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Eosinofil granulomatose med polyangiitt (Churg-Strauss)

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Definisjon:
Idiopatisk diffus vaskulitt i små og middels store arterier ledsaget av alvorlig astma og eosinofili
Forekomst:
Sjelden tilstand
Symptomer:
Hud og lunger er hyppigst involvert, men også hjerte, GI-traktus, lever, nyrer, perifere nerver og andre organsystemer kan bli affisert
Funn:
Det kan være kliniske funn fra alle overnevnte organer
Diagnostikk:
Laboratorieprøver viser vesentlig eosinofili, ofte økt SR og CRP, ANCA pos og RF pos i mange tilfeller. En rekke bildeundersøkelser kan være relevante
Behandling:
Ved lokalisert sykdom er behandling med kortikosteroider ofte tilstrekkelig for å oppnå full remisjon. Ved mer avansert sykdom behandles med cyklofosfamid
  1. King TE. Clinical features and diagnosis of eosinophilic granulomatosis with polyangiitis (Churg-Strauss). UpToDate, last updated Feb 15, 2018. UpToDate  
  2. Groh M, Pagnoux C, Baldini C, et al. Eosinophilic granulomatosis with polyangiitis (Churg-Strauss) (EGPA) Consensus Task Force recommendations for evaluation and management. Eur J Intern Med. 2015;26:545-553. PubMed  
  3. Sinico RA, Bottero P. Churg-Strauss angiitis. Best Pract Res Clin Rheumatol 2009; 23:355. PubMed  
  4. Ntatsaki E, Carruthers D, Chakravarty K, et al; BSR and BHPR Standards, Guidelines and Audit Working Group. BSR and BHPR guideline for the management of adults with ANCA-associated vasculitis. Rheumatology (Oxford). 2014;53:2306-2309. PubMed  
  5. Bibby S, Healy B, Steele R, et al. Association between leukotriene receptor antagonist therapy and Churg-Strauss syndrome: an analysis of the FDA AERS database. Thorax 2010; 65:132. PubMed  
  6. Pagnoux C, Guillevin L. Churg-Strauss syndrome: evidence for disease subtypes? Curr Opin Rheumatol 2010; 22:21. PubMed  
  7. Comarmond C, Pagnoux C, Khellaf M, et al. Eosinophilic granulomatosis with polyangiitis (Churg-Strauss): clinical characteristics and long-term followup of the 383 patients enrolled in the French Vasculitis Study Group cohort. Arthritis Rheum 2013; 65:270. PubMed  
  8. Churg J, Strauss L. Allergic granulomatosis, allergic angiitis, and periarteritis nodosa. Am J Pathol 1951; 27: 277-301. PubMed  
  9. 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  
  10. Cottin V, Bel E, Bottero P, et al. Revisiting the systemic vasculitis in eosinophilic granulomatosis with polyangiitis (Churg-Strauss): A study of 157 patients by the Groupe d'Etudes et de Recherche sur les Maladies Orphelines Pulmonaires and the European Respiratory Society Taskforce on eosinophilic granulomatosis with polyangiitis (Churg-Strauss). Autoimmun Rev 2017; 16:1. PubMed  
  11. King TE. Treatment and prognosis of eosinophilic granulomatosis with polyangiitis (Churg-Strauss). UpToDate, last updated Jun 23, 2020. UpToDate  
  12. Bel EH, Wenzel SE, Thompson PJ, et al. Oral Glucocorticoid-Sparing Effect of Mepolizumab in Eosinophilic Asthma. N Engl J Med 2014; 371: 1189-97. doi:10.1056/NEJMoa1403291 DOI  
  13. Ortega HG, Liu MC, Pavord ID, et al. Mepolizumab Treatment in Patients with Severe Eosinophilic Asthma. N Engl J Med 2014; 371: 1198-1207. doi:10.1056/NEJMoa1403290 DOI  
  • Terje Johannessen, professor i allmennmedisin, Trondheim

Tidligere fagmedarbeidere

  • Bjørnar Grenne, PhD, konst. overlege, Klinikk for hjertemedisin, St. Olavs Hospital, Trondheim
  • Knut Aasarød, dr. med., overlege, Nyreavdelingen, St. Olavs Hospital, Trondheim