Abstract
Granulomatosis with polyangiitis or Wegener’s granulomatosis is a small-vessel vasculitis characterized by necrotizing granulomatous inflammation with involvement of: (1) Upper airways (sinusitis, otitis, ulcerations, bone deformities, subglotic or bronchial stenosis), (2) Lower respiratory tract (cough, chest pain, dyspnea, and hemoptysis), (3) Glomerulonephritis (hematuria, red blood cell casts, proteinuria, and azotemia). When Granulomatosis with polyangiitis (Wegener’s) is suspected, the search to locate involvement of the upper respiratory tract, lungs and airways, CNS, or kidneys should include: (a) A clinical ORL exam and a sinonasal CT or MRI, (b) A high resolution chest CT, (c) An electrocardiogram (ECG), (d) Research of proteinuria and urinary sediment, (e) An ophthalmological exam, (f) Titers of c-ANCA and PR3 (antiprotease 3).
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References
Aberle DR, Gamsu G, Ray CS et al (1988) Asbestos-related pleural and parenchymal fibrosis: detection with high-resolution CT. Radiology 166(3):729–734
Aberle DR, Gamsu G, Lynch D (1990) Thoracic manifestations of Wegener granulomatosis: diagnosis and course. Radiology 174(3 Pt 1):703–709
Allen SD, Harvey CJ (2007) Imaging of Wegener’s granulomatosis. Br J Radiol 80(957):757–765
Ananthakrishnan L, Sharma N, Kanne JP (2009) Wegener’s granulomatosis in the chest: high-resolution CT findings. AJR Am J Roentgenol 192(3):676–682
Anderson JM, Jameson DG, Jefferson JM (1975) Non-healing granuloma and the nervous system. Q J Med 44:309–323
Asmus R, Muhle C, Koltze H et al (1992) The magnetic resonance tomographic signs of Wegener’s granulomatosis in the head area. Rofo 157(1):11–14
Atlas SW, Bilaniuk LT, Zimmerman RA et al (1987a) Orbit: initial experience with surface coil spin-echo MR imaging at 1.5 T. Radiology 164(2):501–509
Atlas SW, Grossman RI, Savino PJ et al (1987b) Surface-coil MR of orbital pseudotumor. AJR Am J Roentgenol 148(4):803–808
Attali P, Begum R, Ban Romdhane H et al (1998) Pulmonary Wegener’s granulomatosis: changes at follow-up CT. Eur Radiol 8(6):1009–1113
Banerjee A, Armas JM, Dempster JH (2001) Wegener’s granulomatosis: diagnostic dilemma. J Laryngol Otol 115(1):46–47
Benoudiba F, Marsot-Dupuch K, Rabia MH et al (2003) Sinonasal Wegener’s granulomatosis: CT characteristics. Neuroradiology 45(2):95–99
Bergin CJ, Wirth RL, Berry GJ et al (1990) Pneumocystis carinii pneumonia: CT and HRCT observations. J Comput Assist Tomogr 14(5):756–759
Bicknell SG, Mason AC (2000) Wegener’s granulomatosis presenting as cryptogenic fibrosing alveolitis on CT. Clin Radiol 55(11):890–891
Bosch X, Guilabert A, Espinosa G et al (2007) Treatment of antineutrophil cytoplasmic antibody associated vasculitis: a systemic review. JAMA 298(6):655–669
Brinar VV, Cikes N, Petelin Z et al (2004) Cerebral demyelination in Wegner’s granulomatosis. Clin Neural Neurosurg 1.6:233–236
Brown KK (2006) Pulmonary vasculitis. Proc Am Thorac Soc 3(1):48–57
Bullen CL, Liesegang TJ, Mcdonald TJ et al (1983) Ocular complications of Wegener’s granulomatosis. Ophthalmology 90(3):279–290
Cabral DA, Urive AG, Benseler S et al (2009) Classification, presentation and initial treatment of Wegner’s granulomatosis in childhood. Arthritis Rheum 60(11):3413–3424
Casian A, Jayne D (2011) Plasma exchange in the treatment of Wegener’s granulomatosis, microscopic polyangiitis, Churg–Strauss syndrome and renal limited vasculitis. Curr Opin Rheumatol 23:12–17
Castaner E, Alguersuari A, Gallourdo W et al (2010) When to suspect pulomonary vasculitis: radiologic and clinical clues. Radiographics 30:33–53
Chung MP, Chin Ay, Lee HY et al (2010) Imaging of pulmonary vasculitis. Radiology 255(2):322–341
Cordier JF, Valeyre D, Guillevin L et al (1990) Pulmonary Wegener’s granulomatosis. A clinical and imaging study of 77 cases. Chest 97(4):906–912
