Zusammenfassung
Wegener-Granulomatose, Churg-Strauss-Syndrom und das chronisch hyperplastische Sinusitis-Asthma-Analgetika-Intoleranz-Syndrom zeigen meist unspezifische Manifestationen im Kopf-Hals-Bereich. Häufig sind diese Beschwerden allerdings Erstsymptome und können so frühzeitig zu einer korrekten Diagnose und einer raschen Einleitung der notwendigen Therapie führen. Die Vieldeutigkeit der Befunde und Beschwerden mit ihren mannigfaltigen und seltenen Differenzialdiagnosen stellt eine Herausforderung für den primär behandelnden Arzt dar. Dies ist aufgrund der Lokalisation der Erstmanifestation in aller Regel der Otorhinolaryngologe. Diagnostik und Therapie erfolgen in interdisziplinärer Kooperation mit Rheumatologen, Pulmonologen, Pathologen, Radiologen, Ophthalmologen, Infektiologen und Nephrologen. Trotz erheblicher wissenschaftlicher und therapeutischer Fortschritte sind die Erkrankungen nicht heilbar. In den letzten Jahrzehnten hat der Erkenntnisgewinn zu einem Wechsel von meist tödlich verlaufender Erkrankung (Wegener-Granulomatose) hin zu einem chronisch rezidivierenden Krankheitsgeschehen geführt. Die pathophysiologischen Zusammenhänge sind allerdings weiterhin weitgehend unbekannt. Das Spektrum der unerwünschten Wirkungen derzeitiger Therapien ist Ansporn für weitere Bemühungen, die Ätiologie der Erkrankungen aufzudecken und neue Therapien zu entwickeln. Aktuelle Forschungsaspekte werden in der vorliegenden Publikation zusammengefasst.
Abstract
Wegener’s granulomatosis, Churg-Strauss syndrome and analgesics intolerance syndrome with polyps demonstrate non-specific manifestations in the head and neck region. These symptoms can often lead to early diagnosis and initiation of the correct therapy. However, symptoms are often ambiguous and many rare differential diagnoses must be borne in mind. This clinical picture presents a challenge for the otorhinolaryngologist, who is commonly the first contacted physician. Diagnostics and therapy have to be carried out in an interdisciplinary approach between rheumatologist, pulmonologist, pathologist, radiologist, ophthalmologist, infection specialist and nephrologist. Despite significant scientific and therapeutic advances, these diseases remain incurable. In recent decades they have lost their life-threatening character (Wegener’s granulomatosis) and are now chronically relapsing diseases. Their aetiology, however, is still unclear and treatment leads to a wide spectrum of undesirable effects. Research work is needed to advance diagnostics and therapy in this field. Recent research aspects are presented in this article.
Abbreviations
- AAS:
-
Chronisch hyperplastisches Asthma-Analgetika-Intoleranz-Syndrom
- ACR:
-
„American College of Rheumatology“
- ANCA:
-
Anti-neutrophiler zytoplasmatischer Autoantikörper
- CSS:
-
Churg-Strauss-Syndrom
- CT:
-
Computertomographie
- ELISA:
-
„Enzyme-linked immunosorbent assay“
- ENT:
-
„Ear-nose-throat“
- Ig:
-
Immunglobulin
- IL-5:
-
Interleukin-5
- IO-SH:
-
Innenohrschwerhörigkeit, Schallempfindungsschwerhörigkeit
- NAB:
-
Nasenatmungsbehinderung
- NaCl:
-
Natriumchlorid
- NLR:
-
„NOD-like-Rezeptor“
- OM:
-
Ohrmuschel
- S. aureus :
-
Staphylococcus aureus
- SH:
-
Schleimhaut
- Sl-SH:
-
Schallleitungsschwerhörigkeit
- TGF:
-
„Transforming growth factor“
- TNF:
-
Tumor-Nekrose-Faktor
- WG:
-
Wegener-Granulomatose
Literatur
The Wegener’s Granulomatosis Etanercept Trial (WGET) Research Group (2005) Etanercept plus standard therapy for Wegener’s granulomatosis. N Engl J Med 352: 351–361
Bacciu A, Bacciu S, Mercante G et al. (2006) Ear, nose and throat manifestations of Churg-Strauss syndrome. Acta Otolaryngol 126: 503–509
Bacciu A, Buzio C, Giordano D et al. (2008) Nasal polyposis in Churg-Strauss syndrome. Laryngoscope 118: 325–329
