Disclaimer: The information contained within the Grand Rounds Archive is intended for use by doctors and other health care professionals. These documents were prepared by resident physicians for presentation and discussion at a conference held at Baylor College of Medicine in Houston, Texas. No guarantees are made with respect to accuracy or timeliness of this material. This material should not be used as a basis for treatment decisions, and is not a substitute for professional consultation and/or peer-reviewed medical literature.Wegener's Granulomatosis Case Presentation: A 37-year-old male presented to the otolaryngology service with a four-year history of right ear pain and tinnitus. He denied otorrhea, but did have occasional dizziness. He also had a history of recurrent sinus infections. He complained of fever, chills, and night sweats for the past two days. He also stated that his voice had become increasingly hoarse, but he denied any shortness of breath or stridor. He also complained of dysphagia to both solids and liquids.On physical exam, he was found to have a saddle nose deformity, nasal crusting and ulceration, and bilateral serous otitis media. His oral cavity had no lesions. On flexible laryngoscopy, he did not appear to have subglottic stenosis. Cultures were taken from his nasal cavity and revealed Staphylococcus aureus and Pseudomonas aeruginosa. Laboratory testing was significant for an erythrocyte sedimentation rate of 73, a positive cANCA, a negative pANCA, a negative ANA, and a negative HIV test. His urinalysis was negative for hematuria or red cell casts and his BUN and creatinine levels were within normal limits. A chest radiograph was negative for infiltrates or nodules. In addition, an audiogram showed bilateral mixed hearing loss.A nasal biopsy was performed and showed acute and chronic inflammation, but no evidence of vasculitis or granuloma. However, based on his clinical presentation and positive cANCA, the patient was diagnosed with limited Wegener's granulomatosis and started on prednisone and methotrexate. The patient subsequently developed necrotizing scleritis and was evaluated by the ophthalmology service. His methotrexate was discontinued and he was begun on cyclophosphamide at 100 mg once a day. He had no improvement in his scleritis symptoms and developed marginal keratitis. His cyclophosphamide dose was increased to 150 mg once a day. His ophthalmologic manifestations became quiescent and he was begun on a slow taper of his prednisone, as well as a maintenance dose of cyclophosphamide (50 mg per day).Approximately one year after his initial treatment, he developed increasing hoarseness and shortness of breath and was re-evaluated by the otolaryngology service. He was found to have significant subglottic stenosis and required an urgent tracheotomy to establish an adequate airway. His cyclophosphamide dose was increased to 100 mg per day and his prednisone dose increased to 10 mg per day. He was doing well until one year later when he began to have blood tinged tracheal secretions. A CXR and a CT of the chest showed a new 1.5 cm non-calcified nodule in the right upper lobe. Currently, his urinalysis is negative for hematuria or red cell casts and he has no other new organ system manifestations. Bibliography: Ataman M, Sarioglu T, Shahidi H, Gürsel B. Wegener's granulomatosis: case report and review of the literature. Rhinology 1994;32:92-97. Bradkey PJ. Wegener's granulomatosis of the ear. J Laryngol Otology 1983;97:623-626. Burlacoff SG, Wong FSH. Wegener's granulomatosis, the great masquerade: a clinical presentation and literature review. J Otolaryngol 1993;22:94-105. Cotch MF, Hoffman GS, Yerg DE, Kaufman GI, Targonski P, Kaslow RA. The epidemiology of Wegener's granulomatosis. Arthritis Rheumatol 1996;39:87-92. Devaney KO, Ferlito A, Hunter BC, Devaney SL, Rinaldo A. Wegener's granulomatosis of the head and neck. Ann Otol Rhinol Laryngol 1998;107:439-445. Duna GF, Galperin C, Hoffman GS. Wegener's granulomatosis. Rheum Dis Clin North Am 1995;21:949-986. Dwyer J, Janzen VD. Wegener's granulomatosis with otological and nervous system involvement. J Otoloaryngol 1981;10:476-480. Edberg JC, Wainstein E, Wu S, Csernok E, Sneller MC, Hoffman GS, Keystone EC, Gross WL, Kimberly RP. Analysis of FcgRII gene polymorphisms in Wegener's granulomatosis. Exp Clin Immunogenet 1997;14:183-195. Fauci AS, Wolff SM. Wegener's granulomatosis: studies in eighteen patients and a review of the literature. Medicine 1973;52:535-561. Fukase S, Ohta N, Inamura K, Kimura Y, Aoyagi M, Koike Y. Diagnostic specificity of anti-neutrophil cytoplasmic antibodies (ANCA) in otorhinolaryngological diseases. Acta Otolaryngol (Stockh) 1994;S511:204-207. Griffith ME, Gaskin G, Pusey CD. Classification, pathogenesis, and treatment of systemic vasculitis. Ren Fail 1996;18:785-802. Griffith ME, Pusey CD. HLA genes in ANCA-associated vasculitides. Exp Clin Immunogenet 1997;14:196-205. Gross WL. Antineutrophil cytoplasmic autoantibody testing in vasculitides. Rheum Dis Clin North Am 1995;21:987-1011. Guillevin L, Cordier J-F, Lhote F, Cohen P, Jarrousse B, Royer I, Lesavre P, Jacquot C, Bindi P, Bielefeld P, Desson J-F, Detree