Skip to main content

Advertisement

SpringerLink
Log in

Effect of delayed diagnosis on disease course and management of Churg–Strauss syndrome: a retrospective study

  • Original Article
  • Published:
Clinical Rheumatology Aims and scope Submit manuscript

Abstract

Delayed diagnosis in patients with Churg–Strauss syndrome (CSS) is largely attributed to the variable and nonspecific presentation of the disease’s initial symptoms. The aim of the study was to evaluate the effect of delayed diagnosis on the course of CSS. We conducted a retrospective study of 30 CSS patients followed up in our department. In each patient, we assessed the delay in CSS diagnosis (the time when patients already fulfilled four out of six of the American College of Rheumatology criteria and the diagnosis was not yet established), the disease activity at the time of diagnosis, and organ involvement during CSS course. A median value of 2 weeks was chosen as the cutoff point after which the diagnosis was considered as delayed. Sixteen patients were diagnosed before (group 1) and 14 patients after this cutoff point (group 2). In group 2, we found a higher Birmingham Vasculitis Activity Score at the moment of diagnosis (20.4 vs 25.1, p < 0.05) and a more severe disease course, resulting in more frequent hospitalization rates (0.64 vs 2.26/year, p < 0.00001), higher corticosteroids dose requirements (5.87 vs 11.57 mg/day converted to methylprednisolone, p < 0.0001), and additional immunosuppressive therapy administration (56.2 vs 92.8 %, p < 0.05) to maintain disease remission. All six perinuclear pattern of antineutrophil cytoplasmic antibobodies (pANCA)-positive patients (20 %) were found in group 1. Concluding, the delay in diagnosis of CSS of more than 2 weeks was found to be associated with a disease course that was more severe. The presence of the pANCA antibodies may occasionally facilitate establishment of the diagnosis.

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

Similar content being viewed by others

References

  1. Szczeklik W, Jakieła B, Adamek D, Musiał J (2011) Cutting edge issues in the Churg–Strauss syndrome. Clin Rev Allergy Immunol. doi:10.1007/s12016-011-8266-y

  2. Churg J, Strauss L (1951) Allergic granulomatosis, allergic angiitis, and periarteritis nodosa. Am J Pathol 27(2):277–301

    Google Scholar 

  3. Szczeklik W, Sokołowska B, Mastalerz L, Grzanka P, Górka J, Pacułt K, Miszalski-Jamka T, Soja J, Musiał J (2010) Pulmonary findings in Churg–Strauss syndrome in chest X-rays and high resolution computed tomography at the time of initial diagnosis. Clin Rheumatol 29(10):1127–1134

    Article  PubMed  Google Scholar 

  4. Szczeklik W, Miszalski-Jamka T, Mastalerz L, Sokolowska B, Dropinski J, Banys R, Hor KN, Mazur W, Musial J (2011) Multimodality assessment of cardiac involvement in Churg–Strauss syndrome patients in clinical remission. Circ J 75(3):649–655

    Article  PubMed  Google Scholar 

  5. Dunogué B, Pagnoux C, Guillevin L (2011) Churg–Strauss syndrome: clinical symptoms, complementary investigations, prognosis and outcome, and treatment. Semin Respir Crit Care Med 32(3):298–309. doi:10.1055/s-0031-1279826

    Article  PubMed  Google Scholar 

  6. Jakiela B, Szczeklik W, Plutecka H, Sokolowska B, Mastalerz L, Sanak M, Bazan-Socha S, Szczeklik A, Musial J (2012) Increased production of IL-5 and dominant Th2-type response in airways of Churg–Strauss syndrome patients. Rheumatology (Oxford) 51(10):1887–1893

    Article  CAS  Google Scholar 

  7. Masi AT, Hunder GG, Lie JT, Michel BA, Bloch DA, Arend WP, Calabrese LH, Edworthy SM, Fauci AS, Leavitt RY et al (1990) The American College of Rheumatology 1990 criteria for the classification of Churg–Strauss syndrome (allergic granulomatosis and angiitis). Arthritis Rheum 33(8):1094–1100

    Article  PubMed  CAS  Google Scholar 

  8. Mukhtyar C, Lee R, Brown D, Carruthers D, Dasgupta B, Dubey S, Flossmann O, Hall C, Hollywood J, Jayne D, Jones R, Lanyon P, Muir A, Scott D, Young L, Luqmani RA (2009) Modification and validation of the Birmingham Vasculitis Activity Score (version 3). Ann Rheum Dis 68:1827–1832

    Article  PubMed  CAS  Google Scholar 

  9. Guillevin L, Pagnoux C, Seror R, Mahr A, Mouthon L, Le Toumelin P, French Vasculitis Study Group (FVSG) (2011) The Five-Factor Score revisited: assessment of prognoses of systemic necrotizing vasculitides based on the French Vasculitis Study Group (FVSG) cohort. Medicine (Baltimore) 90(1):19–27

    Article  Google Scholar 

  10. Helbig G, Kyrcz-Krzemien S (2011) Diagnostic and therapeutic management in patients with hypereosinophilic syndromes. Pol Arch Med Wewn 121(1–2):44–52

    PubMed  Google Scholar 

  11. Szczeklik W, Sokolowska BM, Zuk J, Mastalerz L, Szczeklik A, Musial J (2011) The course of asthma in Churg–Strauss syndrome. J Asthma 48(2):183–187

    Article  PubMed  Google Scholar 

  12. Szczeklik W, Sanak M, Mastalerz L, Sokołowska BM, Gielicz A, Soja J, Kumik J, Musiał J, Szczeklik A (2012) 12-Hydroxy-eicosatetraenoic acid (12-HETE): a biomarker of Churg–Strauss syndrome. Clin Exp Allergy 42:513–522