X Bachert C, Gevaert P, Holtappels G et al. (2001) Total and specific IgE in nasal polyps is related to local eosinophilic inflammation. J Allergy Clin Immunol 107: 607–614
Bachert C, Zele T van, Gevaert P et al. (2003) Superantigens and nasal polyps. Curr Allergy Asthma Rep 3: 523–531
Bandi V, Munnur U, Braman SS (2002) Airway problems in patients with rheumatologic disorders. Crit Care Clin 18: 749–765
Bennett RW, Staker LV (1987) Wegener’s granulomatosis presenting as vertigo. West J Med 146: 359–361
Bibas A, Fahy C, Sneddon L, Bowdler D (2001) Facial paralysis in Wegener’s granulomatosis of the middle ear. J Laryngol Otol 115: 304–306
Calonius IH, Christensen CK (1980) Hearing impairment and facial palsy as initial signs of Wegener’s granulomatosis. J Laryngol Otol 94: 649–657
Choi HK, Liu S, Merkel PA et al. (2001) Diagnostic performance of antineutrophil cytoplasmic antibody tests for idiopathic vasculitides: metaanalysis with a focus on antimyeloperoxidase antibodies. J Rheumatol 28: 1584–1590
X Clement S, Vaudaux P, Francois P et al. (2005) Evidence of an intracellular reservoir in the nasal mucosa of patients with recurrent Staphylococcus aureus rhinosinusitis. J Infect Dis 192: 1023–1028
Cole AM, Tahk S, Oren A et al. (2001) Determinants of Staphylococcus aureus nasal carriage. Clin Diagn Lab Immunol 8: 1064–1069
X De Groot K, Reinhold-Keller E, Tatsis E et al. (1996) Therapy for the maintenance of remission in sixty-five patients with generalized Wegener’s granulomatosis. Methotrexate versus trimethoprim/sulfamethoxazole. Arthritis Rheum 39: 2052–2061
DeRemee RA (1988) The treatment of Wegener’s granulomatosis with trimethoprim/sulfamethoxazole: illusion or vision? Arthritis Rheum 31: 1068–1074
X DeRemee RA, McDonald TJ, Harrison EG Jr, Coles DT (1976) Wegener’s granulomatosis. Anatomic correlates, a proposed classification. Mayo Clin Proc 51: 777–781
X Devaney KO, Travis WD, Hoffman G et al. (1990) Interpretation of head and neck biopsies in Wegener’s granulomatosis. A pathologic study of 126 biopsies in 70 patients. Am J Surg Pathol 14: 555–564
X Donovan R, Johansson SG, Bennich H, Soothill JF (1970) Immunoglobulins in nasal polyp fluid. Int Arch Allergy Appl Immunol 37: 154–166
Fauci AS, Haynes BF, Katz P, Wolff SM (1983) Wegener’s granulomatosis: prospective clinical and therapeutic experience with 85 patients for 21 years. Ann Intern Med 98: 76–85
X Fries JF, Hunder GG, Bloch DA et al. (1990) The American College of Rheumatology 1990 criteria for the classification of vasculitis. Summary. Arthritis Rheum 33: 1135–1136
X Gottschlich S, Ambrosch P, Gross WL, Hellmich B (2004) Wegener’s granulomatosis in the head and neck region. HNO 52: 935–945
Griffith ME, Pusey CD (1997) HLA genes in ANCA-associated vasculitides. Exp Clin Immunogenet 14: 196–205
X Gross WL (1989) Wegener’s granulomatosis. New aspects of the disease course, immunodiagnostic procedures, and stage-adapted treatment. Sarcoidosis 6: 15–29
Gross WL (1994) New developments in the treatment of systemic vasculitis. Curr Opin Rheumatol 6: 11–19
X Gross WL, Schmitt WH, Csernok E (1993) ANCA and associated diseases: immunodiagnostic and pathogenetic aspects. Clin Exp Immunol 91: 1–12
Hagen EC (1997) Sinn und Unsinn der Anwendung von ANCA-Tests. In: Rasmussen N (Hrsg) Rheumatologie in Europa 26/4: 126–130
Hern JD, Hollis LJ, Mochloulis G et al. (1996) Early diagnosis of Wegener’s granulomatosis presenting with facial nerve palsy. J Laryngol Otol 110: 459–461
Holl-Ulrich K, Reinhold-Keller E, Muller A, Feller AC (2002) Pathology of vasculitis: differential diagnosis and selected disorders. Verh Dtsch Ges Pathol 86: 83–90
Ishiyama A, Canalis RF (2001) Otological manifestations of Churg-Strauss syndrome. Laryngoscope 111: 1619–1624
Israel HL (1988) Sulfamethoxazole-trimethoprim therapy for Wegener’s granulomatosis. Arch Intern Med 148: 2293–2295
