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Diagnostik, Therapie und aktuelle Forschungsaspekte ausgewählter chronisch-entzündlicher Erkrankungen mit Beteiligung im Kopf-Hals-Bereich

Diagnosis, therapy and current research aspects of selected chronic inflammatory diseases with head and neck involvement

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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.

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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

  1. 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

    Article  Google Scholar 

  2. Bacciu A, Bacciu S, Mercante G et al. (2006) Ear, nose and throat manifestations of Churg-Strauss syndrome. Acta Otolaryngol 126: 503–509

    Article  PubMed  Google Scholar 

  3. Bacciu A, Buzio C, Giordano D et al. (2008) Nasal polyposis in Churg-Strauss syndrome. Laryngoscope 118: 325–329

    Article  PubMed  Google Scholar 

  4. 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

    Article  Google Scholar 

  5. Bachert C, Zele T van, Gevaert P et al. (2003) Superantigens and nasal polyps. Curr Allergy Asthma Rep 3: 523–531

    Article  PubMed  Google Scholar 

  6. Bandi V, Munnur U, Braman SS (2002) Airway problems in patients with rheumatologic disorders. Crit Care Clin 18: 749–765

    Article  PubMed  Google Scholar 

  7. Bennett RW, Staker LV (1987) Wegener’s granulomatosis presenting as vertigo. West J Med 146: 359–361

    PubMed  CAS  Google Scholar 

  8. 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

    Article  PubMed  CAS  Google Scholar 

  9. Calonius IH, Christensen CK (1980) Hearing impairment and facial palsy as initial signs of Wegener’s granulomatosis. J Laryngol Otol 94: 649–657

    Article  PubMed  CAS  Google Scholar 

  10. 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

    PubMed  CAS  Google Scholar 

  11. 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

    Article  Google Scholar 

  12. Cole AM, Tahk S, Oren A et al. (2001) Determinants of Staphylococcus aureus nasal carriage. Clin Diagn Lab Immunol 8: 1064–1069

    Article  PubMed  CAS  Google Scholar 

  13. 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

    Article  Google Scholar 

  14. DeRemee RA (1988) The treatment of Wegener’s granulomatosis with trimethoprim/sulfamethoxazole: illusion or vision? Arthritis Rheum 31: 1068–1074

    Article  PubMed  CAS  Google Scholar 

  15. 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

    Google Scholar 

  16. 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

    Article  Google Scholar 

  17. X Donovan R, Johansson SG, Bennich H, Soothill JF (1970) Immunoglobulins in nasal polyp fluid. Int Arch Allergy Appl Immunol 37: 154–166

    Google Scholar 

  18. 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

    PubMed  CAS  Google Scholar 

  19. 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

    Google Scholar 

  20. X Gottschlich S, Ambrosch P, Gross WL, Hellmich B (2004) Wegener’s granulomatosis in the head and neck region. HNO 52: 935–945

    Article  Google Scholar 

  21. Griffith ME, Pusey CD (1997) HLA genes in ANCA-associated vasculitides. Exp Clin Immunogenet 14: 196–205

    PubMed  CAS  Google Scholar 

  22. X Gross WL (1989) Wegener’s granulomatosis. New aspects of the disease course, immunodiagnostic procedures, and stage-adapted treatment. Sarcoidosis 6: 15–29

    Google Scholar 

  23. Gross WL (1994) New developments in the treatment of systemic vasculitis. Curr Opin Rheumatol 6: 11–19

    Article  PubMed  CAS  Google Scholar 

  24. X Gross WL, Schmitt WH, Csernok E (1993) ANCA and associated diseases: immunodiagnostic and pathogenetic aspects. Clin Exp Immunol 91: 1–12

    Google Scholar 

  25. Hagen EC (1997) Sinn und Unsinn der Anwendung von ANCA-Tests. In: Rasmussen N (Hrsg) Rheumatologie in Europa 26/4: 126–130

  26. 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

    PubMed  CAS  Google Scholar 

  27. 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

    PubMed  CAS  Google Scholar 

  28. Ishiyama A, Canalis RF (2001) Otological manifestations of Churg-Strauss syndrome. Laryngoscope 111: 1619–1624

    Article  PubMed  CAS  Google Scholar 

  29. Israel HL (1988) Sulfamethoxazole-trimethoprim therapy for Wegener’s granulomatosis. Arch Intern Med 148: 2293–2295

    Article  PubMed  CAS  Google Scholar 

  30. X Jagiello P, Gross WL, Epplen JT (2005) Complex genetics of Wegener granulomatosis. Autoimmun Rev 4: 42–47

    Article  CAS  Google Scholar 

  31. 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

    Article  Google Scholar 

  32. 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

    PubMed  CAS  Google Scholar 

  33. 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

  34. 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

    Google Scholar 

  35. Lehmann H, Kiefer B (1990) Clinical manifestations of Wegener’s granulomatosis. APMIS Suppl 19: 19–20

    PubMed  CAS  Google Scholar 

  36. X Lie JT (1997) Wegener’s granulomatosis: histological documentation of common and uncommon manifestations in 216 patients. Vasa 26: 261–270

