Skip to main content
SpringerLink
Log in

Asthma bronchiale und Churg-Strauss-Syndrom

Bronchial asthma and Churg-Strauss syndrome

  • Leitthema
  • Published:
Der Pneumologe Aims and scope

Zusammenfassung

Das Churg-Strauss-Syndrom (CSS; Synonym für eosinophile granulomatöse Polyangiitis) ist durch ein meist schweres Asthma bronchiale, eine Eosinophilie und eine systemische Gefäßentzündung, die zahlreiche unterschiedliche Organmanifestationen aufweisen kann, gekennzeichnet. Es stellt eine wichtige und therapierelevante Differenzialdiagnose des schweren Asthmas dar. Typisch ist ein phasenhafter Verlauf mit einer Prodromalphase, einer Phase der eosinophilen Gewebsinfiltration und einer vaskulitischen Phase. In Abhängigkeit von der klinischen Präsentation und dem Nachweis von antizytoplasmatischen Antikörpern (ANCA) werden zwei Phänotypen unterschieden. Das Asthma bronchiale beim CSS wird entsprechend der Standards der Asthmatherapie behandelt, in der vaskulitischen Phase ist in Abhängigkeit von der Schwere der Erkrankung und den Organmanifestationen oft eine kombinierte Therapie mit systemischen Kortikosteroiden und Immunsuppressiva erforderlich. Aktuelle Daten sprechen für einen positiven Effekt von Biologicals. Auf Grundlage einer aktuellen Literaturrecherche wird eine Übersicht über die Pathogenese, Klinik, Diagnostik und Therapie des CSS gegeben.

Abstract

Churg-Strauss syndrome (CSS), which is synonymous with eosinophilic granulomatosis with polyangiitis, is characterized by mostly severe bronchial asthma, eosinophilia and systemic vascular inflammation which can have many different forms of organ manifestation. It represents an important therapy-relevant differential diagnosis of severe asthma. Typical features are a course which runs in phases with a prodromal phase, a phase of eosinophilic tissue infiltration and a vasculitis phase. Depending on the clinical presentation and detection of antineutrophil cytoplasm antibodies (ANCA) two phenotypes can be distinguished. Bronchial asthma in CSS is treated according to the standards for asthma therapy and in the vasculitis phase a combined therapy with systemic corticosteroids and immunosuppressants is often necessary depending on the severity of the disease and organ manifestations. Current data suggest positive effects of biologicals. A review of the pathogenesis, clinical features, diagnostics and therapy of CSS is given based on a current literature search.

This is a preview of subscription content, log in via an institution to check access.

Access this article

Log in via an institution

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Institutional subscriptions

Abb. 1

Literatur

  1. Churg J, Strauss J (1951) Allergic granulomatosis, allergic angiitis, and periarteriitis nodosa. Am J Pathol 27:277–301

    PubMed  CAS  Google Scholar 

  2. Eustace JA, Nadasdy T, Choi M (1999) The Churg-Strauss-Syndrome. J Am Soc Nephrol 10:2048–2055

    PubMed  CAS  Google Scholar 

  3. Noth I, Strek ME, Leff AR (2003) Churg-Strauss syndrome. Lancet 15:587–594

    Article  Google Scholar 

  4. Martin RM, Wilton LV, Mann RD (1999) Prevalence of Churg-Strauss syndrome, vasculitis, eosinophilia and associated conditions: retrospective analysis of 58 prescription-event monitoring cohort studies. Pharmacoepidemiol Drug Saf 8:179–189

    Article  PubMed  CAS  Google Scholar 

  5. Perez Alamino R, Martínez C, Espinoza LR (2013) IgG4-associated vasculitis. Curr Rheumatol Rep 15:348–349

    Article  Google Scholar 

  6. Vaglio A, Strehl JD, Manger B et al (2012) IgG4 immune response in Churg-Strauss syndrome. Ann Rheum Dis 71:390–393

    Article  PubMed  CAS  Google Scholar 

  7. Vaglio A, Moosig F, Zwerina J (2012) Churg-Strauss syndrome: update on pathophysiology and treatment. Curr Opin Rheumatol 24:24–30

    Article  PubMed  CAS  Google Scholar 

  8. Vaglio A, Buzio C, Zwerina J (2013) Eosinophilic granulomatosis with polyangiitis (Churg-Strauss): state of the art. Allergy 68:261–273

