Off-Label Use of Biological Therapies in Relapsing and/or Refractory Eosinophilic Granulomatosis with Polyangiitis (Churg–Strauss)

Laure Denis¹, Maxime Samson², Francois Maurier³, Chahéra Khouatra⁴, Vincent Germain⁵, Xavier Delbrel⁶, Philippe Bonniaud⁷, Alban Deroux⁸, Nicolas Girszyn⁹, Claire de Moreuil¹⁰, Dominique Chauveau¹¹, Anne Gondouin¹², Stephane Dominique¹³, Guillaume Le Guenno¹⁴, Laurence Bouillet¹⁵, Bernard Bonnotte², Jean-Emmanuel Kahn¹⁶, Boris Bienvenu¹⁷, Bertrand Godeau¹⁸, Claire Le Jeunne¹⁹, Xavier Puéchal²⁰, Loïc Guillevin for the French Vasculitis Study Group²⁰ and Benjamin Terrier¹⁹, ¹Internal Medicine, CHU Estaing, Clermont-Ferrand, Clermont-Ferrand, France, ²Department of Internal Medicine and Clinical Immunology, Hôpital François Mitterrand, CHU de Dijon, Dijon, France, ³Internal Medicine, Sainte-Blandine de Metz Hospital, Metz, France, ⁴Lyon, Lyon, France, ⁵Rheumatology, CHU Pellegrin, Bordeaux, France, BORDEAUX, France, ⁶Internal Medicine, CH de Pau, PAU, France, ⁷Pulmonology, CHU de Dijon, Dijon, France, ⁸Internal Medicine, CHU de Grenoble, Grenoble, France, ⁹CHU de Rouen, Rouen, France, ¹⁰CHU de Brest, Brest, France, ¹¹Nephrology, CHU de Toulouse, Toulouse, France, ¹²CH de Lons le Saunier, Lons le Saunier, France, ¹³Pulmonology, CHU de Rouen, Rouen, France, ¹⁴Internal Medicine department, Clermont-Ferrand, France, ¹⁵CHU, Grenoble, France, ¹⁶foch hospital, foch, France, ¹⁷Internal Medicine, Hôpital Saint Joseph, Marseille, France, ¹⁸Internal medicine, Hôpital Henri-Mondor, Créteil, France, ¹⁹Service de Médecine Interne, Hôpital Cochin, Centre de référence national pour les maladies systémiques autoimmunes rares d’Ile de France, DHU Authors, Assistance Publique-Hôpitaux de Paris (AP-HP), Paris, France, Paris, France, ²⁰Service de Médecine Interne, Centre de Référence Maladies Auto-Immunes et Auto-Inflammatoires Systémiques Rares, Hôpital Cochin, Paris, France

Meeting: 2017 ACR/ARHP Annual Meeting

Date of first publication: September 18, 2017

Keywords: Biologic agents and Churg-Strauss syndrome

Session Information

Date: Monday, November 6, 2017

Title: Vasculitis Poster II: ANCA-Associated Vasculitis

Session Type: ACR Poster Session B

Session Time: 9:00AM-11:00AM

Background/Purpose: Eosinophilic granulomatosis with polyangiitis (EGPA) (Churg–Strauss) is characterized by pulmonary and systemic small-vessel necrotizing vasculitis, vascular and/or extravascular granulomas, eosinophilia and tissue infiltration by eosinophils, occurring in individuals with asthma. Glucocorticoids (GCs) effectively control the disease, but relapses and/or GC-dependence are frequent, leading to immunosuppressant and/or biological therapy use. We examined off-label biological therapy use for relapsing and/or refractory EGPA.

Methods: This retrospective nationwide study included patients with EGPA meeting ACR criteria and/or Chapel Hill Consensus Conference definitions. Treatment efficacy and safety were recorded. Remission was defined as the absence of asthma and/or sinonasal exacerbations and vasculitis manifestations with a prednisone dose ≤7.5 mg/day, and partial response as requiring >7.5 mg of prednisone daily.

Results: Thirty-three patients (22 men, 11 women; median age 50 years) were included. Thirteen (39%) received rituximab (RTX) and 20 (61%) omalizumab (OMA). Previous treatments were: methylprednisolone infusions (54%), oral GCs (100%), IV cyclophosphamide (60%), azathioprine (75%), methotrexate (24%), mycophenolate mofetil (6%), plasmapheresis (6%) and/or co–trimoxazole (33%). RTX was mainly prescribed for pulmonary involvement (92%), sinusitis (69%), peripheral neuropathy (61.5%) and cardiac involvement (46%). OMA was prescribed for pulmonary involvement (90%) and sinusitis (90%). At inclusion, median (range) BVAS for the RTX and OMA groups, respectively, were 4 (0–19) and 2 (0–9). After median follow-up of 20 months, remissions, partial responses and therapeutic failures, respectively, were 38%, 23% and 38% for RTX recipients, and 40%, 20% and 80% for the OMA group. Median BVAS dropped to 0 for both groups. A GC-sparing effect was obtained for both groups but was greater for RTX recipients: median GC dose decreased from the baseline 16 mg/day to 15 at 3 months, 10 at 6 and 12 months and 7.5 at the last follow-up vs. 12.5 mg/day to 11.25 at 3 months, 12 at 6 months, 10 at 12 months and 9 at the last follow-up for the OMA group. Two patients stopped RTX because of severe asthma flares and another because of refractory disease. Eleven patients stopped OMA: 2 because of vasculitis relapses, 2 for severe asthma flares, 4 had refractory disease and 3 achieved remissions. The latter 3 patients then took azathioprine; no relapses occurred after discontinuing OMA. The only minor side effect, nausea, occurred in the RTX group.

Conclusion: The results of this study suggest that RTX and OMA may achieve GC-sparing in relapsing and/or refractory EGPA, but the latter remains frequent.

Disclosure: L. Denis, None; M. Samson, None; F. Maurier, None; C. Khouatra, None; V. Germain, None; X. Delbrel, None; P. Bonniaud, None; A. Deroux, None; N. Girszyn, None; C. de Moreuil, None; D. Chauveau, None; A. Gondouin, None; S. Dominique, None; G. Le Guenno, None; L. Bouillet, None; B. Bonnotte, None; J. E. Kahn, None; B. Bienvenu, None; B. Godeau, None; C. Le Jeunne, None; X. Puéchal, None; L. Guillevin for the French Vasculitis Study Group, None; B. Terrier, None.

To cite this abstract in AMA style:

Denis L, Samson M, Maurier F, Khouatra C, Germain V, Delbrel X, Bonniaud P, Deroux A, Girszyn N, de Moreuil C, Chauveau D, Gondouin A, Dominique S, Le Guenno G, Bouillet L, Bonnotte B, Kahn JE, Bienvenu B, Godeau B, Le Jeunne C, Puéchal X, Guillevin for the French Vasculitis Study Group L, Terrier B. Off-Label Use of Biological Therapies in Relapsing and/or Refractory Eosinophilic Granulomatosis with Polyangiitis (Churg–Strauss) [abstract]. Arthritis Rheumatol. 2017; 69 (suppl 10). https://acrabstracts.org/abstract/off-label-use-of-biological-therapies-in-relapsing-andor-refractory-eosinophilic-granulomatosis-with-polyangiitis-churg-strauss/. Accessed .

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