The Latest on Eosinophilic Granulomatosis with Polyangiitis

In the end, at least as much as in other chronic rheumatic diseases, management of EGPA involves a long-term discussion with the patient to balance control of symptoms and medication side effects, based more on subjective experiences than on objective findings.

Paul A. Monach, MD, PhD, is chief of the rheumatology section of the VA Boston Healthcare System and an associate professor in the Section of Rheumatology of the Boston University School of Medicine. He specializes in clinical care and clinical research in vasculitis.

Disclosure: I was a site investigator in the recent trial of mepolizumab in EGPA.¹¹

References

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.
2. Comarmond C, Pagnoux C, Khellaf M, et al. 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):270–281.
3. Durel CA, Berthiller J, Caboni S, et al. Long-term followup of a multicenter cohort of 101 patients with eosinophilic granulomatosis with polyangiitis (Churg-Strauss). Arthritis Care Res (Hoboken). 2016 Mar;68(3):374–387.
4. 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.
5. 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 Sept;26(7):545–553.
6. 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.
7. Gayraud M, Guillevin L, le Toumelin P, et al. Long-term followup of polyarteritis nodosa, microscopic polyangiitis, and Churg-Strauss syndrome: Analysis of four prospective trials including 278 patients. Arthritis Rheum. 2001 Mar;44(3):666–675.
8. Samson M, Puéchal X, Devilliers H, et al. Long-term outcomes of 118 patients with eosinophilic granulomatosis with polyangiitis (Churg-Strauss syndrome) enrolled in two prospective trials. J Autoimmun. 2013 Jun;43:60–69.
9. Ribi C, Cohen P, Pagnoux 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 Feb;58(2):586–594.
10. 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 Rheum. 2017;69:2175–2186.
11. Wechsler ME, Akuthota P, Jayne D, et al. Mepolizumab or placebo for eosinophilic granulomatosis with polyangiitis. N Engl J Med. 2017 May;376(20):1921–1932.
12. Mohammad AJ, Hot A, Arndt F, et al. Rituximab for the treatment of eosinophilic granulomatosis with polyangiitis (Churg-Strauss). Ann Rheum Dis. 2016 Feb;75(2):396–401.
13. Grayson PC, Monach PA, Pagnoux C, et al. Value of commonly measured laboratory tests as biomarkers of disease activity and predictors of relapse in eosinophilic granulomatosis with polyangiitis. Rheumatology (Oxford). 2015 Aug;54(8):1351–1359.
14. Dejaco C, Oppl B, Monach P, et al. Serum biomarkers in patients with relapsing eosinophilic granulomatosis with polyangiitis (Churg-Strauss). PLoS One. 2015 Mar 26;10(3):e0121737.