Eosinophilic granulomatosis with polyangiitis (Churg-Strauss syndrome)

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 Dec;53(12):2306-9.Full text  Abstract

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 Sep;26(7):545-53.Full text  Abstract

Chung SA, Langford CA, Maz M, et al. 2021 American College of Rheumatology/Vasculitis Foundation guideline for the management of antineutrophil cytoplasmic antibody-associated vasculitis. Arthritis Rheumatol. 2021 Aug;73(8):1366-83.Full text  Abstract

Hellmich B, Sanchez-Alamo B, Schirmer JH, et al. EULAR recommendations for the management of ANCA-associated vasculitis: 2022 update. Ann Rheum Dis. 2023 Mar 16:ard-2022-223764.Full text  Abstract

1. Jennette JC, Falk RJ, Bacon PA, et al. 2012 revised International Chapel Hill Consensus Conference Nomenclature of Vasculitides. Arthritis Rheum. 2013 Jan;65(1):1-11.Full text  Abstract

2. Grayson PC, Ponte C, Suppiah R, et al. 2022 American College of Rheumatology/European Alliance of Associations for Rheumatology classification criteria for eosinophilic granulomatosis with polyangiitis. Ann Rheum Dis. 2022 Mar;81(3):309-14.Full text  Abstract

3. Churg A, Brallas M, Cronin SR, et al. Formes frustes of Churg-Strauss syndrome. Chest. 1995 Aug;108(2):320-3. Abstract

4. 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. Abstract

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10. 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 Dec;53(12):2306-9.Full text  Abstract

11. Loughlin JE, Cole JA, Rothman KJ, et al. Prevalence of serious eosinophilia and incidence of Churg-Strauss syndrome in a cohort of asthma patients. Ann Allergy Asthma Immunol. 2002 Mar;88(3):319-25. Abstract

12. Harrold LR, Andrade SE, Go AS, et al. Incidence of Churg-Strauss syndrome in asthma drug users: a population-based perspective. J Rheumatol. 2005 Jun;32(6):1076-80. Abstract

13. 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. Abstract

14. Mukhtyar C, Flossmann O, Hellmich B, et al; European Vasculitis Study Group (EUVAS). 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.Full text  Abstract

15. Phillip R, Luqmani R. Mortality in systemic vasculitis: a systematic review. Clin Exp Rheumatol. 2008 Sep-Oct;26(5 suppl 51):S94-104. Abstract

16. Comarmond C, Pagnoux C, Khellaf M, et al; French Vasculitis Study Group. 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 Jan;65(1):270-81.Full text  Abstract

17. Samson M, Puéchal X, Devilliers H, et al; French Vasculitis Study Group. Long-term outcomes of 118 patients with eosinophilic granulomatosis with polyangiitis (Churg-Strauss syndrome) enrolled in two prospective trials. J Autoimmun. 2013 Jun;43:60-9. Abstract

18. Weller PF, Plaut M, Taggart V, et al. The relationship of asthma therapy and Churg-Strauss syndrome: NIH workshop summary report. J Allergy Clin Immunol. 2001 Aug;108(2):175-83.Full text  Abstract

19. Keogh KA. Leukotriene receptor antagonists and Churg-Strauss syndrome: cause, trigger or merely an association? Drug Saf. 2007;30(10):837-43. Abstract

20. Hauser T, Mahr A, Metzler C, 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.Full text  Abstract

21. 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. Abstract

22. Manger BJ, Krapf FE, Gramatzki M, et al. IgE-containing circulating immune complexes in Churg-Strauss vasculitis. Scand J Immunol. 1985 Apr;21(4):369-73. Abstract

23. Pepper RJ, Fabre MA, Pavesio C, et al. Rituximab is effective in the treatment of refractory Churg-Strauss syndrome and is associated with diminished T-cell interleukin-5 production. Rheumatology (Oxford). 2008 Jul;47(7):1104-5.Full text  Abstract

24. Kane GC, Keogh KA. Involvement of the heart by small and medium vessel vasculitis. Curr Opin Rheumatol. 2009 Jan;21(1):29-34. Abstract

25. Xiao H, Heeringa P, Hu P, et al. Antineutrophil cytoplasmic autoantibodies specific for myeloperoxidase cause glomerulonephritis and vasculitis in mice. J Clin Invest. 2002 Oct;110(7):955-63.Full text  Abstract

26. Sinico RA, Di Toma L, Maggiore U, et al. Prevalence and clinical significance of antineutrophil cytoplasmic antibodies in Churg-Strauss syndrome. Arthritis Rheum. 2005 Sep;52(9):2926-35.Full text  Abstract

