas A, Fahy C, Sneddon L, Bowdler D. Facial paralysis in Wegener's granulornatosis of the middle ear. J Laryngol Otol 2001;115:304-06.
42. Takagi D, Nakamaru Y, Maguchi S, Furuta Y, Fukuda S. Otologic manifestations of Wegener's granulornatosis. Laryngoscope 2002;112:1684-90.
43. Handlers JP, Waterman J, Abraham AM, Melrose RJ. Oral features of Wegener's granulomatosis. Arch Otolaryngol 1985;111(4):267-70.
44. O'Devaney K, Ferlito A, Hunter BC, Devaney SL, Rinaldo A. Wegener's granulornatosis of the head and neck. Ann Otol Rhinol Laryngol 1998;107:439-45.
45. Wolff SM, Faucis AS, Horn RG, Dale DC. Wegener's granulomatosis. Ann Intern Med 1974;81:513-25.
46. Straatsma BR. Ocular manifestations of Wegener's granulomatosis. Am J Ophthalmol 1957;44:789-99.
47. Haynes BF, Fishman ML, Fauci AS, Wolff SM, The ocular manifestations of Wegener's granulomatosis. Fifteen years experience and review of the literature. Am J Med 1977;63: 131-41.
48. Langford CA, Sneller MC, Hallahan CW, Hoffman GS, Kammerer WA, Talar-Williams C et al. Clinical features and therapeutic management of subglottic stenosis in patients with Wegener's granulomatosis. Arthritis Rheum 1996;39: 1754-60.
49. Gulati SP, Sachdeva OP, Sachdeva A, Singh U. Wegener's granulornatosis: a case with laryngeal involvement. Indian J Chest Dis Allied Sci 1997;39(2):125-28.
50. Yumoto E, Saeki K, Kadota Y. Subglottic stenosis in Wegener's granulornatosis limited to the head and neck region. Ear Nose Throat J 1997;76(8):571-74.
51. Lebovics RS, Hoffman GS, Leavitt RY, Kerr GS, Travis WD, Kammerer W et al. The management of subglottic stenosis in patients with Wegener's granulomatosis. Laryngoscope 1992;102:1341-45.
52. Jaffe IA. Wegener's granulornatosis and ANCA syndromes. Neurol Clin 1997;15:887-91.
53. Nishino H, Rubino FA, DeRemee RA, Swanson JW, Parisi JE. Neurological involvement in Wegener's granulomatosis. Ann Neurol 1993;33:04-09.
54. Jacob SE, Martin LK, Kerdel FA. Cutaneous Wegener's granulornatosis (malignant pyoderma) in a patient with Crohn's disease. Int J Dermatol 2003;42:896-98.
55. Stein SL, Miller LC, Konnikov N. Wegener's granulomatosis: case report and literature review. Pediatr Dermatol 1998;15: 352-56.
56. Daum TE, Specks U, Colby TV, Edell ES, Brutinel MW, Prakash UB et al. Tracheobronchial involvement in Wegener's granulomatosis. Am J Respir Crit Care Med 1995;151:522-26.
57. Bajema IM, Hagen EC, Vander Woude FJ, Bruijn JA. Wegener's granulomatosis: a meta-analysis of 349 case reports. J Lab Clin Med 1997;129:17-22.
58. Pinching AJ, Lockwood CM, Pussell BA, Rees AJ, Swerry P, Evans DJ et al. Wegener's granulomatosis: observations on 18 patients with severe renal disease. Q J Med 1983;52:435-60.
59. Lloyd G, Lund VJ, Beale T, Howard D. Rhinologic changes in Wegener's granulomatosis. J Laryngol Otol 2002;116(7): 565-69.
60. Langford CA & Hoffman GS. Wegener's granulomatosis. Thorax 1999;54:629-37.
61. Esper GJ, Johnson JS. Update on the treatment of Wegener's granulornatosis. Bull Rheum Dis 1999;48(11):01-04.
62. Jayne D. Update on the European Vasculitis Study Group trials. Curr Opin Rheumatol 2001;13:48-55.
63. Matic G, Michelsen A, Hofmann D, Winkler R, Tiess M, Schneidewind JM et al. Three cases of C-ANCA-positive vasculitis treated with immunoadsotption: possible benefit in early treatment. Ther Apher 2001;5:68-72.
64. Palmer A, Cairns T, Dische F, Gluck G, Gjorstrup P, Parsons V et al. Treatment of rapidly progressive glomerulonephritis by extracorporeal immunoadsorption, predinisolone and cyclophosphamide. Nephrol Dial Transplant 1991;6:536-42.
65. Jordan SC, Toyoda M. Treatment of autoimmune diseases and systemic vasculitis with pooled human intravenous immune globulin. Clin Exp Immunol 1994;1:31-38.
66. Jayne DR, Chapel H, Adu D, Misbath S, O'Donoghue D, Scott D et al. Intravenous immunoglobulin for ANCA-associated systemic vasculitis with persistent disease activity. Qjm 2000;93:433-39.
67. Lockwood CM, Thiru S, Stewart S, Hale G, Isaacs J, Wraight P et al. Treatment of refractory Wegener's granulornatosis with humanized monoclonal antibodies. Qjm 1996;89:903-12.
68. Levy JB, Winearls CG. Rapidly progressive glomerulonephritis: what should be first-line therapy? Nephron 1994;67:402-07.
69. Pusey CD, Rees AJ, Evans DJ, Peters DK, Lockwood CM. Plasma exchange in focal necrotizing glomerulonephritis without anti-GBM antibodies. Kidney Int 1991;40:757-63,
70. DeRemee RA, McDonald TJ, Weiland LH. Wegener's granulomatosis: observations on treatment with antimicrobial agents. Mayo Clin Proc 1985;60:27-32.
71. Israel HL. Sulfamethoxazole-trimethoprim therapy for Wegener's granulomatosis. Arch Intern Med 1988;148:2293-95.
72. West BC ' Todd JR, King JW. Wegener granulomatosis and trimethoprim-sulfamethoxazole. Complete remission after a twenty-year course. Ann Intern Med 1987;106:840-42.
73. Axelson JA, Clark RH, Ancerewicz S. Wegener's granulomatosis and trimethoprime-sulfamethoxazole. Ann Intern Med 1987;17:600.
74. Yuasa K, Tokitsu M, Goto H, Kato H, Shimada K. Wegener's granulomatosis: diagnosis by transbronchial lung biopsy, evaluation by gallium scintigraphy and treatment with trimethoprim/sulfamethoxasole. Am J Med 1988:371-72.
75. Valeriano-Marcet J, Spiera H. Treatment of Wegener's granulomatosis with Sulfamethoxasole-Trimethoprim. Arch Intern Med 1991;151:1649-52.
76. 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 1996;335:16-20.
77. Fauci AS, Wolff SM, Johnson JS. Effect of cyclophosphamide upon the immune response in Wegener's granulomatosis. N Engl J Med 1971;285:1493-96.
78. Jayne DR, Lockwood CM. Intravenous immunoglobulin as sole therapy for systemic vasculitis. Br J Rheumatol 1996;35: 1150-53.