X Jagiello P, Gross WL, Epplen JT (2005) Complex genetics of Wegener granulomatosis. Autoimmun Rev 4: 42–47
X Jennette JC, Falk RJ, Andrassy K et al. (1994) Nomenclature of systemic vasculitides. Proposal of an international consensus conference. Arthritis Rheum 37: 187–192
Jennings CR, Jones NS, Dugar J et al. (1998) Wegener’s granulomatosis–a review of diagnosis and treatment in 53 subjects. Rhinology 36: 188–191
X Lamprecht P, Voswinkel J, Lilienthal T et al. (2002) Effectiveness of TNF-alpha blockade with infliximab in refractory Wegener’s granulomatosis. Rheumatology (Oxford) 41: 1303–1307
X Leavitt RY, Fauci AS, Bloch DA et al. (1990) The American College of Rheumatology 1990 criteria for the classification of Wegener’s granulomatosis. Arthritis Rheum 33: 1101–1107
Lehmann H, Kiefer B (1990) Clinical manifestations of Wegener’s granulomatosis. APMIS Suppl 19: 19–20
X Lie JT (1997) Wegener’s granulomatosis: histological documentation of common and uncommon manifestations in 216 patients. Vasa 26: 261–270
Mahr A, Guillevin L, Poissonnet M, Ayme S (2004) 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 51: 92–99
McCaffrey TV, McDonald TJ, Facer GW, DeRemee RA (1980) Otologic manifestations of Wegener’s granulomatosis. Otolaryngol Head Neck Surg 88: 586–593
McDonald TJ, DeRemee RA (1983) Wegener’s granulomatosis. Laryngoscope 93: 220–231
McDonald TJ, DeRemee RA (1993) Head and neck involvement in Wegener’s granulomatosis (WG). Adv Exp Med Biol 336: 309–313
X Mukhtyar C, Luqmani R (2005) Current state of tumour necrosis factor {alpha} blockade in Wegener’s granulomatosis. Ann Rheum Dis (Suppl 4) 64: iv31–iv36
Mygind N, Dahl R, Bachert C (2000) Nasal polyposis, eosinophil dominated inflammation, and allergy. Thorax (Suppl 2) 55: S79–S83
Napier SS, Allen JA, Irwin CR, McCluskey DR (1993) ‚Strawberry gums’–a case of Wegener’s granulomatosis. Br Dent J 175: 327–329
Nikolaou AC, Vlachtsis KC, Daniilidis MA et al. (2001) Wegener’s granulomatosis presenting with bilateral facial nerve palsy. Eur Arch Otorhinolaryngol 258: 198–202
O’Devaney K, Ferlito A, Hunter BC et al. (1998) Wegener’s granulomatosis of the head and neck. Ann Otol Rhinol Laryngol 107: 439–445
Ohtani I, Baba Y, Suzuki C et al. (2000) Temporal bone pathology in Wegener’s granulomatosis. Fukushima J Med Sci 46: 31–39
X Paulsen JI, Rudert H (2001) Manifestations of primary vasculitis in the ENT region. Z Rheumatol 60: 219–225
Pirsig W, Pentz S, Lenders H (1993) Repair of saddle nose deformity in Wegener’s granulomatosis and ectodermal dysplasia. Rhinology 31: 69–72
Proctor RA, Eiff C von, Kahl BC et al. (2006) Small colony variants: a pathogenic form of bacteria that facilitates persistent and recurrent infections. Nat Rev Microbiol 4: 295–305
Rasmussen N, Petersen J, Remvig L, Andersen V (1990) Treatment of Wegener’s granulomatosis with trimethoprim-sulfamethoxazole. APMIS Suppl 19: 61–62
X Reinhold-Keller E, Beuge N, Latza U et al. (2000) An interdisciplinary approach to the care of patients with Wegener’s granulomatosis: long-term outcome in 155 patients. Arthritis Rheum 43: 1021–1032
X Reinhold-Keller E, De Groot K, Rudert H et al. (1996) Response to trimethoprim/sulfamethoxazole in Wegener’s granulomatosis depends on the phase of disease. QJM 89: 15–23
Riechelmann H, Essig A, Deutschle T et al. (2005) Nasal carriage of Staphylococcus aureus in house dust mite allergic patients and healthy controls. Allergy 60: 1418–1423
X Rosenstiel P, Till A, Schreiber S (2007) NOD-like receptors and human diseases. Microbes Infect 9: 648–657
Rusterholz D, Schlegel C (2000) Infectious aspects of Wegener’s granulomatosis. Schweiz Med Wochenschr Suppl 125: 41S–43S
Sandborn WJ, Hanauer SB, Katz S et al. (2001) Etanercept for active Crohn’s disease: a randomized, double-blind, placebo-controlled trial. Gastroenterology 121: 1088–1094
