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ANCA-Associated Vasculitis—ENT Involvement

  • Trimarchi MatteoEmail author
  • Galli Andrea
  • Roberto Teggi
Chapter
Part of the Rare Diseases of the Immune System book series (RDIS)

Abstract

Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) are idiopathic, immunologically mediated diseases predominantly affecting small vessels throughout the body (capillaries, venules arterioles, and small arteries), which are pathogenetically associated with pauci-immune vasculitis and with either proteinase 3-ANCA (PR3-ANCA or c-ANCA) or myeloperoxidase-ANCA (MPO-ANCA or p-ANCA). The 2012 revised Chapel Hill Consensus Conference (CHCC) defined microscopic polyangiitis (MPA), granulomatosis with polyangiitis (GPA, formerly Wegener’s granulomatosis), eosinophilic granulomatosis with polyangiitis (EGPA, also known as Churg–Strauss syndrome), and single-organ manifestations (e.g., acute renal-limited disease known as pauci-immune necrotizing crescentic glomerulonephritis, NCGN) as the main clinic-pathological variants within the AAV spectrum.

Head and neck involvement in the course of AAV is a quite frequent finding, and not uncommonly a first and stand-alone sign of active disease. This is particularly true for GPA and EGPA, especially in limited forms of disease: for this reason, otorhinolaryngologists become key figures in the multidisciplinary team approaching these conditions, especially in the diagnostic phase.

Collaterally to AAV, the so-called cocaine-induced midline destructive lesions (CIMDL) are emerging pathologies caused by habitual cocaine insufflation commonly presenting with clinical, endoscopic, serological, and histopathological features resembling those of systemic vasculitis. Differential diagnosis of CIMDL from a sinonasal, limited form of GPA is often challenging, owing to their large similarity and the reluctance of patients in admitting cocaine addiction: this must always be kept in mind in AAV diagnostic algorithms.

Keywords

ANCA-associated vasculitis Granulomatosis with polyangiitis Eosinophilic granulomatosis with polyangiitis Microscopic polyangiitis Cocaine-induced midline destructive lesions ENT involvement Granulomatosis 

