Close
New

Medscape is available in 5 Language Editions – Choose your Edition here.

 

Eosinophilic Granulomatosis with Polyangiitis (Churg-Strauss Syndrome)

  • Author: Spencer T Lowe, MD; Chief Editor: Herbert S Diamond, MD  more...
 
Updated: Jul 05, 2016
 

Background

Eosinophilic granulomatosis with polyangiitis (EGPA)—or, as it was traditionally termed, Churg-Strauss syndrome—is a rare systemic necrotizing vasculitis that affects small-to-medium-sized vessels and is associated with severe asthma and blood and tissue eosinophilia.[1] Like granulomatosis with polyangiitis (Wegener granulomatosis), and the microscopic form of periarteritis (ie, microscopic polyangiitis), EGPA is an antineutrophil cytoplasmic antibody (ANCA)–associated vasculitide.[2, 3, 4, 5]

In 1951, Churg and Strauss first described the syndrome in 13 patients who had asthma, eosinophilia, granulomatous inflammation, necrotizing systemic vasculitis, and necrotizing glomerulonephritis.[3] In 1990, the American College of Rheumatology (ACR) proposed the following six criteria for the diagnosis of Churg-Strauss syndrome[6] :

  • Asthma (wheezing, expiratory rhonchi)
  • Eosinophilia of more than 10% in peripheral blood
  • Paranasal sinusitis
  • Pulmonary infiltrates (may be transient)
  • Histological proof of vasculitis with extravascular eosinophils
  • Mononeuritis multiplex or polyneuropathy

The presence of four or more criteria yields a sensitivity of 85% and a specificity of 99.7%. The 1994 Chapel Hill consensus conference on the classification of vasculitides did not modify the ACR criteria.[7]

The image below depicts transient pulmonary infiltrates.

Transient pulmonary infiltrates in a patient with Transient pulmonary infiltrates in a patient with Churg-Strauss syndrome (CSS).

See Vasculitis: Case Presentations, a Critical Images slideshow, for more information on clinical, histologic and radiographic imaging findings in various forms of vasculitis.

Next

Pathophysiology

Churg-Strauss syndrome is a granulomatous small-vessel vasculitis. The cause of this allergic angiitis and granulomatosis is unknown.[8] No data have been reported regarding the role of immune complexes or cell-mediated mechanisms in this disease, although autoimmunity is evident with the presence of hypergammaglobulinemia, increased levels of immunoglobulin E (IgE), rheumatoid factor, and ANCA.

A Churg-Strauss syndrome–like syndrome develops as a rare complication in people with asthma who are steroid-dependent and who are treated with leukotriene receptor antagonists (eg, montelukast, zafirlukast) upon reduction in their oral steroid dose.[9] The Churg-Strauss syndrome–like complication is reported in people whose withdrawal of oral steroids is also facilitated by inhaled steroids. This complication is probably related to steroid withdrawal, which unmasks underlying Churg-Strauss syndrome,[10, 11, 12] rather than to the drugs themselves. However, in rare cases, this syndrome has developed when a leukotriene receptor antagonist has been substituted for inhaled steroids without a history of oral steroid withdrawal.[13]

HLA-DRB4 positivity may be a genetic risk factor for the development of Churg-Strauss syndrome and may increase the likelihood of vasculitic manifestations of the disease.[14]

Previous
Next

Epidemiology

Frequency

United States

The incidence of Churg-Strauss syndrome in the United States is 1-3 cases per 100,000 adults per year.[15]

International

The international incidence of Churg-Strauss syndrome is approximately 2.5 cases per 100,000 adults per year.

Mortality/Morbidity

The principal causes of morbidity and mortality in Churg-Strauss syndrome are myocarditis and myocardial infarction secondary to coronary arteritis.[16] With treatment, the 1-year survival rate is 90% and the 5-year survival rate is 62%.[2, 17]

Sex- and Age-related Demographics

Churg-Strauss syndrome is slightly more common in males than in females.The age at onset varies from 15-70 years, with a mean age of approximately 38 years. Churg-Strauss syndrome in pediatric patients is well described, but mostly as case reports.[18] The mean age at diagnosis is around 50 years.

Previous
 
 
Contributor Information and Disclosures
Author

Spencer T Lowe, MD Rheumatologist, Private Practice, Peninsula Medical Group, Burlingame, CA

Spencer T Lowe, MD is a member of the following medical societies: American College of Rheumatology, California Medical Association, International Society for Clinical Densitometry

Disclosure: Nothing to disclose.