F, Dubois A, Hachulla E, Hoen B, Jacomy D, Seigneuric C, Lauque D, Stern M, Longy-Boursier M. A prospective, multicenter, randomized trial comparing steroids and pulse cyclophosphamide versus steroids and oral cyclophosphamide in the treatment of generalized Wegener's granulomatosis. Arthritis Rheum 1997;40:2187-2198. Guillevin L, Lhote F. Classification and management of necrotising vasculitides. Drugs 1997;53:805-816. Guyot J-P, Baud C, Montandon P. Wegener's granulomatosis with otological disorders as primary symptoms. ORL J 1990;52:327-334. Hartl DM, Aïdan P, Brugière O, Sterkers O. Wegener's granulomatosis presenting as a recurrence of chronic otitis media. Am J Otolaryngol 1998;19:54-60. Hoffman GS. Treatment of Wegener's granulomatosis: time to change the standard of care? Arthritis Rheum 1997;40:2099-2104. Hoffman GS. Wegener's granulomatosis. In: JH Klippel, PA Dieppe (eds), Rheumatology, 2nd Edition, Volume 2. Philadelphia: Mosley 1998, pp 7.22.1-7.22.10. Hoffman GS. "Wegener's granulomatosis": the path traveled since 1931. Medicine 1994;73:235-329. Hoffman GS, Drucker Y, Cotch MF, Locker GA, Easley K, Kwoh K. Wegener's granulomatosis: patient-reported effects of disease on health, function, and income. Arthritis Rheum 1998;41:2257-2262. Hoffman GS, Kerr GS, Leavitt RY, Hallahan CW, Lebovics RS, Travis WD, Rottem M, Fauci AS. Wegener granulomatosis: an analysis of 158 patients. Ann Intern Med 1992;116:488-498. Hoffman GS, Specks U. Antineutrophil cytoplasmic antibodies. Arthritis Rheum 1998;41:1524-1537. Illum P, Thorling K. Otological manifestations of Wegener's granulomatosis. Laryngoscope 1982;92:801-804. Jaffe IA. Wegener's granulomatosis and ANCA syndromes. Neurol Clin 1997;15:887-891. Kletzker GR, Smith PG, McIntire LD, Leonetti JP. Presentation and management of uncommon lesions of the middle ear. Am J Otology 1995;16:634-642. Kornblut AD, Wolff SM, deFries HO, Fauci AS. Wegener's granulomatosis. In: Symposium on Granulomatous Disorders of the Head and Neck. Otolaryngol Clin North Am 1982;15:673-683. Kornblut AD, Wolff SM, Fauci AS. Ear disease in patients with Wegener's granulomatosis. Laryngoscope 1982;92:713-717. Langford CA, Sneller MC, Hallahan CW, Hoffman GS, Kammerer WA, Talar-Williams C, Fauci AS, Lebovics RS. Clinical features and therapeutic management of subglottic stenosis in patients with Wegener's granulomatosis. Arthritis Rheum 1996;39:1754-1760. Leavitt 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. The American College of Rheumatology 1990 criteria for the classification of Wegener's granulomatosis. Arthritis Rheum 1990;33:1101-1107. Lie JT. Wegener's granulomatosis: histological documentation of common and uncommon manifestations in 216 patients. Vasa 1997;20:261-270. Lynch JP, III, Hoffman GS. Wegener's granulomatosis: controversies and current concepts. Comp Ther 1998;24:421-440. McCaffrey TV, McDonald TJ, Facer GW, DeRemee RA. Otologic manifestations of Wegener's granulomatosis. Otolaryngol Head Neck Surg 1980;88:586-593. McDonald TJ, DeRemee RA. Wegener's granulomatosis. Laryngoscope 1983;93:220-231. Macias JD, Wackym PA, McCabe BF. Early diagnosis of otologic Wegener's granulomatosis using the serologic marker C-ANCA. Ann Otol Rhinol Laryngol 1993;102:337-341. Makura ZGG, Robson AK. Wegener's granulomatosis presenting as a temporal headache. J Laryngol Otology 1996;110:802-804. Mandell BR, Hoffman GS. Differentiating the vasculitides. Rheum Dis Clin North Am 1994;20:409-442. Matt BH. Wegener's granulomatosis, acute laryngotracheal airway obstruction and death in a 17-year old female: case report and review of the literature. Int J Pediatr Otorhinolaryngol 1996;37:163-172. Moussa A E-M, Abou-Elhmd KA. Wegener's granulomatosis presenting as mastoiditis. Ann Otol Rhinol Laryngol 1998;107:560-563. Murty GE. Wegener's granulomatosis: otorhinolaryngological manifestations. Clin Otolaryngol 1990;15:385-393. Pathak I, Bryce G. Wegener's granulomatosis masquerading as mastoiditis and lateral-sinus thrombosis. J Otolaryngol 1997;26:207-209. Rottem M, Fauci A, Hallahan CW, Kerr GS, Lebovics R, Leavitt RY, Hoffman GS. Wegener granulomatosis in children and adolescents: clinical presentation and outcome. J Pediatr 1993;122:26-32. Savage COS, Harper L, Adu D. Primary systemic vasculitis. Lancet 1997;349:553-558. Schultz DR, Tozman EC. Antineutrophil cytoplasmic antibodies: major autoantigens, pathophysiology, and disease associations. Semin Arthritis Rheum 1995;25:143-159. Stegeman CA, Tervaert JWC, de Jong PE, Kallenberg CGM. Trimethoprim-sulfamethoxazole (co-trimoxazole) for the prevention of relapses of Wegener's granulomatosis. New Eng J Med 1996;335:16-20. Vartiainen E, Nuutinen J. Head and neck manifestations of Wegener's granulomatosis. Ear Nose Throat J 1992;71:423-428. Wegener's granulomatosis: changing perceptions of a once-fatal disease. In: Wegener's Granulomatosis Minisymposium. G.S. Hoffman (ed). Clevel Clin J Med 1997;61:412-427. Yumoto E, Saeki K, Kadota Y. Subglottic stenosis in Wegener's granulomatosis limited to the head and neck region. Ear Nose Throat J 1997;76:571-574. Click here to view slides from this presentation (pdf fie). 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