Courcoutsakis NA, Langford CA, Sneller MC et al (1997) Orbital involvement in Wegener granulomatosis: MR findings in 12 patients. J Comput Assist Tomogr 21(3):452–458
Daum TE, Specks U, Colby TV et al (1995) Tracheobronchial involvement in Wegener’s granulomatosis. Am J Respir Crit Care Med 151(2 Pt 1):522–526
De Groot K, Schmidt DK, Arlt et al (2001) Standarized neurologic evaluations of 128 patients with Wegener’s granulomatosis. Klin Monbl Augenheilkd 201(5): 309–16
Drachman DA (2001) Neurological complications of Wegener’s granulomatosis. Arch Neurol 58:1215–1221
Duncker G, Gross WL, Nolle B et al (1992) Orbital involvement in Wegener’s granulomatosis. Klin Monbl Augenheilkd 201(5):309–316
Epstein DM, Gefter WB, Miller WT et al (1980) Spontaneous pneumothorax: an uncommon manifestation of Wegener granulomatosis. Radiology 135(2):327–328
Falk RJ, Gross WL, Guillevin L et al (2011a) Granulomatosis with polyangiitis (Wegner’s): an alternative name of Wegener’s granulomatosis. Arthritis Rheum 63(4):863–864
Falk RJ, Gross WL, Guillevin L et al (2011b) Granulomatosis with polyangiitis (Wegner’s): an alternative name of Wegener’s granulomatosis. J Am Soc Nephrol (in press)
Falk RJ, Gross WL, Guillevin L et al (2011c) Granulomatosis with polyangiitis (Wegner’s): an alternative name of Wegener’s granulomatosis. Ann Rheum Dis 70(4):704
Fauci AS, Wolff SM (1973) Wegener’s granulomatosis: studies in eighteen patients and a review of the literature. Medicine 52(6):535–561
Fauci AS, Haynes BF, Katz P et al (1983) Wegener’s granulomatosis: prospective clinical and therapeutic experience with 85 patients for 21 years. Ann Intern Med 98(1):76–85
Flye MW, Mundinger GH Jr, Fauci AS (1979) Diagnostic and therapeutic aspects of the surgical approach to Wegener’s granulomatosis. J Thorac Cardiovasc Surg 77(3):331–337
Foo SS, Weisbrod GL, Herman SJ et al (1990) Wegener granulomatosis presenting on CT with atypical bronchovasocentric distribution. J Comput Assist Tomogr 14(6):1004–1006
Frankel SK, Cosgrove GP, Fisher A et al (2006) Update in the diagnosis and management of pulmonary vasculitis. Chest 129:452–465
Franks TJ, Koss MN (2000) Pulmonary capillaritis. Curr Opin Pulm Med 6(5):430–435
Frazier AA, Rosado-De-Christenson ML, Galvin JR et al (1998) Pulmonary angiitis and granulomatosis: radiologic-pathologic correlation. Radiographics 18(3):687–710, quiz 727
Frith P (2010) The eye in general medicine. In: Warrell DA, Cox TM, Firth JD (eds) Oxford book of medicine, vol 3, 5th edn. Oxford University Press, New York, pp 5242–5243
Fujisawa I (2004) Magnetic resonance imaging of the hypothalamic-neurohypophyseal system. J Neuroendocrinol 16(4):297–302
Guillevin L, Pagnoux (2007) Tratement of ANCA-associated vascularitidis. Presse Med 36(5 Pt 2):922–927
Hoffman GS, Kerr GS, Leavitt RY et al (1992) Wegener granulomatosis: an analysis of 158 patients. Ann Intern Med 116(6):488–498
Holle JU, Gross WL (2011) Neurological involvement in Wegener’s granulomatosis. Curr Opin Rheumatol 23:7–11
Jayne D (2010) The kidney in systemic vasculitis. In: Warrell DA, Cox TM, Firth JD (eds) Oxford book of medicine, vol 3, 5th edn. Oxford University Press, New York, pp 4032–4040
Jayne DR, Chapel H, Aolu D et al (2000) Intravenous immunoglobulin for ANCA-associated systemic vasculitis with persistent disease activity. QJM 93:433–439
Jennette JC, Falk RJ (1997) Small-vessel vasculitis. N Engl J Med 337(21):1512–1523
Jennette JC, Falk RJ, Milling DM (1994) Pathogenesis of vasculitis. Semin Neurol 14(4):291–299
Jones RB, Tensert JW, Hanser T (2010) Rituximab versus cyclophosphamide in ANCA-associated vasculitis. N Engl J Med 363(3):211–220
Katzenstein AL, Locke WK (1995) Solitary lung lesions in Wegener’s granulomatosis. Pathologic findings and clinical significance in 25 cases. Am J Surg Pathol 19(5):545–552
Kim Y, Lee KS, Jung KJ et al (1999) Halo sign on high resolution CT: findings in spectrum of pulmonary diseases with pathologic correlation. J Comput Assist Tomogr 23(4):622–626