Coauthor(s)

Christopher L Tracy, MD Associate Program Director, Rheumatology Fellowship Program, Walter Reed National Military Medical Center

Christopher L Tracy, MD is a member of the following medical societies: American College of Physicians, American College of Rheumatology, American Medical Association

Disclosure: Nothing to disclose.

Specialty Editor Board

Francisco Talavera, PharmD, PhD Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Received salary from Medscape for employment. for: Medscape.

Lawrence H Brent, MD Associate Professor of Medicine, Jefferson Medical College of Thomas Jefferson University; Chair, Program Director, Department of Medicine, Division of Rheumatology, Albert Einstein Medical Center

Lawrence H Brent, MD is a member of the following medical societies: American Association for the Advancement of Science, American Association of Immunologists, American College of Physicians, American College of Rheumatology

Disclosure: Serve(d) as a director, officer, partner, employee, advisor, consultant or trustee for: Janssen<br/>Serve(d) as a speaker or a member of a speakers bureau for: Abbvie; Genentech; Pfizer; Questcor.

Chief Editor

Herbert S Diamond, MD Visiting Professor of Medicine, Division of Rheumatology, State University of New York Downstate Medical Center; Chairman Emeritus, Department of Internal Medicine, Western Pennsylvania Hospital

Herbert S Diamond, MD is a member of the following medical societies: Alpha Omega Alpha, American College of Physicians, American College of Rheumatology, American Medical Association, Phi Beta Kappa

Disclosure: Nothing to disclose.

Additional Contributors

Mehran Farid-Moayer, MD Adjunct Clinical Faculty, Department of Psychiatry, Sleep Disorders Clinic, Stanford Medical Center

Mehran Farid-Moayer, MD is a member of the following medical societies: American Academy of Sleep Medicine, American Medical Association

Disclosure: Nothing to disclose.

Acknowledgements

The authors and editors of Medscape Reference gratefully acknowledge the contributions of previous coauthor, Sandra L Sessoms, MD, to the development and writing of this article.

References
  1. [Guideline] Groh M, Pagnoux C, Baldini C, Bel E, Bottero P, Cottin V, et al. Eosinophilic granulomatosis with polyangiitis (Churg-Strauss) (EGPA) Consensus Task Force recommendations for evaluation and management. Eur J Intern Med. 2015 Sep. 26 (7):545-53. [Medline]. [Full Text].

  2. Guillevin L, Cohen P, Gayraud M, et al. Churg-Strauss syndrome. Clinical study and long-term follow-up of 96 patients. Medicine (Baltimore). 1999 Jan. 78(1):26-37. [Medline].

  3. Churg J, Strauss L. Allergic granulomatosis, allergic angiitis, and periarteritis nodosa. Am J Pathol. 1951 Mar-Apr. 27(2):277-301. [Medline].

  4. Churg A. Pulmonary angiitis and granulomatosis revisited. Hum Pathol. 1983 Oct. 14(10):868-83. [Medline].

  5. Keogh KA, Specks U. Churg-Strauss syndrome: clinical presentation, antineutrophil cytoplasmic antibodies, and leukotriene receptor antagonists. Am J Med. 2003 Sep. 115(4):284-90. [Medline].

  6. 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 Aug. 33(8):1094-100. [Medline].

  7. Jennette JC, Falk RJ, Andrassy K, et al. Nomenclature of systemic vasculitides. Proposal of an international consensus conference. Arthritis Rheum. 1994 Feb. 37(2):187-92. [Medline].

  8. Hellmich B, Ehlers S, Csernok E, Gross WL. Update on the pathogenesis of Churg-Strauss syndrome. Clin Exp Rheumatol. 2003 Nov-Dec. 21(6 Suppl 32):S69-77. [Medline].

  9. Franco J, Artes MJ. Pulmonary eosinophilia associated with montelukast. Thorax. 1999 Jun. 54(6):558-60. [Medline].

  10. Wechsler ME, Finn D, Gunawardena D, et al. Churg-Strauss syndrome in patients receiving montelukast as treatment for asthma. Chest. 2000 Mar. 117(3):708-13. [Medline].