    Google Scholar 

  13. Bourgarit A, Le Toumelin P, Pagnoux C, Cohen P, Mahr A, Le Guern V, Mouthon L, Guillevin L; French Vasculitis Study Group (2005) Deaths occurring during the first year after treatment onset for polyarteritis nodosa, microscopic polyangiitis, and Churg–Strauss syndrome: a retrospective analysis of causes and factors predictive of mortality based on 595 patients. Medicine (Baltimore) 84(5):323–330

    Article  Google Scholar 

  14. Nazareth R, Mason JC (2011) Takayasu arteritis: severe consequences of delayed diagnosis. QJM 104(9):797–800

    Article  PubMed  CAS  Google Scholar 

  15. Sittiwangkul R, Pongprot Y, Silvilairat S, Phornphutkul C (2011) Delayed diagnosis of Kawasaki disease: risk factors and outcome of treatment. Ann Trop Paediatr 31(2):109–114

    Article  PubMed  CAS  Google Scholar 

  16. Minich LL, Sleeper LA, Atz AM, McCrindle BW, Lu M, Colan SD, Printz BF, Klein GL, Sundel RP, Takahashi M, Li JS, Vetter VL, Newburger JW, Investigators PHN (2007) Delayed diagnosis of Kawasaki disease: what are the risk factors? Pediatrics 120(6):e1434–e1440

    Article  PubMed  Google Scholar 

  17. Ezeonyeji AN, Borg FA, Dasgupta B (2011) Delays in recognition and management of giant cell arteritis: results from a retrospective audit. Clin Rheumatol 30(2):259–262

    Article  PubMed  Google Scholar 

  18. Ribi C, Cohen P, Pagnoux C, Mahr A, Arène JP, Lauque D, Puéchal X, Letellier P, Delaval P, Cordier JF, Guillevin L; French Vasculitis Study Group (2008) Treatment of Churg–Strauss syndrome without poor-prognosis factors: a multicenter, prospective, randomized, open-label study of seventy-two patients. Arthritis Rheum 58(2):586–594

    Article  Google Scholar 

  19. Guillevin L, Cohen P, Gayraud M, Lhote F, Jarrousse B, Casassus P (1999) Churg–Strauss syndrome. Clinical study and long-term follow-up of 96 patients. Medicine (Baltimore) 78:26.

    Google Scholar 

  20. Keogh KA, Specks U (2003) Churg–Strauss syndrome: clinical presentation, antineutrophil cytoplasmic antibodies, and leukotriene receptor antagonists. Am J Med 115(4):284–290

    Article  PubMed  CAS  Google Scholar 

  21. Lanham JG, Elkon KB, Pusey CD, Hughes GR (1984) Systemic vasculitis with asthma and eosinophilia: a clinical approach to the Churg–Strauss syndrome. Medicine (Baltimore) 63(2):65–81

    CAS  Google Scholar 

  22. Sinico RA, Bottero P (2009) Churg–Strauss angiitis. Best Pract Res Clin Rheumatol 23(3):355–366

    Article  PubMed  Google Scholar 

  23. Pagnoux C, Guilpain P, Guillevin L (2007) Churg–Strauss syndrome. Curr Opin Rheumatol 19(1):25–32

    Article  PubMed  Google Scholar 

  24. Guillevin L, Lhote F, Gayraud M, Cohen P, Jarrousse B, Lortholary O, Thibult N, Casassus P (1996) Prognostic factors in polyarteritis nodosa and Churg–Strauss syndrome. A prospective study in 342 patients. Medicine (Baltimore) 75(1):17–28

    Article  CAS  Google Scholar 

  25. Danieli MG, Cappelli M, Malcangi G, Logullo F, Salvi A, Danieli G (2004) Long term effectiveness of intravenous immunoglobulin in Churg–Strauss syndrome. Ann Rheum Dis 63(12):1649–1654

    Google Scholar 

Download references

Disclosures

None.

Author information

Authors and Affiliations

  1. Department of Medicine, Jagiellonian University Medical College, ul. Skawińska 8, 31-066, Krakow, Poland

    Barbara Sokołowska, Wojciech Szczeklik, Lucyna Mastalerz, Paweł Kuczia, Michał Wodkowski, Edyta Stodółkiewicz, Karolina Macioł & Jacek Musiał

Authors
  1. Barbara Sokołowska
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. Wojciech Szczeklik
    View author publications

    You can also search for this author in PubMed Google Scholar

  3. Lucyna Mastalerz
    View author publications

    You can also search for this author in PubMed Google Scholar

  4. Paweł Kuczia
    View author publications

    You can also search for this author in PubMed Google Scholar

  5. Michał Wodkowski
    View author publications

    You can also search for this author in PubMed Google Scholar

  6. Edyta Stodółkiewicz
    View author publications

    You can also search for this author in PubMed Google Scholar

  7. Karolina Macioł
    View author publications

    You can also search for this author in PubMed Google Scholar

  8. Jacek Musiał
    View author publications

    You can also search for this author in PubMed Google Scholar

Corresponding author

Correspondence to Barbara Sokołowska.

Rights and permissions

Reprints and permissions

About this article

Cite this article

Sokołowska, B., Szczeklik, W., Mastalerz, L. et al. Effect of delayed diagnosis on disease course and management of Churg–Strauss syndrome: a retrospective study. Clin Rheumatol 32, 349–354 (2013). https://doi.org/10.1007/s10067-012-2127-9

Download citation

  • Received:

  • Revised:

  • Accepted:

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s10067-012-2127-9

Keywords

Search

Navigation