References

  1. 1.
    Jennette JC, Falk RJ, Hu P, Xiao H. Pathogenesis of antineutrophil cytoplasmic autoantibody-associated small-vessel vasculitis. Annu Rev Pathol. 2013;8:139–60.CrossRefGoogle Scholar
  2. 2.
    Berden A, Goceroglu A, Jayne D, et al. Diagnosis and management of ANCA associated vasculitis. BMJ. 2012;344:e26.CrossRefGoogle Scholar
  3. 3.
    Jennette JC, Falk RJ, Bacon PA, et al. 2012 revised international Chapel Hill consensus conference nomenclature of Vasculitides. Arthritis Rheum. 2013;65(1):1–11.CrossRefGoogle Scholar
  4. 4.
    Fujimoto S, Watts RA, Kobayashi S, et al. Comparison of the epidemiology of anti-neutrophil cytoplasmic antibody-associated vasculitis between Japan and the U.K. Rheumatology (Oxford). 2011;50(10):1916–20.CrossRefGoogle Scholar
  5. 5.
    Hellmich B, Flossmann O, Gross WL, et al. EULAR recommendations for conducting clinical studies and/or clinical trials in systemic vasculitis: focus on anti-neutrophil cytoplasm antibody-associated vasculitis. Ann Rheum Dis. 2007;66(5):605–17.CrossRefGoogle Scholar
  6. 6.
    Hoffman GS, Langford CA. Are there different forms of life in the antineutrophil cytoplasmic antibody universe? Ann Intern Med. 2005;143(9):683–5.CrossRefGoogle Scholar
  7. 7.
    Jennette JC, Falk RJ. Small-vessel vasculitis. N Engl J Med. 1997;337(21):1512–23.CrossRefGoogle Scholar
  8. 8.
    Chung SA, Seo P. Microscopic polyangiitis. Rheum Dis Clin N Am. 2010;36(3):545–58.CrossRefGoogle Scholar
  9. 9.
    Greco A, De Virgilio A, Rizzo MI, et al. Microscopic polyangiitis: advances in diagnostic and therapeutic approaches. Autoimmun Rev. 2015;14(9):837–44.CrossRefGoogle Scholar
  10. 10.
    Guillevin L, Durand-Gasselin B, Cevallos R, et al. Microscopic polyangiitis: clinical and laboratory findings in eighty-five patients. Arthritis Rheum. 1999;42(3):421–30.Google Scholar
  11. 11.
    Savage CO, Winearls CG, Evans DJ, Rees AJ, Lockwood CM. Microscopic polyarteritis: presentation, pathology and prognosis. Q J Med. 1985;56(220):467–83.Google Scholar
  12. 12.
    Adu D, Howie AJ, Scott DG, Bacon PA, McGonigle RJ, Micheal J. Polyarteritis and the kidney. Q J Med. 1987;62(239):221–37.Google Scholar
  13. 13.
    Serra A, Cameron JS, Turner DR, et al. Vasculitis affecting the kidney: presentation, histopathology and long-term outcome. Q J Med. 1984;53(210):181–207.Google Scholar
  14. 14.
    Veldman J. Immune-mediated sensorineural hearing loss. Auris Nasus Larynx. 1998;25(3):309–17.CrossRefGoogle Scholar
  15. 15.
    Falk RJ, Gross WL, Guillevin L, et al. Granulomatosis with polyangiitis (Wegener’s): an alternative name for Wegener’s granulomatosis. Arthritis Rheum. 2011;63(4):863–4.Google Scholar
  16. 16.
    Mark EJ, Matsubara O, Tan-Liu NS, Fienberg R. The pulmonary biopsy in the early diagnosis of Wegener’s (pathergic) granulomatosis: a study based on 35 open lung biopsies. Hum Pathol. 1988;19(9):1065–71.CrossRefGoogle Scholar
  17. 17.
    Kornblut AD, Wolff SM, deFries HO, Fauci AS. Wegener’s granulomatosis. Laryngoscope. 1980;90(9):1453–65.CrossRefGoogle Scholar
  18. 18.
    Trimarchi M, Sinico RA, Teggi R, Bussi M, Specks U, Meroni PL. Otorhinolaryngological manifestations in granulomatosis with polyangiitis (Wegener’s). Autoimmun Rev. 2013;12(4):501–5.CrossRefGoogle Scholar
  19. 19.
    Hartl DM, Aidan P, Brugiere O, Sterkers O. Wegener’s granulomatosis presenting as a recurrence of chronic otitis media. Am J Otolaryngol. 1998;19(1):54–60.CrossRefGoogle Scholar
  20. 20.
    Sinico RA, Di Toma L, Maggiore U, et al. Renal involvement in Churg-Strauss syndrome. Am J Kidney Dis. 2006;47(5):770–9.CrossRefGoogle Scholar
  21. 21.
    Takagi D, Nakamaru Y, Maguchi S, Furuta Y, Fukuda S. Otologic manifestations of Wegener’s granulomatosis. Laryngoscope. 2002;112(9):1684–90.CrossRefGoogle Scholar