  11. Hauser T, Mahr A, Metzler C, Coste J, Sommerstein R, Gross WL, et al. The leucotriene receptor antagonist montelukast and the risk of Churg-Strauss syndrome: a case-crossover study. Thorax. 2008 Aug. 63(8):677-82. [Medline].

  12. Wechsler ME, Garpestad E, Flier SR, et al. Pulmonary infiltrates, eosinophilia, and cardiomyopathy following corticosteroid withdrawal in patients with asthma receiving zafirlukast. JAMA. 1998 Feb 11. 279(6):455-7. [Medline].

  13. Tuggey JM, Hosker HS. Churg-Strauss syndrome associated with montelukast therapy. Thorax. 2000 Sep. 55(9):805-6. [Medline].

  14. Vaglio A, Martorana D, Maggiore U, Grasselli C, Zanetti A, Pesci A, et al. HLA-DRB4 as a genetic risk factor for Churg-Strauss syndrome. Arthritis Rheum. 2007 Sep. 56(9):3159-66. [Medline].

  15. Eustace JA, Nadasdy T, Choi M. Disease of the month. The Churg Strauss Syndrome. J Am Soc Nephrol. 1999 Sep. 10(9):2048-55. [Medline].

  16. Hasley PB, Follansbee WP, Coulehan JL. Cardiac manifestations of Churg-Strauss syndrome: report of a case and review of the literature. Am Heart J. 1990 Oct. 120(4):996-9. [Medline].

  17. Keogh KA, Specks U. Churg-Strauss syndrome: update on clinical, laboratory and therapeutic aspects. Sarcoidosis Vasc Diffuse Lung Dis. 2006 Mar. 23(1):3-12. [Medline].

  18. Boyer D, Vargas SO, Slattery D, Rivera-Sanchez YM, Colin AA. Churg-Strauss syndrome in children: a clinical and pathologic review. Pediatrics. 2006 Sep. 118(3):e914-20. [Medline].

  19. Wechsler ME, Finn D, Gunawardena D, Westlake R, Barker A, Haranath SP, et al. Churg-Strauss syndrome in patients receiving montelukast as treatment for asthma. Chest. 2000 Mar. 117(3):708-13. [Medline].

  20. Sinico RA, Sabadini E, Maresca AM. Mesalazine-induced Churg-Strauss syndrome in a patient with Crohn's disease and sclerosing cholangitis. Clin Exp Rheumatol. 2006 Mar-Apr. 24(2 Suppl 41):S104. [Medline].

  21. Orriols R, Munoz X, Ferrer J, Huget P, Morell F. Cocaine-induced Churg-Strauss vasculitis. Eur Respir J. 1996 Jan. 9(1):175-7. [Medline].

  22. Sokolowska BM, Szczeklik WK, Wludarczyk AA, Kuczia PP, Jakiela BA, Gasior JA, et al. ANCA-positive and ANCA-negative phenotypes of eosinophilic granulomatosis with polyangiitis (EGPA): outcome and long-term follow-up of 50 patients from a single Polish center. Clin Exp Rheumatol. 2014 May-Jun. 32(3 Suppl 82):S41-7. [Medline].

  23. Sablé-Fourtassou R, Cohen P, Mahr A, Pagnoux C, Mouthon L, Jayne D, et al. Antineutrophil cytoplasmic antibodies and the Churg-Strauss syndrome. Ann Intern Med. 2005 Nov 1. 143(9):632-8. [Medline].

  24. Choi YH, Im JG, Han BK, Kim JH, Lee KY, Myoung NH. Thoracic manifestation of Churg-Strauss syndrome: radiologic and clinical findings. Chest. 2000 Jan. 117(1):117-24. [Medline].

  25. Kim Y, Lee KS, Choi DC, et al. The spectrum of eosinophilic lung disease: radiologic findings. J Comput Assist Tomogr. 1997 Nov-Dec. 21(6):920-30. [Medline].

  26. Buschman DL, Waldron JA Jr, King TE Jr. Churg-Strauss pulmonary vasculitis. High-resolution computed tomography scanning and pathologic findings. Am Rev Respir Dis. 1990 Aug. 142(2):458-61. [Medline].

  27. Worthy SA, Müller NL, Hansell DM, Flower CD. Churg-Strauss syndrome: the spectrum of pulmonary CT findings in 17 patients. AJR Am J Roentgenol. 1998 Feb. 170(2):297-300. [Medline].

