Abstract
Churg-Strauss syndrome is an uncommon disease of unknown cause described initially by Churg and Strauss in 1951. Even though it was initially thought to be a variant of polyarteritis nodosa, its pathological, clinical, and laboratory features show that it is related to the small vessel vasculitides, and it is now classified as an antineutrophil cytoplasmic antibody–associated vasculitis. The presence of asthma, usually of adult onset, along with other allergic symptoms, peripheral and tissue eosinophilia, is specific to this disease. These features usually help clinicians distinguish it from other types of small vessel vasculitis and should alert clinicians about its presence. Two different clinical subtypes defined by the presence of antineutrophil cytoplasmic antibodies recently have been recognized. Recent advances in the treatment and pathophysiology of Churg-Strauss syndrome are reviewed in this article.
Similar content being viewed by others
References
Papers of particular interest, published recently, have been highlighted as: • Of importance •• Of major importance
Falk RJ et al. Granulomatosis with polyangiitis (Wegener’s): an alternative name for Wegener’s granulomatosis. Arthritis Rheum. 2011;63(4):863–4.
Lie JT. Illustrated histopathologic classification criteria for selected vasculitis syndromes. American College of Rheumatology Subcommittee on Classification of Vasculitis. Arthritis Rheum. 1990;33(8):1074–87.
Churg J, Strauss L. Allergic granulomatosis, allergic angiitis, and periarteritis nodosa. Am J Pathol. 1951;27(2):277–301.
Rose GA, Spencer H. Polyarteritis nodosa. Q J Med. 1957;26(101):43–81.
• Baldini, C., et al., Clinical manifestations and treatment of Churg-Strauss syndrome. Rheum Dis Clin North Am, 2010. 36(3): p. 527–43. This is an excellent recent review of CSS, with emphasis on clinical and therapeutic issues.
Kahn JE, Bletry O, Guillevin L. Hypereosinophilic syndromes. Best Pract Res Clin Rheumatol. 2008;22(5):863–82.
Sinico RA, Bottero P. Churg-Strauss angiitis. Best Pract Res Clin Rheumatol. 2009;23(3):355–66.
Abril A, Calamia KT, Cohen MD. The Churg Strauss syndrome (allergic granulomatous angiitis): review and update. Semin Arthritis Rheum. 2003;33(2):106–14.
Mahr A et al. 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. 2004;51(1):92–9.
• Bochner, B.S. and G.J. Gleich, What targeting eosinophils has taught us about their role in diseases. J Allergy Clin Immunol, 2010. 126(1): p. 16–25; quiz 26–7. This is a basic review on eosinophils and their role in the pathogenesis of related conditions written in language that should be easy to understand for clinicians with minimal basic science background.
Akuthota P, Wang H, Weller PF. Eosinophils as antigen-presenting cells in allergic upper airway disease. Curr Opin Allergy Clin Immunol. 2010;10(1):14–9.
Kouro T, Takatsu K. IL-5- and eosinophil-mediated inflammation: from discovery to therapy. Int Immunol. 2009;21(12):1303–9.
Weller PF. Human eosinophils. J Allergy Clin Immunol. 1997;100(3):283–7.
Schmitt WH et al. Churg-Strauss syndrome: serum markers of lymphocyte activation and endothelial damage. Arthritis Rheum. 1998;41(3):445–52.
Hellmich B, Csernok E, Gross WL. Proinflammatory cytokines and autoimmunity in Churg-Strauss syndrome. Ann N Y Acad Sci. 2005;1051:121–31.
• Terrier, B., et al., Interleukin-25: a cytokine linking eosinophils and adaptive immunity in Churg-Strauss syndrome. Blood, 2010. 116(22): p. 4523–31. This is a very interesting paper explaining a new potential immunologic mechanism of eosinophil activation in CSS.
Dallos T et al. CCL17/thymus and activation-related chemokine in Churg-Strauss syndrome. Arthritis Rheum. 2010;62(11):3496–503.
Chen M, Kallenberg CG. New advances in the pathogenesis of ANCA-associated vasculitides. Clin Exp Rheumatol. 2009;27(1 Suppl 52):S108–14.
Xiao H et al. Antineutrophil cytoplasmic autoantibodies specific for myeloperoxidase cause glomerulonephritis and vasculitis in mice. J Clin Invest. 2002;110(7):955–63.
Sable-Fourtassou R et al. Antineutrophil cytoplasmic antibodies and the Churg-Strauss syndrome. Ann Intern Med. 2005;143(9):632–8.
Sinico RA et al. Prevalence and clinical significance of antineutrophil cytoplasmic antibodies in Churg-Strauss syndrome. Arthritis Rheum. 2005;52(9):2926–35.
Keogh KA, Specks U. Churg-Strauss syndrome: clinical presentation, antineutrophil cytoplasmic antibodies, and leukotriene receptor antagonists. Am J Med. 2003;115(4):284–90.
Kallenberg CG. Churg-Strauss syndrome: just one disease entity? Arthritis Rheum. 2005;52(9):2589–93.
Lane SE et al. Are environmental factors important in primary systemic vasculitis? A case-control study. Arthritis Rheum. 2003;48(3):814–23.
Cuchacovich R, Justiniano M, Espinoza LR. Churg-Strauss syndrome associated with leukotriene receptor antagonists (LTRA). Clin Rheumatol. 2007;26(10):1769–71.
Wechsler ME et al. Churg-Strauss syndrome in patients treated with omalizumab. Chest. 2009;136(2):507–18.
Holle JU, Wieczorek S, Gross WL. Genetic association studies in ANCA-associated vasculitides: what we have learnt so far and what needs to be done in the future. Clin Exp Rheumatol. 2010;28(1 Suppl 57):5–7.
