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DOI: 10.1055/a-1580-7037
Granulomatose mit Polyangiitis – Manifestationen im Kopf-Hals-Bereich
Granulomatosis with polyangiitis – manifestations in the head and neck area![](https://www.thieme-connect.de/media/lro/202202/lookinside/thumbnails/lro-0978_10-1055-a-1580-7037-1.jpg)
Zusammenfassung
Die Granulomatose mit Polyangiitis ist eine seltene chronische rheumatologische Systemerkrankung, die mit einer Vaskulitis der kleinen und mittleren Gefäße einhergeht. Am häufigsten betrifft sie die oberen Atemwege, die Lunge und die Nieren. Die Beschwerden sind unspezifisch, häufig beklagen die Patienten anfangs eine Nasenatmungsbehinderung, Borkenbildung in der Nase, Ulzera der Mundschleimhäute oder Epistaxis. Nicht selten wird deshalb der Hals-Nasen-Ohren-Arzt zu Beginn der Krankheit hinzugezogen. Langfristig können schwerwiegende kardiale, renale oder pulmonale Komplikationen auftreten. Die Ätiologie ist bis heute nicht komplett geklärt. Als Therapie wird eine Immunsuppression eingeleitet. Klinische und laborchemische Kontrollen sind lebenslang obligat.
Abstract
Granulomatosis with polyangiitis is a rare chronic rheumatologic systemic disease with a vasculitis of small- and medium-size vessels. Mostly the upper airways, lung and kidneys are affected. Symptoms are unspecific. Patients complain about stuffy nose, crustiness of nasal secretions, ulcera of the oral mucosa or epistaxis. The otorhinolaryngologist may be the first one to evaluate the patient’s health condition. Long term complications may be cardial, renal or pulmonal failure. To this day the aetiology is still unknown. Severe disease is treated with a combination of immunosuppressive medications. Clinic examinations and laboratory tests should be carried out for life-time.
Schlüsselwörter
Granulomatose mit Polyangiitis - ANCA-assoziierte Vaskulitis - Autoimmunerkrankung - Immunsuppression - seltene ErkrankungenKey words
granulomatosis with polyangiitis - ANCA-associated vasculitis - autoimmune disease - immunosuppression - rare diseasePublication History
Received: 10 January 2021
Accepted: 03 August 2021
Article published online:
15 November 2021
© 2021. Thieme. All rights reserved.
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Literatur
- 1 Jennette JC, Falk RJ, Bacon PA. et al. 2012 revised International Chapel Hill Consensus Conference Nomenclature of Vasculitides. Arthritis Rheum 2013; 65: 1-11 DOI: 10.1002/art.37715.
- 2 Leavitt RY, Fauci AS, Bloch DA. et al. The American College of Rheumatology 1990 criteria for the classification of Wegener’s granulomatosis. Arthritis Rheum 1990; 33: 1101-1107 DOI: 10.1002/art.1780330807.
- 3 Jennette JC, Falk RJ, Andrassy K. et al. Nomenclature of systemic vasculitides. Proposal of an international consensus conference. Arthritis Rheum 1994; 37: 187-192 DOI: 10.1002/art.1780370206.
- 4 Communications E. Pareto-Reihe Radiologie Thorax. Im Internet (Stand: 07.01.2021): https://eref.thieme.de/ebooks/916404#/ebook_916404_SL34998880
- 5 Vahid B, Wildemore B, Nguyen C. et al. Positive C-ANCA and cavitary lung lesion: recurrence of Wegener granulomatosis or aspergillosis?. South Med J 2006; 99: 753-756 DOI: 10.1097/01.smj.0000217201.80408.8a.
- 6 Pearce FA, Grainge MJ, Lanyon PC. et al. The incidence, prevalence and mortality of granulomatosis with polyangiitis in the UK Clinical Practice Research Datalink. Rheumatology (Oxford) 2017; 56: 589-596 DOI: 10.1093/rheumatology/kew413.
- 7 Watts RA, Lane SE, Scott DG. et al. Epidemiology of vasculitis in Europe. Ann Rheum Dis 2001; 60: 1156-1157 DOI: 10.1136/ard.60.12.1156a.
- 8 Jennette JC, Falk RJ, Gasim AH. Pathogenesis of antineutrophil cytoplasmic autoantibody vasculitis. Curr Opin Nephrol Hypertens 2011; 20: 263-270 DOI: 10.1097/MNH.0b013e3283456731.
- 9 Xiao H, Hu P, Falk RJ. et al. Overview of the Pathogenesis of ANCA-Associated Vasculitis. Kidney Dis (Basel) 2016; 1: 205-215 DOI: 10.1159/000442323.
- 10 Hauschild S, Schmitt WH, Csernok E. et al. ANCA in systemic vasculitides, collagen vascular diseases, rheumatic disorders and inflammatory bowel diseases. Adv Exp Med Biol 1993; 336: 245-251 DOI: 10.1007/978-1-4757-9182-2_36.
- 11 Yates M, Watts R. ANCA-associated vasculitis. Clin Med (Lond) 2017; 17: 60-64 DOI: 10.7861/clinmedicine.17-1-60.