Komocsi A, Reuter M, Heller M et al (2003) Active disease and residual damage in treated Wegener’s granulomatosis: an observational study using pulmonary high-resolution computed tomography. Eur Radiol 13(1):36–42
Kuhlman JE, Hruban RH, Fishman EK (1991) Wegener granulomatosis: CT features of parenchymal lung disease. J Comput Assist Tomogr 15(6):948–952
Lamprecht P, Gross WL (2004) Wegener’s granulomatosis. Herz 29:47–56
Langford CA, Sneller MC, Hallahan CW et al (1996) Clinical features and therapeutic management of subglottic stenosis in patients with Wegener’s granulomatosis. Arthritis Rheum 39(10):1754–1760
Leavitt RY, Fauci AS (1986) Pulmonary vasculitis. Am Rev Respir Dis 134(1):149–166
Lee KS, Kim TS, Fujimoto K et al (2003) Thoracic manifestation of Wegener’s granulomatosis: CT findings in 30 patients. Eur Radiol 13(1):43–51
Lohrmann C, Uhl M, Warnatz K et al (2006) Sinonasal computed tomography in patients with Wegener’s granulomatosis. J Comput Assist Tomogr 30(1):122–125
Lugmani RA, Bacon PA, Beamon M et al (1994) Classical versus non-renal Wegener’s Granulomatosis. Q J Med 87(3):161–167
Maguire R, Fauci AS, Doppman JL et al (1978) Unusual radiographic features of Wegener’s granulomatosis. AJR Am J Roentgenol 130(2):233–238
Martinez de Pero M, Chaudray A, Jones RB et al (2009) B-cell depletion with rituximab for refractory head and neck Wegener’s granulomatosis a cohort study. Clin Otolayngol 34:328–335
Maskell GF, Lockwood CM, Flower CD (1993) Computed tomography of the lung in Wegener’s granulomatosis. Clin Radiol 48(6):377–380
Mentzel HJ, Neumann T, Fitzek C et al (2003) MR Imaging in Wegener granulomatosis of the spinal cord. AJNR Am J Neuroradiol 24(1):18–21
Muhle C, Reinhold-Keller E, Richter C et al (1997) MRI of the nasal cavity, the paranasal sinuses and orbits in Wegener’s granulomatosis. Eur Radiol 7(4):566–570
Murphy JM, Gomez-Anson B, Gillard JH et al (1999) Wegener granulomatosis: MR imaging findings in brain and meninges. Radiology 213(3):794–799
Neale G (2010) Vascular and collagen disorders. In: Warrell DA, Cox TM, Firth JD (eds) Oxford textbook of medicine, vol 2, 5th edn. Oxford University Press New York, pp 2421–2424
Newman NJ, Slamovits TL, Friedland S et al (1995) Neuro-ophtalmic manifestations of meningocerebral inflammation from the limited forms of Wegener’s granulomatosis. Am J Ophtalmol 120:613–621
Nishino H, Rubino FA, Deremee RA et al (1993) Neurological involvement in Wegener’s granulomatosis: an analysis of 324 consecutive patients at the Mayo Clinic. Ann Neurol 33(1):4–9
Novack R, Wachtler P, Kunz J et al (2009) Cranial nerve palsy in Wegener’s granulomatosis: lesions for clinical cases. J Neurol 256:119–304
Pagnoux C, Guillevin L (2005) Peripheral neuropathy in systemic vasculitides. Curr Opin Rheumatol 58:1215–1221
Pamir MN, Zirh TA, Ozek MM et al (1993) Magnetic resonance imaging in the diagnosis of idiopathic giant-cell granulomatous hypophysitis: a rare cause of hyperprolactinaemia. Neurochirurgia 36(1):20–25
Papiris SA, Manoussakis MN, Drosos AA et al (1992) Imaging of thoracic Wegener’s granulomatosis: the computed tomographic appearance. Am J Med 93(5):529–536
Patchefsky AS, Banner M, Freundlich IM (1971) Desquamative interstitial pneumonia. Significance of intranuclear viral-like inclusion bodies. Ann Intern Med 74(3):322–327
Pivonello R, De Bellis A, Faggiano A et al (2003) Central diabetes insipidus and autoimmunity: relationship between the occurrence of antibodies to arginine vasopressin-secreting cells and clinical, immunological, and radiological features in a large cohort of patients with central diabetes insipidus of known and unknown etiology. J Clin Endocrinol Metab 88(4):1629–1636
Polychronopoulos VS, Prakash UB, Golbin JM et al (2007) Airway involvement in Wegener’s granulomatosis. Rheum Dis Clin North Am 33(4):755–775
Primack SL, Hartman TE, Lee KS et al (1994) Pulmonary nodules and the CT halo sign. Radiology 190(2):513–515