  22. 22.
    Gubbels SP, Barkhuizen A, Hwang PH. Head and neck manifestations of Wegener’s granulomatosis. Otolaryngol Clin N Am. 2003;36(4):685–705.CrossRefGoogle Scholar
  23. 23.
    Sinico RA, Di Toma L, Maggiore U, et al. Prevalence and clinical significance of antineutrophil cytoplasmic antibodies in Churg-Strauss syndrome. Arthritis Rheum. 2005;52(9):2926–35.CrossRefGoogle Scholar
  24. 24.
    Sable-Fourtassou R, Cohen P, Mahr A, et al. Antineutrophil cytoplasmic antibodies and the Churg-Strauss syndrome. Ann Intern Med. 2005;143(9):632–8.CrossRefGoogle Scholar
  25. 25.
    Baldini C, Talarico R, Della Rossa A, Bombardieri S. Clinical manifestations and treatment of Churg-Strauss syndrome. Rheum Dis Clin N Am. 2010;36(3):527–43.CrossRefGoogle Scholar
  26. 26.
    Papadimitraki ED, Kyrmizakis DE, Kritikos I, Boumpas DT. Ear-nose-throat manifestations of autoimmune rheumatic diseases. Clin Exp Rheumatol. 2004;22(4):485–94.PubMedGoogle Scholar
  27. 27.
    Mahr A, Guillevin L, Poissonnet M, Ayme S. Prevalences of polyarteritis nodosa, microscopic polyangiitis, Wegener’s granulomatosis, and Churg-Strauss syndrome in a French urban multiethnic population in 2000: a capture-recapture estimate. Arthritis Rheum. 2004;51(1):92–9.CrossRefGoogle Scholar
  28. 28.
    Sinico RA, Bottero P. Churg-Strauss angiitis. Best Pract Res Clin Rheumatol. 2009;23(3):355–66.CrossRefGoogle Scholar
  29. 29.
    Trimarchi M, Bussi M, Sinico RA, Meroni P, Specks U. Cocaine-induced midline destructive lesions - an autoimmune disease? Autoimmun Rev. 2013;12(4):496–500.CrossRefGoogle Scholar
  30. 30.
    Seyer BA, Grist W, Muller S. Aggressive destructive midfacial lesion from cocaine abuse. Oral Surg Oral Med Oral Pathol Oral Radiol Endod. 2002;94(4):465–70.CrossRefGoogle Scholar
  31. 31.
    Trimarchi M, Gregorini G, Facchetti F, et al. Cocaine-induced midline destructive lesions: clinical, radiographic, histopathologic, and serologic features and their differentiation from Wegener granulomatosis. Medicine (Baltimore). 2001;80(6):391–404.CrossRefGoogle Scholar
  32. 32.
    Trimarchi M, Nicolai P, Lombardi D, et al. Sinonasal osteocartilaginous necrosis in cocaine abusers: experience in 25 patients. Am J Rhinol. 2003;17(1):33–43.CrossRefGoogle Scholar
  33. 33.
    Metaxaris G, Prokopakis EP, Karatzanis AD, et al. Otolaryngologic manifestations of small vessel vasculitis. Auris Nasus Larynx. 2002;29(4):353–6.CrossRefGoogle Scholar
  34. 34.
    Greco A, Marinelli C, Fusconi M, et al. Clinic manifestations in granulomatosis with polyangiitis. Int J Immunopathol Pharmacol. 2016;29(2):151–9.CrossRefGoogle Scholar
  35. 35.
    McDonald TJ, DeRemee RA. Wegener's granulomatosis. Laryngoscope. 1983;93(2):220–31.CrossRefGoogle Scholar
  36. 36.
    Rasmussen N. Management of the ear, nose, and throat manifestations of Wegener granulomatosis: an otorhinolaryngologist's perspective. Curr Opin Rheumatol. 2001;13(1):3–11.CrossRefGoogle Scholar
  37. 37.
    Wojciechowska J, Krajewski W, Krajewski P, Krecicki T. Granulomatosis with Polyangiitis in otolaryngologist practice: a review of current knowledge. Clin Exp Otorhinolaryngol. 2016;9(1):8–13.CrossRefGoogle Scholar
  38. 38.
    Kwan AS, Rose GE. Lacrimal drainage surgery in Wegener’s granulomatosis. Br J Ophthalmol. 2000;84(3):329–31.CrossRefGoogle Scholar
  39. 39.
    Muhle C, Reinhold-Keller E, Richter C, et al. MRI of the nasal cavity, the paranasal sinuses and orbits in Wegener’s granulomatosis. Eur Radiol. 1997;7(4):566–70.CrossRefGoogle Scholar
  40. 40.
    Trimarchi M, Bozzolo E, Pilolli F, et al. Nasal mucosa narrow band imaging in granulomatosis with polyangiitis (Wegener granulomatosis): a preliminary study. Am J Rhinol Allergy. 2015;29(3):170–4.Google Scholar