- 12 Csernok E, Ernst M, Schmitt W. et al. Activated neutrophils express proteinase 3 on their plasma membrane in vitro and in vivo. Clin Exp Immunol 1994; 95: 244-250 DOI: 10.1111/j.1365-2249.1994.tb06518.x.
- 13 Lamprecht P, Csernok E, Gross WL. Effector memory T cells as driving force of granuloma formation and autoimmunity in Wegener’s granulomatosis. J Intern Med 2006; 260: 187-191 DOI: 10.1111/j.1365-2796.2006.01698.x.
- 14 McKinney EF, Willcocks LC, Broecker V. et al. The immunopathology of ANCA-associated vasculitis. Semin Immunopathol 2014; 36: 461-478 DOI: 10.1007/s00281-014-0436-6.
- 15 Jennette JC, Falk RJ. Pathogenesis of the vascular and glomerular damage in ANCA-positive vasculitis. Nephrol Dial Transplant 1998; 13 (Suppl. 01) 16-20 DOI: 10.1093/ndt/13.suppl_1.16.
- 16 Pendergraft WF, Preston GA, Shah RR. et al. Autoimmunity is triggered by cPR-3(105–201), a protein complementary to human autoantigen proteinase-3. Nat Med 2004; 10: 72-79 DOI: 10.1038/nm968.
- 17 Laudien M, Gadola SD, Podschun R. et al. Nasal carriage of Staphylococcus aureus and endonasal activity in Wegener s granulomatosis as compared to rheumatoid arthritis and chronic Rhinosinusitis with nasal polyps. Clin Exp Rheumatol 2010; 28: 51-55
- 18 Lamprecht P, Fischer N, Huang J. et al. Changes in the composition of the upper respiratory tract microbial community in granulomatosis with polyangiitis. J Autoimmun 2019; 97: 29-39 DOI: 10.1016/j.jaut.2018.10.005.
- 19 Merkel PA, Xie G, Monach PA. et al. Identification of Functional and Expression Polymorphisms Associated With Risk for Antineutrophil Cytoplasmic Autoantibody-Associated Vasculitis. Arthritis Rheumatol 2017; 69: 1054-1066 DOI: 10.1002/art.40034.
- 20 Devaney KO, Travis WD, Hoffman G. et al. Interpretation of head and neck biopsies in Wegener’s granulomatosis. A pathologic study of 126 biopsies in 70 patients. Am J Surg Pathol 1990; 14: 555-564 DOI: 10.1097/00000478-199006000-00006.
- 21 Maguchi S, Fukuda S, Takizawa M. Histological findings in biopsies from patients with cytoplasmic-antineutrophil cytoplasmic antibody (cANCA)-positive Wegener’s granulomatosis. Auris Nasus Larynx 2001; 28: S53-S58 DOI: 10.1016/s0385-8146(01)00072-4.
- 22 Kühn D, Hospowsky C, Both M. et al. Manifestation of granulomatosis with polyangiitis in head and neck. Clin Exp Rheumatol 2018; 36 (Suppl. 111) 78-84
- 23 Gottschlich S, Ambrosch P, Kramkowski D. et al. Head and neck manifestations of Wegener’s granulomatosis. Rhinology 2006; 44: 227-233
- 24 D’Anza B, Langford CA, Sindwani R. Sinonasal imaging findings in granulomatosis with polyangiitis (Wegener granulomatosis): A systematic review. Am J Rhinol Allergy 2017; 31: 16-21 DOI: 10.2500/ajra.2017.31.4408.
- 25 Pendolino AL, Unadkat S, Zhang H. et al. The role of surgery in antineutrophil cytoplasmic antibody-associated vasculitides affecting the nose and sinuses: A systematic review. SAGE Open Med 2020; 8: 2050312120936731 DOI: 10.1177/2050312120936731.
- 26 Sachse F, Stoll W. [Nasal surgery in patients with systemic disorders]. Laryngorhinootologie 2010; 89 (Suppl. 01) S103-115 DOI: 10.1055/s-0029-1246127.
- 27 Ito O, Yano T, Ito M. Can a Saddle Nose Deformity of Granulomatosis with Polyangiitis be Repaired?. Aesthetic Plast Surg 2017; 41: 1463-1464 DOI: 10.1007/s00266-017-0888-x.
- 28 Hoffman GS, Kerr GS, Leavitt RY. et al. Wegener granulomatosis: an analysis of 158 patients. Ann Intern Med 1992; 116: 488-498 DOI: 10.7326/0003-4819-116-6-488.
- 29 Santos F, Salviz M, Domond H. et al. Otopathology of Vasculitis in Granulomatosis With Polyangitis. Otol Neurotol 2015; 36: 1657-1662 DOI: 10.1097/MAO.0000000000000868.