Wieczorek S, Holle JU, Epplen JT. Recent progress in the genetics of Wegener’s granulomatosis and Churg-Strauss syndrome. Curr Opin Rheumatol. 2010;22(1):8–14.
Vaglio A et al. HLA-DRB4 as a genetic risk factor for Churg-Strauss syndrome. Arthritis Rheum. 2007;56(9):3159–66.
Wieczorek S et al. Associations of Churg-Strauss syndrome with the HLA-DRB1 locus, and relationship to the genetics of antineutrophil cytoplasmic antibody-associated vasculitides: comment on the article by Vaglio et al. Arthritis Rheum. 2008;58(1):329–30.
Lanham JG et al. Systemic vasculitis with asthma and eosinophilia: a clinical approach to the Churg-Strauss syndrome. Medicine (Baltimore). 1984;63(2):65–81.
Lhote F, Guillevin L. Polyarteritis nodosa, microscopic polyangiitis, and Churg-Strauss syndrome. Clinical aspects and treatment. Rheum Dis Clin North Am. 1995;21(4):911–47.
Papadimitraki ED et al. Ear-nose-throat manifestations of autoimmune rheumatic diseases. Clin Exp Rheumatol. 2004;22(4):485–94.
Chumbley LC, Harrison Jr EG, DeRemee RA. Allergic granulomatosis and angiitis (Churg-Strauss syndrome). Report and analysis of 30 cases. Mayo Clin Proc. 1977;52(8):477–84.
Erzurum SC et al. Pleural effusion in Churg-Strauss syndrome. Chest. 1989;95(6):1357–9.
Masi AT et al. The American College of Rheumatology 1990 criteria for the classification of Churg-Strauss syndrome (allergic granulomatosis and angiitis). Arthritis Rheum. 1990;33(8):1094–100.
Guillevin L et al. Churg-Strauss syndrome. Clinical study and long-term follow-up of 96 patients. Medicine (Baltimore). 1999;78(1):26–37.
Davis MD et al. Cutaneous manifestations of Churg-Strauss syndrome: a clinicopathologic correlation. J Am Acad Dermatol. 1997;37(2 Pt 1):199–203.
Rashtak S, Pittelkow MR. Skin involvement in systemic autoimmune diseases. Curr Dir Autoimmun. 2008;10:344–58.
Finan MC, Winkelmann RK. The cutaneous extravascular necrotizing granuloma (Churg-Strauss granuloma) and systemic disease: a review of 27 cases. Medicine (Baltimore). 1983;62(3):142–58.
• Dennert, R.M., et al., Cardiac involvement in Churg-Strauss syndrome. Arthritis Rheum, 2010. 62(2): p. 627–34. This article is an excellent review of cardiac issues in CSS, the main cause of mortality in these patients. Thus, this is something of which clinicians should be aware.
Neumann T et al. Cardiac involvement in Churg-Strauss syndrome: impact of endomyocarditis. Medicine (Baltimore). 2009;88(4):236–43.
Pagnoux C et al. Presentation and outcome of gastrointestinal involvement in systemic necrotizing vasculitides: analysis of 62 patients with polyarteritis nodosa, microscopic polyangiitis, Wegener granulomatosis, Churg-Strauss syndrome, or rheumatoid arthritis-associated vasculitis. Medicine (Baltimore). 2005;84(2):115–28.
Sharma MC, Safaya R, Sidhu BS. Perforation of small intestine caused by Churg-Strauss syndrome. J Clin Gastroenterol. 1996;23(3):232–5.
Sinico RA et al. Renal involvement in Churg-Strauss syndrome. Am J Kidney Dis. 2006;47(5):770–9.
Hall FC, Walport MJ. Hypereosinophilic syndromes: association with vasculitis, fibrosis and autoimmunity. Clin Exp Allergy. 1993;23(7):542–7.
• Pagnoux, C. and L. Guillevin, Churg-Strauss syndrome: evidence for disease subtypes? Curr Opin Rheumatol, 2010. 22(1): p. 21–8. This is another excellent clinical review from the French Vasculitis Group, probably the group with the most cumulative experience in CSS and vasculitis in general. This touches on the provocative subject of two different clinical subtypes of this condition.
Mukhtyar C et al. Outcomes from studies of antineutrophil cytoplasm antibody associated vasculitis: a systematic review by the European League Against Rheumatism Systemic Vasculitis Task Force. Ann Rheum Dis. 2008;67(7):1004–10.
Ribi C et al. Treatment of Churg-Strauss syndrome without poor-prognosis factors: a multicenter, prospective, randomized, open-label study of seventy-two patients. Arthritis Rheum. 2008;58(2):586–94.
Tatsis E, Schnabel A, Gross WL. Interferon-alpha treatment of four patients with the Churg-Strauss syndrome. Ann Intern Med. 1998;129(5):370–4.
•• Stone, J.H., et al., Rituximab versus cyclophosphamide for ANCA-associated vasculitis. N Engl J Med, 2010. 363(3): p. 221–32. This was a landmark study on rituximab for ANCA-associated vasculits, which is becoming an extremely important therapeutic tool in this group of diseases.
Kim S et al. Mepolizumab as a steroid-sparing treatment option in patients with Churg-Strauss syndrome. J Allergy Clin Immunol. 2010;125(6):1336–43.
Disclosure
No potential conflicts of interest relevant to this article were reported.
Author information
Authors and Affiliations
Corresponding author
Rights and permissions
About this article
Cite this article
Abril, A. Churg-Strauss Syndrome: An Update. Curr Rheumatol Rep 13, 489–495 (2011). https://doi.org/10.1007/s11926-011-0205-7
Published:
Issue Date:
DOI: https://doi.org/10.1007/s11926-011-0205-7