Eosinophilic granulomatosis with polyangiitis - Symptoms, Causes, Images, and Treatment Options

Diseases

Highlights & Basics

Key Highlights

Eosinophilic granulomatosis with polyangiitis (also known as Churg-Strauss syndrome) is characterized by the pathologic triad of tissue eosinophilia, granulomatous inflammation, and vasculitis.
The cornerstone of treatment is corticosteroids. Severe disease is treated in a similar manner to other antineutrophil cytoplasmic antibody associated vasculitides, with additional immunosuppressive agents.
Cardiac involvement causes significant morbidity and mortality.
Typically patients are initially diagnosed with asthma, and then months to years later develop manifestations due to tissue eosinophilia, followed by vasculitis.

Quick Reference

History & Exam
- Key Factors
- Other Factors
More information...
Diagnostics Tests
More information...
Treatment Options
More information...

Definition

Epidemiology

Etiology

Pathophysiology

Images

CT scan of the chest obtained (A) during symptom exacerbation, revealing pulmonary infiltrates in the right inferior lobe (A3) and the left inferior lobe (A4). Re-evaluation 15 days later (B) revealed the migratory character of the lesions, with complete disappearance of the previously described infiltrates and the presence of new areas of ground glass opacities within the right inferior lobe (B2) and the left lingular and left inferior lobe (B4). (C) Six months after treatment, all the lung lesions described had healed completely
Histologic evaluation of the lung biopsy specimen, revealing the presence of an inflammatory infiltrate composed predominantly of eosinophils found within both the vascular lumen and the vascular wall
Cytologic examination of the bronchoalveolar lavage fluid, revealing the presence of a vast predominance of eosinophils (A), representing 27% of the cellular elements. The other cellular elements found were macrophages (C) and lymphocytes (B)

Citations

Key Articles

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.[Abstract][Full Text]
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.[Abstract][Full Text]
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.[Abstract][Full Text]
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.[Abstract][Full Text]

Other Online Resources

BMJ: plasma exchange and glucocorticoid dosing for patients with ANCA-associated vasculitis: a clinical practice guideline

Referenced Articles

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.[Abstract][Full Text]
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.[Abstract][Full Text]
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]
5. Solans R, Bosch JA, Perez-Bocanegra C, et al. Churg-Strauss syndrome: outcome and long-term follow-up of 32 patients. Rheumatology (Oxford). 2001 Jul;40(7):763-71.[Abstract][Full Text]
6. Watts RA, Carruthers DM, Scott DG. Epidemiology of systemic vasculitis: changing incidence or definition? Semin Arthritis Rheum. 1995 Aug;25(1):28-34.[Abstract]
7. Watts RA, Lane SE, Bentham G, et al. Epidemiology of systemic vasculitis: a ten-year study in the United Kingdom. Arthritis Rheum. 2000 Feb;43(2):414-9.[Abstract]
8. Gonzalez-Gay MA, Garcia-Porrua C, Guerrero J, et al. The epidemiology of the primary systemic vasculitides in northwest Spain: implications of the Chapel Hill Consensus Conference definitions. Arthritis Rheum. 2003 Jun 15;49(3):388-93.[Abstract][Full Text]
9. Reinhold-Keller E, Herlyn K, Wagner-Bastmeyer R, et al. Stable incidence of primary systemic vasculitides over five years: results from the German vasculitis register. Arthritis Rheum. 2005 Feb 15;53(1):93-9.[Abstract][Full Text]
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.[Abstract][Full Text]
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.[Abstract][Full Text]
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.[Abstract][Full Text]
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.[Abstract][Full Text]
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.[Abstract][Full Text]
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.[Abstract][Full Text]
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.[Abstract][Full Text]
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.[Abstract][Full Text]
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.[Abstract][Full Text]
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.[Abstract][Full Text]
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.[Abstract][Full Text]
32. Buzio C, Oliva E. Diagnosis of Churg Strauss syndrome: eotaxin-3 makes it easier. Rheumatology (Oxford). 2011 Oct;50(10):1737-8.[Abstract][Full Text]
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.[Abstract][Full Text]
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.[Abstract][Full Text]
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.[Abstract][Full Text]
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.[Abstract][Full Text]
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.[Abstract][Full Text]
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.[Abstract][Full Text]
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.[Abstract][Full Text]
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.[Abstract][Full Text]
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.[Abstract][Full Text]
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]
54. Huang L, Morris A, Limper AH, et al. An Official ATS Workshop Summary: recent advances and future directions in Pneumocystis pneumonia (PCP). Proc Am Thorac Soc. 2006 Nov;3(8):655-64.[Abstract]
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.[Abstract][Full Text]
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.[Abstract][Full Text]
59. Seeliger B, Förster M, Happe J, et al. Interferon-alpha 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.[Abstract][Full Text]
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.[Abstract][Full Text]
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.[Abstract][Full Text]
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.[Abstract][Full Text]
65. Dennert RM, van Paassen P, Schalla S, et al. Cardiac involvement in Churg-Strauss syndrome. Arthritis Rheum. 2010 Feb;62(2):627-34.[Abstract][Full Text]
66. Talar-Williams C, Hijazi YM, Walther MM, et al. Cyclophosphamide-induced cystitis and bladder cancer in patients with Wegener granulomatosis. Ann Intern Med. 1996 Mar 1;124(5):477-84.[Abstract]