- 30 Michel O, Bamborschke S, Nekic M. et al. Beta-trace protein (prostaglandin D synthase)--a stable and reliable protein in perilymph. Ger Med Sci 2005; 3: Doc04
- 31 LL 030 141 Lumbalpunktion und Liquordiagnostik 2019 – Deutsche Gesellschaft für Neurologie e. V. Im Internet (Stand: 09.01.2021): https://dgn.org/leitlinien/ll-030-141-lumbalpunktion-und-liquordiagnostik-2019/
- 32 Specks U, Colby TV, Olsen KD. et al. Salivary gland involvement in Wegener’s granulomatosis. Arch Otolaryngol Head Neck Surg 1991; 117: 218-223 DOI: 10.1001/archotol.1991.01870140106018.
- 33 Gluth MB, Shinners PA, Kasperbauer JL. Subglottic stenosis associated with Wegener’s granulomatosis. Laryngoscope 2003; 113: 1304-1307 DOI: 10.1097/00005537-200308000-00008.
- 34 Carnevale C, Arancibia-Tagle D, Sarría-Echegaray P. et al. Head and Neck Manifestations of Granulomatosis with Polyangiitis: A Retrospective analysis of 19 Patients and Review of the Literature. Int Arch Otorhinolaryngol 2019; 23: 165-171 DOI: 10.1055/s-0038-1675759.
- 35 Solans-Laqué R, Bosch-Gil J, Canela M. et al. Clinical features and therapeutic management of subglottic stenosis in patients with Wegener’s granulomatosis. Lupus 2008; 17: 832-836 DOI: 10.1177/0961203308089693.
- 36 Ponniah I, Shaheen A, Shankar KA. et al. Wegener’s granulomatosis: the current understanding. Oral Surg Oral Med Oral Pathol Oral Radiol Endod 2005; 100: 265-270 DOI: 10.1016/j.tripleo.2005.04.018.
- 37 Ghiasi M. Strawberry Gingivitis in Granulomatosis with Polyangiitis. N Engl J Med 2017; 377: 2073 DOI: 10.1056/NEJMicm1706987.
- 38 Almouhawis HA, Leao JC, Fedele S. et al. Wegener’s granulomatosis: a review of clinical features and an update in diagnosis and treatment. J Oral Pathol Med 2013; 42: 507-516 DOI: 10.1111/jop.12030.
- 39 Tarzi RM, Pusey CD. Current and future prospects in the management of granulomatosis with polyangiitis (Wegener’s granulomatosis). Ther Clin Risk Manag 2014; 10: 279-293 DOI: 10.2147/TCRM.S41598.
- 40 de Groot 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 2009; 150: 670-680 DOI: 10.7326/0003-4819-150-10-200905190-00004.
- 41 Jones RB, Tervaert JWC, Hauser T. et al. Rituximab versus cyclophosphamide in ANCA-associated renal vasculitis. N Engl J Med 2010; 363: 211-220 DOI: 10.1056/NEJMoa0909169.
- 42 Voswinkel J, Mueller A, Kraemer JA. et al. B lymphocyte maturation in Wegener’s granulomatosis: a comparative analysis of VH genes from endonasal lesions. Ann Rheum Dis 2006; 65: 859-864 DOI: 10.1136/ard.2005.044909.
- 43 Walsh M, Merkel PA, Peh C-A. et al. Plasma Exchange and Glucocorticoids in Severe ANCA-Associated Vasculitis. New England Journal of Medicine 2020; 382: 622-631 DOI: 10.1056/NEJMoa1803537.
- 44 Yates M, Watts RA, Bajema IM. et al. EULAR/ERA-EDTA recommendations for the management of ANCA-associated vasculitis. Ann Rheum Dis 2016; 75: 1583-1594 DOI: 10.1136/annrheumdis-2016-209133.
- 45 Guillevin L, Pagnoux C, Karras A. et al. Rituximab versus azathioprine for maintenance in ANCA-associated vasculitis. N Engl J Med 2014; 371: 1771-1780 DOI: 10.1056/NEJMoa1404231.
- 46 Zycinska K, Wardyn KA, Zielonka TM. et al. Co-trimoxazole and prevention of relapses of PR3-ANCA positive vasculitis with pulmonary involvement. Eur J Med Res 2009; 14 (Suppl. 04) 265-267 DOI: 10.1186/2047-783x-14-s4-265.
- 47 Holle JU, Reinhold-Keller E, Gross WL. [Update on granulomatosis with polyangitis (GPA, Wegener’s granulomatosis)]. Z Rheumatol 2012; 71: 745-753 DOI: 10.1007/s00393-012-0982-z.
- 48 Robson J, Doll H, Suppiah R. et al. Damage in the anca-associated vasculitides: long-term data from the European vasculitis study group (EUVAS) therapeutic trials. Ann Rheum Dis 2015; 74: 177-184 DOI: 10.1136/annrheumdis-2013-203927.
- 49 Flossmann O, Berden A, de Groot K. et al. Long-term patient survival in ANCA-associated vasculitis. Ann Rheum Dis 2011; 70: 488-494 DOI: 10.1136/ard.2010.137778.
- 50 Wallace ZS, Lu N, Unizony S. et al. Improved survival in granulomatosis with polyangiitis: A general population-based study. Semin Arthritis Rheum 2016; 45: 483-489 DOI: 10.1016/j.semarthrit.2015.07.009.