  41. 41.
    Langford CA, Hoffman GS. Rare diseases.3: Wegener’s granulomatosis. Thorax. 1999;54(7):629–37.CrossRefGoogle Scholar
  42. 42.
    Nicklasson B, Stangeland N. Wegener’s granulomatosis presenting as otitis media. J Laryngol Otol. 1982;96(3):277–80.CrossRefGoogle Scholar
  43. 43.
    Illum P, Thorling K. Otological manifestations of Wegener’s granulomatosis. Laryngoscope. 1982;92(7 Pt 1):801–4.CrossRefGoogle Scholar
  44. 44.
    Moussa AE, Abou-Elhmd KA. Wegener’s granulomatosis presenting as mastoiditis. Ann Otol Rhinol Laryngol. 1998;107(7):560–3.CrossRefGoogle Scholar
  45. 45.
    Wierzbicka M, Szyfter W, Puszczewicz M, Borucki L, Bartochowska A. Otologic symptoms as initial manifestation of wegener granulomatosis: diagnostic dilemma. Otol Neurotol. 2011;32(6):996–1000.CrossRefGoogle Scholar
  46. 46.
    Holle JU, Gross WL. Neurological involvement in Wegener’s granulomatosis. Curr Opin Rheumatol. 2011;23(1):7–11.CrossRefGoogle Scholar
  47. 47.
    Teszler CB, Williams MT, Belange G, Ayache D. Labyrinthitis related to Wegener granulomatosis: magnetic resonance imaging findings. Otol Neurotol. 2008;29(5):721–2.PubMedGoogle Scholar
  48. 48.
    Dagum P, Roberson JB Jr. Otologic Wegener’s granulomatosis with facial nerve palsy. Ann Otol Rhinol Laryngol. 1998;107(7):555–9.CrossRefGoogle Scholar
  49. 49.
    Fenton JE, O'Sullivan TJ. The otological manifestations of Wegener’s granulomatosis. J Laryngol Otol. 1994;108(2):144–6.CrossRefGoogle Scholar
  50. 50.
    Kobari Y, Nagasawa T. Otitis media with ANCA-associated vasculitis: a new concept and the associated criteria. Intern Med. 2017;56(24):3365–7.CrossRefGoogle Scholar
  51. 51.
    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. 1983;98(1):76–85.CrossRefGoogle Scholar
  52. 52.
    Knecht K, Mishriki YY. More than a mouth ulcer. Oral ulcer due to Wegener's granulomatosis. Postgrad Med. 1999;105(5):200, 3.CrossRefGoogle Scholar
  53. 53.
    Patten SF, Tomecki KJ. Wegener’s granulomatosis: cutaneous and oral mucosal disease. J Am Acad Dermatol. 1993;28(5. Pt 1):710–8.CrossRefGoogle Scholar
  54. 54.
    Knight JM, Hayduk MJ, Summerlin DJ, Mirowski GW. "Strawberry" gingival hyperplasia: a pathognomonic mucocutaneous finding in Wegener granulomatosis. Arch Dermatol. 2000;136(2):171–3.CrossRefGoogle Scholar
  55. 55.
    Trimarchi M, Bondi S, Della Torre E, Terreni MR, Bussi M. Palate perforation differentiates cocaine-induced midline destructive lesions from granulomatosis with polyangiitis. Acta Otorhinolaryngol Ital. 2017;37(4):281–5.PubMedPubMedCentralGoogle Scholar
  56. 56.
    Specks U, Colby TV, Olsen KD, DeRemee RA. Salivary gland involvement in Wegener’s granulomatosis. Arch Otolaryngol Head Neck Surg. 1991;117(2):218–23.CrossRefGoogle Scholar
  57. 57.
    Ah-See KW, McLaren K, Maran AG. Wegener’s granulomatosis presenting as major salivary gland enlargement. J Laryngol Otol. 1996;110(7):691–3.CrossRefGoogle Scholar
  58. 58.
    Daum TE, Specks U, Colby TV, et al. Tracheobronchial involvement in Wegener’s granulomatosis. Am J Respir Crit Care Med. 1995;151(2 Pt 1):522–6.CrossRefGoogle Scholar
  59. 59.
    Gluth MB, Shinners PA, Kasperbauer JL. Subglottic stenosis associated with Wegener’s granulomatosis. Laryngoscope. 2003;113(8):1304–7.CrossRefGoogle Scholar
  60. 60.
    Hoffman GS, Thomas-Golbanov CK, Chan J, Akst LM, Eliachar I. Treatment of subglottic stenosis, due to Wegener’s granulomatosis, with intralesional corticosteroids and dilation. J Rheumatol. 2003;30(5):1017–21.PubMedGoogle Scholar
  61. 61.
    Cordier JF, Valeyre D, Guillevin L, Loire R, Brechot JM. Pulmonary Wegener’s granulomatosis. A clinical and imaging study of 77 cases. Chest. 1990;97(4):906–12.CrossRefGoogle Scholar