X Scadding GK, Durham SR, Mirakian R et al. (2008) BSACI guidelines for the management of rhinosinusitis and nasal polyposis. Clin Exp Allergy 38: 260–275
X Schafer D, Baenkler HW (2005) Functional eicosanoid test and typing (FET) of peripheral blood cells in eicosanoids related diseases. J Physiol Pharmacol (Suppl 5) 56: 103–118
X Schreiber S, Rosenstiel P, Albrecht M et al. (2005) Genetics of Crohn disease, an archetypal inflammatory barrier disease. Nat Rev Genet 6: 376–388
X Schwarz-Eywill M, Weiss A, Unger L, Nusslein H (2000) Relevance of a standardized staging study in patients with systemic vasculitis. Z Rheumatol 59: 183–190
X Settipane GA, Chafee FH (1977) Nasal polyps in asthma and rhinitis. A review of 6,037 patients. J Allergy Clin Immunol 59: 17–21
Slavin RG (1992) Allergy is not a significant cause of nasal polyps. Arch Otolaryngol Head Neck Surg 118: 771
X Stegeman CA, Tervaert JW, De Jong PE, Kallenberg CG (1996) Trimethoprim-sulfamethoxazole (co-trimoxazole) for the prevention of relapses of Wegener’s granulomatosis. Dutch Co-Trimoxazole Wegener Study Group. N Engl J Med 335: 16–20
Stewart C, Cohen D, Bhattacharyya I et al. (2007) Oral manifestations of Wegener’s granulomatosis: a report of three cases and a literature review. J Am Dent Assoc 138: 338–348
Stone JH, Francis HW (2000) Immune-mediated inner ear disease. Curr Opin Rheumatol 12: 32–40
Stone JH, Uhlfelder ML, Hellmann DB et al. (2001) Etanercept combined with conventional treatment in Wegener’s granulomatosis: a six-month open-label trial to evaluate safety. Arthritis Rheum 44: 1149–1154
Targan SR, Hanauer SB, Van Deventer SJ et al. (1997) A short-term study of chimeric monoclonal antibody cA2 to tumor necrosis factor alpha for Crohn’s disease. Crohn’s Disease cA2 Study Group. N Engl J Med 337: 1029–1035
Tos M, Sasaki Y, Ohnishi M et al. (1992) Fireside conference 2. Pathogenesis of nasal polyps. Rhinol Suppl 14: 181–185
X Zele T van, Gevaert P, Watelet JB et al. (2004) Staphylococcus aureus colonization and IgE antibody formation to enterotoxins is increased in nasal polyposis. J Allergy Clin Immunol 114: 981–983
Vaudaux P, Kelley WL, Lew DP (2006) Staphylococcus aureus small colony variants: difficult to diagnose and difficult to treat. Clin Infect Dis 43: 968–970
Velten FW, Bayerl C, Baenkler HW, Schaefer D (2006) Functional eicosanoid test and typing (FET) in acetylsalicylic acid intolerant patients with urticaria. J Physiol Pharmacol (Suppl 12) 57: 35–46
Eiff C von, Becker K, Metze D et al. (2001) Intracellular persistence of Staphylococcus aureus small-colony variants within keratinocytes: a cause for antibiotic treatment failure in a patient with darier’s disease. Clin Infect Dis 32: 1643–1647
X Waetzig GH, Rosenstiel P, Arlt A et al. (2005) Soluble tumor necrosis factor (TNF) receptor-1 induces apoptosis via reverse TNF signaling and autocrine transforming growth factor-beta1. FASEB J 19: 91–93
Waetzig GH, Schreiber S (2004) Mechanisms of infliximab: the reverse side of a drug effect. Inflamm Bowel Dis (Suppl 1) 10: S38–S43
Wegener F (1939) Über eine eigenartige rhinogene Granulomatose mit besonderer Beteiligung des Arteriensystems und der Nieren. Beitr Pathol Anatom 102: 36–68
Danksagung
Gefördert durch: DFG Klinische Forschergruppe (KFO) 170: Frühpathogenese der Wegenerschen Granulomatose: Von der natürlichen Abwehr mit Granulombildung zur Autoimmunität.
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Laudien, M., Ambrosch, P., Till, A. et al. Diagnostik, Therapie und aktuelle Forschungsaspekte ausgewählter chronisch-entzündlicher Erkrankungen mit Beteiligung im Kopf-Hals-Bereich. Z. Rheumatol. 67, 397–406 (2008). https://doi.org/10.1007/s00393-008-0324-3
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DOI: https://doi.org/10.1007/s00393-008-0324-3