    Article  PubMed  CAS  Google Scholar 

  9. Yamamoto M, Takahashi H, Suzuki C et al (2010) Analysis of serum IgG subclasses in Churg-Strauss syndrome–the meaning of elevated serum levels of IgG4. Intern Med 49:1365–1370

    Article  PubMed  Google Scholar 

  10. Szczeklik W, Jakieła B, Adamek D et al (2013) Cutting edge issues in the Churg-Strauss syndrome. Clin Rev Allergy Immunol 44:39–50

    Article  PubMed  CAS  Google Scholar 

  11. Bibby S, Healy B, Steele R et al (2010) Association between leukotriene receptor antagonist therapy and Churg-Strauss syndrome: an analysis of the FDA AERS database. Thorax 65:132–138

    Article  PubMed  CAS  Google Scholar 

  12. Wieczorek S, Holle JU, Epplen JT (2010) Recent progress in the genetics of Wegener’s granulomatosis and Churg-Strauss syndrome. Curr Opin Rheumatol 22:8–14

    Article  PubMed  CAS  Google Scholar 

  13. Lanham JG, Elkon KB, Pusey CD et al (1984) Systemic vasculitis with asthma and eosinophilia: a clinical approach to the Churg-Strauss Syndrome. Medicine (Baltimore) 63:65–81

  14. Zander DS, Farver CF (Hrsg) (2008) Pulmonary pathology. Churchill Livingstone Elsevier

  15. Sablé-Fourtassou R, Cohen P, Mahr A et al (2005) Antineutrophil cytoplasmic antibodies and the Churg-Strauss syndrome. Ann Intern Med 143:632–638

    Article  PubMed  Google Scholar 

  16. Sinico RA, Di Toma L, Maggiore U et al (2005) Prevalence and clinical significance of antineutrophil cytoplasmic antibodies in Churg-Strauss syndrome. Arthritis Rheum 52:2926–2935

    Article  PubMed  CAS  Google Scholar 

  17. Steinfeld S, Golstein M, De Vuyst P (1994) Chronic Eosinophilic Pneumonia (CEP) as a presenting feature of Churg-Strauss Syndrome (CSS). Eur Respir J 7:2098

    PubMed  CAS  Google Scholar 

  18. Szczeklik W, Sokolowska BM, Zuk J et al (2011) The course of asthma in Churg-Strauss-Syndrome. J Asthma 48:183–187

    Article  PubMed  Google Scholar 

  19. Cottin V, Khouatra C, Dubost R et al (2009) Persistent airflow obstruction in asthma of patients with Churg-Strauss syndrome and long-term follow-up. Allergy 64:589–595

    Article  PubMed  CAS  Google Scholar 

  20. Lanham JG, Elkon KB, Pusey CD et al (1984) Systemic vasculitis with asthma and eosinophilia: a clinical approach to the Churg-Strauss syndrome. Medicine (Baltimore) 63:65–81

    Google Scholar 

  21. Chumbley LC, Harrison EG Jr, DeRemee RA (1977) Allergic granulomatosis and angiitis (Churg-Strauss syndrome). Report and analysis of 30 cases. Mayo Clin Proc 52:477–484

    PubMed  CAS  Google Scholar 

  22. Tsurikisawa N, Tsuburai T, Saito H et al (2007) A retrospective study of bronchial hyperresponsiveness in patients with asthma before the onset of Churg-Strauss syndrome. Allergy Asthma Proc 28:336–343

    Article  PubMed  Google Scholar 

  23. Bottero P, Bonini M, Vecchio F et al (2007) The common allergens in the Churg-Strauss syndrome. Allergy 62:1288–1294

    Article  PubMed  CAS  Google Scholar 

  24. Szczeklik W, Mastalerz L, Sokolowska B et al (2008) Aspirin tolerance and leukotriene biosynthesis in Churg-Strauss syndrome. Allergy 63:949–950

    Article  PubMed  CAS  Google Scholar 

  25. Whyte AF, Smith WB, Sinkar SN et al (2013) Clinical and laboratory characteristics of 19 patients with Churg-Strauss syndrome from a single South Australian centre. Intern Med J 43:784–790