27. Sable-Fourtassou R, Cohen P, Mahr A, et al. Antineutrophil cytoplasmic antibodies and Churg-Strauss syndrome. Ann Intern Med. 2005 Nov 1;143(9):632-8. Abstract

28. 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 Sep;26(7):545-53.Full text  Abstract

29. 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 Jan;16(1):1-9. Abstract

30. Cools J, DeAngelo DJ, Gotlib J, et al. A tyrosine kinase created by fusion of the PDGFRA and FIP1L1 genes as a therapeutic target of imatinib in idiopathic hypereosinophilic syndrome. N Engl J Med. 2003 Mar 27;348(13):1201-14.Full text  Abstract

31. Pardanani A, Ketterling RP, Brockman SR, et al. CHIC2 deletion, a surrogate for FIP1L1-PDGFRA fusion, occurs in systemic mastocytosis associated with eosinophilia and predicts response to imatinib mesylate therapy. Blood. 2003 Nov 1;102(9):3093-6.Full text  Abstract

32. Buzio C, Oliva E. Diagnosis of Churg Strauss syndrome: eotaxin-3 makes it easier. Rheumatology (Oxford). 2011 Oct;50(10):1737-8.Full text  Abstract

33. Zwerina J, Bach C, Martorana D, et al. Eotaxin-3 in Churg-Strauss syndrome: a clinical and immunogenetic study. Rheumatology (Oxford). 2011 Oct;50(10):1823-7.Full text  Abstract

34. Chung SA, Langford CA, Maz M, et al. 2021 American College of Rheumatology/Vasculitis Foundation guideline for the management of antineutrophil cytoplasmic antibody-associated vasculitis. Arthritis Rheumatol. 2021 Aug;73(8):1366-83.Full text  Abstract

35. Allenbach Y, Seror R, Pagnoux C, et al. High frequency of venous thromboembolic events in Churg-Strauss syndrome, Wegener's granulomatosis and microscopic polyangiitis but not polyarteritis nodosa: a systematic retrospective study on 1130 patients. Ann Rheum Dis. 2009 Apr;68(4):564-7. Abstract

36. Keogh KA, Kane GC, Specks U. Prevalence of venous thromboembolism in patients with Churg-Strauss syndrome. Am J Respir Crit Care Med. 2009;179:A4401.

37. 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. Abstract

38. Guillevin L, Pagnoux C, Seror R, et al; French Vasculitis Study Group (FVSG). The Five-Factor Score revisited: assessment of prognoses of systemic necrotizing vasculitides based on the French Vasculitis Study Group (FVSG) cohort. Medicine (Baltimore). 2011 Jan;90(1):19-27. Abstract

39. Bourgarit A, Le Toumelin P, Pagnoux C, et al. 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). 2005 Sep;84(5):323-30. Abstract

40. Hellmich B, Gross WL. Recent progress in the pharmacotherapy of Churg-Strauss syndrome. Expert Opin Pharmacother. 2004 Jan;5(1):25-35.Full text  Abstract

41. Moosig F, Gross WL, Herrmann K, et al. Targeting interleukin-5 in refractory and relapsing Churg-Strauss syndrome. Ann Intern Med. 2011 Sep 6;155(5):341-3. Abstract

42. Wechsler ME, Akuthota P, Jayne D, et al. Mepolizumab or placebo for eosinophilic granulomatosis with polyangiitis. N Engl J Med. 2017 May 18;376(20):1921-32.Full text  Abstract

43. Puéchal X, Pagnoux C, Baron G, et al. Adding azathioprine to remission-induction glucocorticoids for eosinophilic granulomatosis with polyangiitis (Churg-Strauss), microscopic polyangiitis, or polyarteritis nodosa without poor prognosis factors: a randomized, controlled trial. Arthritis Rheumatol. 2017 Nov;69(11):2175-86. Abstract

44. Ribi C, Cohen P, Pagnoux C, et al; French Vasculitis Study Group. 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.Full text  Abstract

45. Guillevin L, Le Thi Huong Du, Godeau P, et al. Clinical findings and prognosis of polyarteritis nodosa and Churg-Strauss angiitis: a study in 165 patients. Br J Rheumatol. 1988 Aug;27(4):258-64. Abstract

46. de Groot K, Harper L, Jayne DR, et al. Pulse versus daily oral cyclophosphamide for induction of remission in antineutrophil cytoplasmic antibody-associated vasculitis: a randomized trial. Ann Intern Med. 2009 May 19;150(10):670-80. Abstract