  62. 62.
    Lebovics RS, Hoffman GS, Leavitt RY, et al. The management of subglottic stenosis in patients with Wegener’s granulomatosis. Laryngoscope. 1992;102(12 Pt 1):1341–5.CrossRefGoogle Scholar
  63. 63.
    McDonald TJ, Neel HB 3rd, DeRemee RA. Wegener’s granulomatosis of the subglottis and the upper portion of the trachea. Ann Otol Rhinol Laryngol. 1982;91(6 Pt 1):588–92.CrossRefGoogle Scholar
  64. 64.
    Utzig MJ, Warzelhan J, Wertzel H, Berwanger I, Hasse J. Role of thoracic surgery and interventional bronchoscopy in Wegener’s granulomatosis. Ann Thorac Surg. 2002;74(6):1948–52.CrossRefGoogle Scholar
  65. 65.
    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(1):131–41.CrossRefGoogle Scholar
  66. 66.
    Greco A, Rizzo MI, De Virgilio A, et al. Churg-Strauss syndrome. Autoimmun Rev. 2015;14(4):341–8.CrossRefGoogle Scholar
  67. 67.
    Nakamaru Y, Takagi D, Suzuki M, et al. Otologic and Rhinologic manifestations of eosinophilic granulomatosis with polyangiitis. Audiol Neurootol. 2016;21(1):45–53.CrossRefGoogle Scholar
  68. 68.
    Seccia V, Fortunato S, Cristofani-Mencacci L, et al. Focus on audiologic impairment in eosinophilic granulomatosis with polyangiitis. Laryngoscope. 2016;126(12):2792–7.CrossRefGoogle Scholar
  69. 69.
    Allard FD, Yee EU, Freitag SK. Dacryocystitis secondary to intranasal cocaine abuse: a case report and literature review. Orbit. 2013;32(6):405–8.CrossRefGoogle Scholar
  70. 70.
    Alexandrakis G, Tse DT, Rosa RH Jr, Johnson TE. Nasolacrimal duct obstruction and orbital cellulitis associated with chronic intranasal cocaine abuse. Arch Ophthalmol. 1999;117(12):1617–22.CrossRefGoogle Scholar
  71. 71.
    Iqbal N. Hearing loss in amphetamine users. J Psychoactive Drugs. 2002;34(4):401–7.CrossRefGoogle Scholar
  72. 72.
    Ciorba A, Bovo R, Prosser S, Martini A. Considerations on the physiopathological mechanism of inner ear damage induced by intravenous cocaine abuse: cues from a case report. Auris Nasus Larynx. 2009;36(2):213–7.CrossRefGoogle Scholar
  73. 73.
    Nicoucar K, Sakbani K, Vukanovic S, Guyot JP. Intralabyrinthine haemorrhage following cocaine consumption. Acta Otolaryngol. 2005;125(8):899–901.CrossRefGoogle Scholar
  74. 74.
    Bauman JL, DiDomenico RJ. Cocaine-induced channelopathies: emerging evidence on the multiple mechanisms of sudden death. J Cardiovasc Pharmacol Ther. 2002;7(3):195–202.CrossRefGoogle Scholar
  75. 75.
    Trimarchi M, Miluzio A, Nicolai P, Morassi ML, Bussi M, Marchisio PC. Massive apoptosis erodes nasal mucosa of cocaine abusers. Am J Rhinol. 2006;20(2):160–4.CrossRefGoogle Scholar
  76. 76.
    Wiesner O, Russell KA, Lee AS, et al. Antineutrophil cytoplasmic antibodies reacting with human neutrophil elastase as a diagnostic marker for cocaine-induced midline destructive lesions but not autoimmune vasculitis. Arthritis Rheum. 2004;50(9):2954–65.CrossRefGoogle Scholar
  77. 77.
    Peikert T, Finkielman JD, Hummel AM, et al. Functional characterization of antineutrophil cytoplasmic antibodies in patients with cocaine-induced midline destructive lesions. Arthritis Rheum. 2008;58(5):1546–51.CrossRefGoogle Scholar

Copyright information

© Springer Nature Switzerland AG 2020

Authors and Affiliations

  • Trimarchi Matteo
    • 1
    • 2
    • 3
    Email author
  • Galli Andrea
    • 1
    • 2
  • Roberto Teggi
    • 1
    • 2
  1. 1.Otorhinolaryngology—Head and Neck Surgery DepartmentSan Raffaele HospitalMilanItaly
  2. 2.ENT—Head and Neck Surgery DepartmentIRCCS San RaffaeleMilanItaly
  3. 3.Department of OtorhinolaryngologySan Raffaele Hospital, Vita-Salute UniversityMilanItaly

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