Prince JS, Duhamel DR, Levin DL et al (2002) Nonneoplastic lesions of the tracheobronchial wall: radiologic findings with bronchoscopic correlation. Radiographics 22 Spec No(S215–S230)
Provenzale JM, Mukherji S, Allen NB et al (1996) Orbital involvement by Wegener’s granulomatosis: imaging findings. AJR Am J Roentgenol 166(4):929–934
Ranjan A, Chandy MJ (1994) Intrasellar tuberculoma. Br J Neurosurg 8(2):179–185
Reuter M, Schnabel A, Wesner F et al (1998) Pulmonary Wegener’s granulomatosis: correlation between high-resolution CT findings and clinical scoring of disease activity. Chest 114(2):500–506
Schitani A, Mukobayashi C, Oohata H et al (1997) Wegener’s granulomatosis with dural involvement as the initial clinical manifestation. Intern Med 36:514–516
Seror R, Mahr A, Ramanoelina J et al (2006) Central nervous system involvement in Wegener granulomatosis. Medicine 85(1):54–65
Sheehan RE, Flint JD, Muller NL (2003) Computed tomography features of the thoracic manifestations of Wegener granulomatosis. J Thorac Imaging 18(1):34–41
Sugimoto T, Deji N, Kume S et al (2007) Pulmonary–renal syndrome, diffuse pulmonary hemorrhage and glomerulonephritis, associated with Wegener’s granulomatosis effectively treated with early plasma exchange therapy. Intern Med 46(1):49–53
Summers RM, Aggarwal NR, Sneller MC et al (2002) CT virtual bronchoscopy of the central airways in patients with Wegener’s granulomatosis. Chest 121(1):242–250
Talerman A, Wright D (1972) Laryngeal obstruction due to Wegener’s granulomatosis. Arch Otolaryngol 96(4):376–379
Taylor SR, Salama AD, Joshi L et al (2009) Rituximab is effective in the treatment of refractory Wegener’s granulomatosis. Arthritis Rheum 60:1540–1547
Thomas K (1970) Laryngeal manifestations of Wegener’s granuloma. J Laryngol Otol 84(1):101–106
Wadsworth DT, Siegel MJ, Day DL (1994) Wegener’s granulomatosis in children: chest radiographic manifestations. AJR Am J Roentgenol 163(4):901–904
Webb WR, Stein MG, Finkbeiner WE et al (1988) Normal and diseased isolated lungs: high-resolution CT. Radiology 166(1 Pt 1):81–87
Weir IH, Muller NL, Chiles C et al (1992) Wegener’s granulomatosis: findings from computed tomography of the chest in 10 patients. Can Assoc Radiol J 43(1):31–34
Wells AU, du Bois RM (2010) The lung in vasculitis. In: Warrell DA, Cox TM,Firth JD (eds) Oxford book of medicine, vol 2, 5th edition. Oxford University Press, New York, pp 3395–3402
Wolff SM, Fauci AS, Horn RG et al (1974) Wegener’s granulomatosis. Ann Intern Med 81(4):513–525
Wolk RB (1984) Sarcoidosis of the orbit with bone destruction. AJNR Am J Neuroradiol 5(2):204–205
Yang C, Talbot JM, Hwang PH (2001) Bony abnormalities of the paranasal sinuses in patients with Wegener’s granulomatosis. Am J Rhinol 15(2):121–125
Yong TY, Li JY, Amato L et al (2008) Pituitary involvement in Wegener’s granulomatosis. Pituitary 11(1):77–84
Yoshikawa Y, Watanabe T (1986) Pulmonary lesions in Wegener’s granulomatosis: a clinicopathologic study of 22 autopsy cases. Hum Pathol 17(4):401–410
Zouaoui A, Maillard JC, Dormont D et al (1992) MRI in neurosarcoidosis. J Neuroradiol 19(4):271–284
Zycinska K, Wardyn KA, Zielonka TM et al (2007) The role ANCA and anti-GBM antibodies in pulmonary–renal syndrome due to Wegener’s granulomatosis. J Physiol Pharmacol 58(Suppl 5 Pt 2):839–846
Zycinska K, Wardyn KA, Zycinski Z et al (2008) Association between clinical activity and high-resolution tomography findings in pulmonary Wegener’s granulomacytosis. J Physiol Pharmacol 59(Suppl 6):833–838
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Silvera, S., Palangié, E., Guillevin, L., Hendaoui, L., Legmann, P. (2011). Imaging Features of Granulomatosis with Polyangiitis (Wegener’s). In: Hendaoui, L., Stanson, A., Bouhaouala, M., Joffre, F. (eds) Systemic Vasculitis. Medical Radiology(). Springer, Berlin, Heidelberg. https://doi.org/10.1007/174_2011_289
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