47. Hellmich B, Sanchez-Alamo B, Schirmer JH, et al. EULAR recommendations for the management of ANCA-associated vasculitis: 2022 update. Ann Rheum Dis. 2023 Mar 16:ard-2022-223764.Full text  Abstract

48. Terrier B, Pugnet G, de Moreuil C, et al. Rituximab versus conventional therapeutic strategy for remission induction in eosinophilic granulomatosis with polyangiitis: a double-blind, randomized, controlled trial [abstract presented at ACR Convergence 2021]. Arthritis Rheumatol. 2021;73(9).Full text

49. ClinicalTrials.gov. Maintenance of remission with rituximab versus azathioprine for newly-diagnosed or relapsing eosinophilic granulomatosis with polyangiitis (MAINRITSEG). ClinicalTrials.gov Identifier: NCT03164473. Dec 2022 [internet publication].Full text

50. Walsh M, Collister D, Zeng L, et al. The effects of plasma exchange in patients with ANCA-associated vasculitis: an updated systematic review and meta-analysis. BMJ. 2022 Feb 25;376:e064604.Full text  Abstract

51. Ortega HG, Liu MC, Pavord ID, et al. Mepolizumab treatment in patients with severe eosinophilic asthma. N Engl J Med. 2014 Sep 25;371(13):1198-207.Full text  Abstract

52. Teixeira V, Mohammad AJ, Jones RB, et al. Efficacy and safety of rituximab in the treatment of eosinophilic granulomatosis with polyangiitis. RMD Open. 2019;5(1):e000905.Full text  Abstract

53. Canzian A, Venhoff N, Urban ML, et al. Use of biologics to treat relapsing and/or refractory eosinophilic granulomatosis with polyangiitis: data from a European collaborative study. Arthritis Rheumatol. 2021 Mar;73(3):498-503. Abstract

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55. American College of Rheumatology. Guideline for prevention and treatment of glucocorticoid-induced osteoporosis. Oct 2023 [internet publication].Full text

56. Somers EC, Marder W, Christman GM, et al. Use of a gonadotropin-releasing hormone analog for protection against premature ovarian failure during cyclophosphamide therapy in women with severe lupus. Arthritis Rheum. 2005 Sep;52(9):2761-7.Full text  Abstract

57. Manger K, Wildt L, Kalden JR, et al. Prevention of gonadal toxicity and preservation of gonadal function and fertility in young women with systemic lupus erythematosus treated by cyclophosphamide: the PREGO-Study. Autoimmun Rev. 2006 Apr;5(4):269-72. Abstract

58. Metzler C, Schnabel A, Gross WL, et al. 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.Full text  Abstract

59. Seeliger B, Förster M, Happe J, et al. Interferon-α for induction and maintenance of remission in eosinophilic granulomatosis with polyangiitis: a single-center retrospective observational cohort study. J Rheumatol. 2017 Jun;44(6):806-14.Full text  Abstract

60. Metzler C, Csernok E, Gross WL, et al. Interferon-alpha for maintenance of remission in Churg-Strauss syndrome: a long-term observational study. Clin Exp Rheumatol. 2010 Jan-Feb;28(1 suppl 57):24-30.Full text  Abstract

61. Manka LA, Guntur VP, Denson JL, et al. Efficacy and safety of reslizumab in the treatment of eosinophilic granulomatosis with polyangiitis. Ann Allergy Asthma Immunol. 2021 Jun;126(6):696-701;e1. Abstract

62. Guntur VP, Manka LA, Denson JL, et al. Benralizumab as a steroid-sparing treatment option in eosinophilic granulomatosis with polyangiitis. J Allergy Clin Immunol Pract. 2021 Mar;9(3):1186-93;e1. Abstract

63. Basta F, Mazzuca C, Nucera E, et al. Omalizumab in eosinophilic granulomatosis with polyangiitis: friend or foe? A systematic literature review. Clin Exp Rheumatol. 2020 Mar-Apr;38 suppl 124(2):214-20.Full text  Abstract

64. Neumann T, Manger B, Schmid M, et al. Cardiac involvement in Churg-Strauss syndrome: impact of endomyocarditis. Medicine (Baltimore). 2009 Jul;88(4):236-43.Full text  Abstract

65. Dennert RM, van Paassen P, Schalla S, et al. Cardiac involvement in Churg-Strauss syndrome. Arthritis Rheum. 2010 Feb;62(2):627-34.Full text  Abstract

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