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Churg-Strauss-Syndrom (eosinophile Granulomatose mit Polyangiitis, EGPA)

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Definition:
Idiopathische systemische Vaskulitis von kleinen und mittelgroßen Arterien, begleitet von schwerem Asthma und Eosinophilie.
Häufigkeit:
Inzidenz etwa 2–4:1.000.000 in USA und Westeuropa.
Symptome:
Asthma bronchiale als Erstsymptom, im Verlauf systemische Ausbreitung.
Befunde:
Stark variable Organmanifestationen, u. a. kutan, kardial und renal.
Diagnostik:
Nachweis von Asthma sowie Eosinophilie im Blutbild, zudem klinische Manifestationen der Vaskulitis, ggf. Biopsie.
Therapie:
Immunsuppression.
  • Schirmer JH, Aries PM, De Groot K, et al. Im Auftrag der Deutschen Gesellschaft für Rheumatologie (DGRh). S1-Leitlinie Diagnostik und Therapie der ANCA-assoziierten Vaskulitiden. Stand 2017. www.doi.org 
  1. 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. www.ncbi.nlm.nih.gov  
  2. Churg J, Strauss L. Allergic granulomatosis, allergic angiitis, and periarteritis nodosa. Am J Pathol 1951; 27: 277-301. PubMed  
  3. Noth I, Strek ME, Leff AR. Seminar: Churg-Strauss syndrome. The Lancet 2003; 361: 587-94. www.ncbi.nlm.nih.gov  
  4. Lowe ST. Eosinophilic Granulomatosis with Polyangiitis (Churg-Strauss Syndrome). Medscape, last updated Dec 24, 2018. emedicine.medscape.com  
  5. Moosig F, Bremer JP, Hellmich B, et al. A vasculitis centre based management strategy leads to improved outcome in eosinophilic granulomatosis and polyangiitis (Churg–Strauss, EGPA): monocentric experiences in 150 patients. Ann Rheum Dis 2013; 72(6): 1011-7. www.ncbi.nlm.nih.gov  
  6. Berti A, Cornec D, Crowson CS, et al. The Epidemiology of Antineutrophil Cytoplasmic Autoantibody-Associated Vasculitis in Olmsted County, Minnesota: A Twenty-Year US Population-Based Study. Arthritis Rheumatol 2017; 69(12): 2338-50. www.ncbi.nlm.nih.gov  
  7. Watts RA, Lane SE, Bentham G, Scott DG. Epidemiology of systemic vasculitis: a ten-year study in the United Kingdom. Arthritis Rheum 2000; 43: 414-19. PubMed  
  8. Sada KE, Amano K, Uehara R, et al. A nationwide survey on the epidemiology and clinical features of eosinophilic granulomatosis with polyangiitis (Churg-Strauss) in Japan. Mod Rheumatol 2014; 24(4): 640-4. www.ncbi.nlm.nih.gov  
  9. Vaglio A, Martorana D, Maggiore U, et al. HLA-DRB4 as a genetic risk factor for Churg-Strauss syndrome. Arthritis Rheum 2007; 56(9): 3159-66. www.ncbi.nlm.nih.gov  
  10. Wieczorek S, Hellmich B, Arning L, et al. Functionally relevant variations of the interleukin-10 gene associated with antineutrophil cytoplasmic antibody-negative Churg-Strauss syndrome, but not with Wegener's granulomatosis. Arthritis Rheum 2008; 58(6): 1839-48. www.ncbi.nlm.nih.gov  
  11. Wu EY, Hernandez ML, Jennette JC, et al. Eosinophilic Granulomatosis with Polyangiitis: Clinical Pathology Conference and Review. J Allergy Clin Immunol Pract 2018; 6(5): 1496-504. www.ncbi.nlm.nih.gov  
  12. Lilly CM, Churg A, Lazarovich M, et al. Asthma therapies and Churg-Strauss syndrome. J Allergy Clin Immunol 2002; 109(1): 1-19. www.ncbi.nlm.nih.gov  