  28. Mavrogeni S, Karabela G, Gialafos E, Stavropoulos E, Spiliotis G, Katsifis G, et al. Cardiac involvement in ANCA (+) and ANCA (-) Churg-Strauss syndrome evaluated by cardiovascular magnetic resonance. Inflamm Allergy Drug Targets. 2013 Oct. 12(5):322-7. [Medline].

  29. Lie JT. Histopathologic specificity of systemic vasculitis. Rheum Dis Clin North Am. 1995 Nov. 21(4):883-909. [Medline].

  30. Katzenstein AL. Diagnostic features and differential diagnosis of Churg-Strauss syndrome in the lung. A review. Am J Clin Pathol. 2000 Nov. 114(5):767-72. [Medline].

  31. Chemmalakuzhy AJ, Zhou XJ, Hedayati SS. ANCA-negative glomerulonephritis associated with nonasthmatic Churg-Strauss syndrome. Nat Clin Pract Nephrol. 2008 Oct. 4(10):568-74. [Medline].

  32. Lhote F, Cohen P, Guilpain P, Guillevin L. [Churg-Strauss syndrome]. Rev Prat. 2008 Jun 15. 58(11):1165-74. [Medline].

  33. Hervier B, Durant C, Masseau A, Ponge T, Hamidou M, Mussini JM. Use of muscle biopsies for diagnosis of systemic vasculitides. J Rheumatol. 2011 Mar. 38(3):470-4. [Medline].

  34. Grau RG. Churg-Strauss syndrome: 2005-2008 update. Curr Rheumatol Rep. 2008 Dec. 10(6):453-8. [Medline].

  35. Mukhtyar C, Flossmann O, Hellmich B, Bacon P, Cid M, Cohen-Tervaert JW, 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 Jul. 67(7):1004-10. [Medline].

  36. Mohammad AJ, Hot A, Arndt F, Moosig F, Guerry MJ, Amudala N, et al. Rituximab for the treatment of eosinophilic granulomatosis with polyangiitis (Churg-Strauss). Ann Rheum Dis. 2016 Feb. 75 (2):396-401. [Medline].

  37. Jachiet M, Samson M, Cottin V, Kahn JE, Le Guenno G, et al. Anti-IgE monoclonal antibody (omalizumab) in refractory and relapsing eosinophilic granulomatosis with polyangiitis (Churg-Strauss): Data from 17 patients. Arthritis Rheumatol. 2016 Mar 4. [Medline].

  38. Tiliakos A 4th, Shaia S, Hostoffer R, Kent L. The use of infliximab in a patient with steroid-dependent churg-strauss syndrome. J Clin Rheumatol. 2004 Apr. 10(2):96-7. [Medline].

  39. Casian A, Jayne D. Plasma exchange in the treatment of Wegener's granulomatosis, microscopic polyangiitis, Churg-Strauss syndrome and renal limited vasculitis. Curr Opin Rheumatol. 2011 Jan. 23(1):12-7. [Medline].

  40. Guillevin L, Cevallos R, Durand-Gasselin B, et al. Treatment of glomerulonephritis in microscopic polyangiitis and Churg-Strauss syndrome. Indications of plasma exchanges, Meta-analysis of 2 randomized studies on 140 patients, 32 with glomerulonephritis. Ann Med Interne (Paris). 1997. 148(3):198-204. [Medline].

  41. Bonaci-Nikolic B, Nikolic MM, Andrejevic S, Zoric S, Bukilica M. Antineutrophil cytoplasmic antibody (ANCA)-associated autoimmune diseases induced by antithyroid drugs: comparison with idiopathic ANCA vasculitides. Arthritis Res Ther. 2005. 7(5):R1072-81. [Medline].

  42. Cohen P, Guillevin L, Baril L, et al. Persistence of antineutrophil cytoplasmic antibodies (ANCA) in asymptomatic patients with systemic polyarteritis nodosa or Churg-Strauss syndrome: follow-up of 53 patients. Clin Exp Rheumatol. 1995 Mar-Apr. 13(2):193-8. [Medline].

  43. Espinoza LR. Combination therapy to treat churg-strauss syndrome: corticosteroids with short- or long-term cyclophosphamide pulses. Curr Rheumatol Rep. 2008 Dec. 10(6):427-9. [Medline].