    Article  PubMed  CAS  Google Scholar 

  26. Livi V, Cancellieri A, Patelli M et al (2012) Tracheobronchial involvement in Churg-Strauss syndrome. J Bronchology Interv Pulmonol 19:81–82

    Article  PubMed  Google Scholar 

  27. Hara Y, Kanoh S, Shinkai M et al (2012) Churg-Strauss syndrome with endobronchial eosinophilic vasculitis. Intern Med 51:3227

    Article  PubMed  Google Scholar 

  28. Vinit J, Bielefeld P, Muller G et al (2010) Heart involvement in Churg-Strauss syndrome: retrospective study in French Burgundy population in past 10 years. Eur J Intern Med 21:341–346

    Article  PubMed  CAS  Google Scholar 

  29. Mavrogeni S, Karabela G, Gialafos E et al (2013) Cardiac involvement in ANCA (+) and ANCA (-) Churg-Strauss Syndrome evaluated by cardiovascular magnetic resonance. Inflamm Allergy Drug Targets 12:322–327

    Article  PubMed  CAS  Google Scholar 

  30. Marmursztejn J, Guillevin L, Trebossen R et al (2013) Churg-Strauss syndrome cardiac involvement evaluated by cardiac magnetic resonance imaging and positron-emission tomography: a prospective study on 20 patients. Rheumatology (Oxford) 52:642–650

    Google Scholar 

  31. Samson M, Puéchal X, Devilliers H et al (2013) Long-term outcomes of 118 patients with eosinophilic granulomatosis with polyangiitis (Churg-Strauss syndrome) enrolled in two prospective trials. J Autoimmun 43:60–69

    Article  PubMed  Google Scholar 

  32. Comarmond C, Pagnoux C, Khellaf M et al (2013) 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 65:270–281

    Article  PubMed  Google Scholar 

  33. Cohen P, Pagnoux C, Mahr A et al (2007) Churg-Strauss syndrome with poor-prognosis factors: a prospective multicenter trial comparing glucocorticoids and six or twelve cyclophosphamide pulses in forty-eight patients. Arthritis Rheum 57:686–693

    Article  PubMed  CAS  Google Scholar 

  34. Clain JM, Specks U (2013) Rituximab for ANCA-associated vasculitis: the experience in the United States. Presse Med 42:530–532

    Article  PubMed  Google Scholar 

  35. Josselin-Mahr L, Werbrouck-Chiraux A, Garderet L et al (2013) Efficacy of imatinib mesylate in a case of Churg-Strauss syndrome: evidence forthe pathogenic role of a tyrosine kinase? Rheumatology (Oxford) 27 (Epub ahead of print)

  36. Ballanti E, Di Muzio G, Novelli L et al (2012) Churg-Strauss syndrome with neurologic manifestations: successful treatment with intravenous immunoglobulins. Isr Med Assoc J 14:583–585

    PubMed  Google Scholar 

  37. Kahn JE, Grandpeix-Guyodo C, Marroun I et al (2010) Sustained response to mepolizumab in refractory Churg-Strauss syndrome. J Allergy Clin Immunol 125:267–270

    Article  PubMed  CAS  Google Scholar 

  38. Kim S, Marigowda G, Oren E et al (2010) Mepolizumab as a steroid-sparing treatment option in patients with Churg-Strauss syndrome. J Allergy Clin Immunol 125:1336–1343

    Article  PubMed  CAS  Google Scholar 

Download references

Einhaltung ethischer Richtlinien

Interessenkonflikt. J. Schreiber gibt an, dass kein Interessenkonflikt besteht. Der Beitrag beinhaltet keine Studien an Menschen oder Tieren.

Author information

Authors and Affiliations

  1. Abteilung für Pneumologie, Universitätsklinikum Magdeburg, Leipziger Str. 44, 39120, Magdeburg, Deutschland

    J. Schreiber

Authors
  1. J. Schreiber
    View author publications

    You can also search for this author in PubMed Google Scholar

Corresponding author

Correspondence to J. Schreiber.

Rights and permissions

Reprints and permissions

About this article

Cite this article

Schreiber, J. Asthma bronchiale und Churg-Strauss-Syndrom. Pneumologe 10, 418–425 (2013). https://doi.org/10.1007/s10405-013-0710-7

Download citation

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s10405-013-0710-7

Schlüsselwörter

Keywords

Search

Navigation