    Google Scholar 

  37. 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

    Article  PubMed  Google Scholar 

  38. McCaffrey TV, McDonald TJ, Facer GW, DeRemee RA (1980) Otologic manifestations of Wegener’s granulomatosis. Otolaryngol Head Neck Surg 88: 586–593

    PubMed  CAS  Google Scholar 

  39. McDonald TJ, DeRemee RA (1983) Wegener’s granulomatosis. Laryngoscope 93: 220–231

    Article  PubMed  CAS  Google Scholar 

  40. McDonald TJ, DeRemee RA (1993) Head and neck involvement in Wegener’s granulomatosis (WG). Adv Exp Med Biol 336: 309–313

    PubMed  CAS  Google Scholar 

  41. 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

    Google Scholar 

  42. Mygind N, Dahl R, Bachert C (2000) Nasal polyposis, eosinophil dominated inflammation, and allergy. Thorax (Suppl 2) 55: S79–S83

  43. Napier SS, Allen JA, Irwin CR, McCluskey DR (1993) ‚Strawberry gums’–a case of Wegener’s granulomatosis. Br Dent J 175: 327–329

    Article  PubMed  CAS  Google Scholar 

  44. Nikolaou AC, Vlachtsis KC, Daniilidis MA et al. (2001) Wegener’s granulomatosis presenting with bilateral facial nerve palsy. Eur Arch Otorhinolaryngol 258: 198–202

    Article  PubMed  CAS  Google Scholar 

  45. 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

    Google Scholar 

  46. Ohtani I, Baba Y, Suzuki C et al. (2000) Temporal bone pathology in Wegener’s granulomatosis. Fukushima J Med Sci 46: 31–39

    PubMed  CAS  Google Scholar 

  47. X Paulsen JI, Rudert H (2001) Manifestations of primary vasculitis in the ENT region. Z Rheumatol 60: 219–225

    Article  Google Scholar 

  48. Pirsig W, Pentz S, Lenders H (1993) Repair of saddle nose deformity in Wegener’s granulomatosis and ectodermal dysplasia. Rhinology 31: 69–72

    PubMed  CAS  Google Scholar 

  49. 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

    Article  PubMed  CAS  Google Scholar 

  50. Rasmussen N, Petersen J, Remvig L, Andersen V (1990) Treatment of Wegener’s granulomatosis with trimethoprim-sulfamethoxazole. APMIS Suppl 19: 61–62

    PubMed  CAS  Google Scholar 

  51. 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

    Article  Google Scholar 

  52. 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

    Google Scholar 

  53. 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

    Article  PubMed  CAS  Google Scholar 

  54. X Rosenstiel P, Till A, Schreiber S (2007) NOD-like receptors and human diseases. Microbes Infect 9: 648–657

    Article  CAS  Google Scholar 

  55. Rusterholz D, Schlegel C (2000) Infectious aspects of Wegener’s granulomatosis. Schweiz Med Wochenschr Suppl 125: 41S–43S

    Google Scholar 

  56. 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

    Article  PubMed  CAS  Google Scholar 

  57. 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

    Google Scholar 

  58. 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

    Google Scholar 

  59. 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

    Google Scholar 

  60. 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

    Article  Google Scholar 

  61. 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

    Article  Google Scholar 

  62. Slavin RG (1992) Allergy is not a significant cause of nasal polyps. Arch Otolaryngol Head Neck Surg 118: 771

    PubMed  CAS  Google Scholar 

  63. 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

    Article  Google Scholar 

  64. 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

    PubMed  Google Scholar 

  65. Stone JH, Francis HW (2000) Immune-mediated inner ear disease. Curr Opin Rheumatol 12: 32–40

    Article  PubMed  CAS  Google Scholar 

  66. 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

    Article  PubMed  CAS  Google Scholar 

  67. 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

    Article  PubMed  CAS  Google Scholar 

  68. Tos M, Sasaki Y, Ohnishi M et al. (1992) Fireside conference 2. Pathogenesis of nasal polyps. Rhinol Suppl 14: 181–185

    PubMed  CAS  Google Scholar 

  69. 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

    Article  CAS  Google Scholar 

  70. 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

    Article  PubMed  Google Scholar 

  71. 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

    Google Scholar 

  72. 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

    Article  Google Scholar 

  73. 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

    Google Scholar 

  74. Waetzig GH, Schreiber S (2004) Mechanisms of infliximab: the reverse side of a drug effect. Inflamm Bowel Dis (Suppl 1) 10: S38–S43

    Article  Google Scholar 

  75. Wegener F (1939) Über eine eigenartige rhinogene Granulomatose mit besonderer Beteiligung des Arteriensystems und der Nieren. Beitr Pathol Anatom 102: 36–68

    Google Scholar 

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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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