  44. Yamasaki A, Tomita K, Fujii Y, Hasegawa Y, Watanabe M, Sano H, et al. Repressed ileal artery aneurysms in Churg-Strauss syndrome following combination treatment with glucocorticoid and cyclophosphamide. Rheumatol Int. 2008 Aug 8. [Medline].

  45. Ribi C, Cohen P, Pagnoux C, Mahr A, Arène JP, Lauque D, 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 Feb. 58(2):586-94. [Medline].

  46. Tatsis E, Schnabel A, Gross WL. Interferon-alpha treatment of four patients with the Churg-Strauss syndrome. Ann Intern Med. 1998 Sep 1. 129(5):370-4. [Medline].

  47. Metzler C, Schnabel A, Gross WL, Hellmich B. A phase II study of interferon-alpha for the treatment of refractory Churg-Strauss syndrome. Clin Exp Rheumatol. 2008 May-Jun. 26(3 Suppl 49):S35-40. [Medline].

  48. Beasley R, Bibby S, Weatherall M. Leukotriene receptor antagonist therapy and Churg-Strauss syndrome: culprit or innocent bystander?. Thorax. 2008 Oct. 63(10):847-9. [Medline].

  49. Giavina-Bianchi P, Giavina-Bianchi M, Agondi R, Kalil J. Three months' administration of anti-IgE to a patient with Churg-Strauss syndrome. J Allergy Clin Immunol. 2007 May. 119(5):1279; author reply 1279-80. [Medline].

  50. Green RL, Vayonis AG. Churg-Strauss syndrome after zafirlukast in two patients not receiving systemic steroid treatment. Lancet. 1999 Feb 27. 353(9154):725-6. [Medline].

  51. Guillevin L, Lhote F, Gayraud M, et al. Prognostic factors in polyarteritis nodosa and Churg-Strauss syndrome. A prospective study in 342 patients. Medicine (Baltimore). 1996 Jan. 75(1):17-28. [Medline].

  52. Kaushik VV, Reddy HV, Bucknall RC. Successful use of rituximab in a patient with recalcitrant Churg-Strauss syndrome. Ann Rheum Dis. 2006 Aug. 65(8):1116-7. [Medline].

  53. Kümpers P, Erdbrügger U, Grossheim M, Meyer GP, Hiss M, Gwinner W, et al. Endothelial microparticles as a diagnostic aid in Churg-Strauss vasculitis-induced cardiomyopathy. Clin Exp Rheumatol. 2008 May-Jun. 26(3 Suppl 49):S86-9. [Medline].

  54. Lie JT. Illustrated histopathologic classification criteria for selected vasculitis syndromes. American College of Rheumatology Subcommittee on Classification of Vasculitis. Arthritis Rheum. 1990 Aug. 33(8):1074-87. [Medline].

  55. Pabst S, Tiyerili V, Grohé C. Apparent response to anti-IgE therapy in two patients with refractory "forme fruste" of Churg-Strauss syndrome. Thorax. 2008 Aug. 63(8):747-8. [Medline].

  56. Roccatello D, Baldovino S, Alpa M, Rossi D, Napoli F, Naretto C, et al. Effects of anti-CD20 monoclonal antibody as a rescue treatment for ANCA-associated idiopathic systemic vasculitis with or without overt renal involvement. Clin Exp Rheumatol. 2008 May-Jun. 26(3 Suppl 49):S67-71. [Medline].

  57. Todd DC, Cockcroft DW. Prolonged survival in Churg-Strauss syndrome. Ann Allergy Asthma Immunol. 2004 Jan. 92(1):92-3. [Medline].

  58. Greco A, Rizzo MI, De Virgilio A, Gallo A, Fusconi M, Ruoppolo G, et al. Churg-Strauss syndrome. Autoimmun Rev. 2015 Apr. 14 (4):341-8. [Medline].

 
Previous
Next
 
Transient pulmonary infiltrates in a patient with Churg-Strauss syndrome (CSS).
Eosinophilic granuloma in a patient with Churg-Strauss syndrome (CSS).
The skin rashes of Churg-Strauss syndrome. The biopsy of this rash showed eosinophilic leukocytoclastic angiitis with poorly formed granulomas.
 
 
 
All material on this website is protected by copyright, Copyright © 1994-2016 by WebMD LLC. This website also contains material copyrighted by 3rd parties.