  13. Vaglio A, Buzio C, Zwerina J. Eosinophilic granulomatosis with polyangiitis (Churg-Strauss): state of the art.. Allergy 2013; 68(3): 261-73. www.ncbi.nlm.nih.gov  
  14. 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. www.ncbi.nlm.nih.gov  
  15. Lanham JG, Elkon KB, Pusey CD, et al. Systemic vasculitis with asthma and eosinophilia: a clinical approach to the Churg-Strauss syndrome. Medicine (Baltimore) 1984; 63: 65-81. www.ncbi.nlm.nih.gov  
  16. Masi AT, Hunder GG, Lie JT, et al. The American College of Rheumatology 1990 criteria for the classification of Churg-Strauss syndrome (allergic granulomatosis and angiitis). Arthritis Rheum 1990; 33: 1094-100. PubMed  
  17. Cottin V, Bel E, Bottero P, et al. Respiratory manifestations of eosinophilic granulomatosis with polyangiitis (Churg-Strauss). Eur Respir J 2016; 48(5): 1429-41. www.ncbi.nlm.nih.gov  
  18. Guillevin L, Cohen P, Gayraud M, et al. Churg-Strauss syndrome: clinical study and long-term follow-up of 96 patients. Medicine 1999; 78: 26-37. PubMed  
  19. Schirmer JH, Aries PM, De Groot K, et al. S1-Leitlinie Diagnostik und Therapie der ANCA-assoziierten Vaskulitiden. Stand 2017. link.springer.com  
  20. Koike H, Sobue G. Clinicopathological features of neuropathy in anti-neutrophil cytoplasmic antibody-associated vasculitis. Clin Exp Nephrol 2013; 17(5): 683-5. www.ncbi.nlm.nih.gov  
  21. Hazebroek MR, Kemna MJ, Schalla S, et al. Prevalence and prognostic relevance of cardiac involvement in ANCA-associated vasculitis: eosinophilic granulomatosis with polyangiitis and granulomatosis with polyangiitis. Int J Cardiol 2015; 199: 170-9. www.ncbi.nlm.nih.gov  
  22. Hagen EC, Daha MR, Hermans J, et al. Diagnostic value of standardized assays for anti-neutrophil cytoplasmic antibodies in idiopathic systemic vasculitis. Kidney Int 1998; 53: 743-53. PubMed  
  23. Wechsler ME, Akuthota P, Jayne D, et al. Mepolizumab or Placebo for Eosinophilic Granulomatosis with Polyangiitis. NEJM 2017; 376: 1921-1932. www.nejm.org  
  24. Fredheim JM, Vedeler CA. Nevrologisk presentasjon av Churg-Strauss' syndrom. Tidsskr Nor Lægeforen nr. 8, 2003; 123: 1078-80. tidsskriftet.no  
  25. Hoffman G, Kerr GS, Leavitt RY, et al. Wegener granulomatosis: an analysis of 158 patients. Ann Intern Med 1992; 116: 488-98. www.ncbi.nlm.nih.gov  
  26. Aptekar RG, Atkinson JP, Deckler JL, Wolff S, Chu E. Bladder toxicity with chronic oral cyclophosphamide therapy in non-malignant disease. Arthritis Rheum 1973; 16: 461-67. PubMed  
  27. Plotz PH, Klippel JH, Deckler JL, et al. Bladder complications in patients receiving cyclophosphamide for systemic lupus erythematosus and rheumatoid arthritis. Ann Intern Med 1979; 91: 221-23. Annals of Internal Medicine  
  28. Fairchild WV, Spence CR, Solomon HD, Gangai M. The incidence of bladder cancer after cyclophosphamide therapy. J Urol 1979; 122: 163-64. PubMed  
  29. Ansell ID, Castro JE. Carcinoma of the bladder complicating cyclophosphamide therapy. Br J Urol 1975; 47: 413-18. PubMed  
  • Lino Witte, Dr. med., Arzt in Weiterbildung, Innere Medizin, Frankfurt