FRI0278 OFF-LABEL USE OF BIOLOGICAL THERAPIES IN RELAPSING AND/OR REFRACTORY EOSINOPHILIC GRANULOMATOSIS WITH POLYANGIITIS (CHURG-STRAUSS)
Authors: Alice Canzian, Nils Venhoff, Silvia Sartorelli, Anne Marie Ruppert, Matthieu Groh, Camille Taille, Virginie Rieu, Perrine Smets, Francois Maurier, Nicolas Girszyn, Maxime Samson, Claire De Moreuil, Gregory Pugnet, Xavier Delbrel, Jean-Emmanuel Kahn, Xavier Puéchal, Giacomo Emmi, Loïc Guillevin, Lorenzo Dagna, Jens Thiel, Augusto Vaglio, Benjamin Terrier
Journal: Annals of the Rheumatic Diseases (2019)

<h3>Background:</h3> Eosinophilic granulomatosis with polyangiitis (EGPA), formerly named Churg-Strauss syndrome, is a small-vessel necrotizing vasculitis associated with eosinophilia and asthma. Glucocorticoids (GCs) usually control the disease, but relapses and GC-dependant asthma are frequent, leading to potential biological therapy use. <h3>Objectives:</h3> We examined off-label biological therapy use for relapsing/refractory EGPA. <h3>Methods:</h3> Remission was defined as the absence of asthma and vasculitis manifestations with a prednisone dose ≤5 mg/day, and partial response as the absence of manifestations requiring prednisone dose between 6 and 10 mg/day. <h3>Results:</h3> One hundred and eigteen patients (66 men, 52 women; median age 50.5 years) were included. Fifty (42%) patients received rituximab (RTX), 38 (32%) mepolizumab (MEPO), and 30 (26%) omalizumab (OMA). Previous treatments were: oral GCs (98%), methylprednisolone infusions (51%), azathioprine (68%), cyclophosphamide (47%), methotrexate (30%), mycophenolate mofetil (8%). At inclusion, median (interquartile range) BVAS in the RTX, OMA and MEPO groups were 8 (4.5-13), 2 (1.5-4) and 2 (2-5), respectively, median (IQR) daily GCs dose were 20 mg/day (15-40), 20 mg/day (10-37.5), 10 mg/day (7.5-20). GC-dependant asthma was found in 39 (78%) of the RTX group, 36 (95%) in the MEPO group and 28 (93%) in the OMA group. After median follow-up of 22.8 months (IQR 10-47), remissions, partial responses and therapeutic failures, respectively, were noted in 50%, 16% and 34% for RTX recipients, 17%, 38% and 45% for the OMA group and 84%, 3% and 13% for the MEPO group. Median BVAS dropped to 0 at 6 and 12 months and at last follow-up in all groups. A GC-sparing effect seemed more important with RTX and MEPO. Median GCs dose decreased from the baseline 20 mg/day to 8.5 at 6 months, 7.5 at 12 months and 5 at last follow-up in the RTX group, from 20 mg/day to 12 at 6 months, 10 at 12 months and 10 at last follow-up in the OMA group, and from 10 mg/day to 5 at 6 months, 3 at 12 months and 5 at last follow-up in the MEPO group. In the MEPO group, no difference was noted between patients receiving 100 mg and those 300 mg monthly.Nine (18%) patients stopped RTX because of refractory disease, and 12 (24%) experienced adverse events, including severe infections in 5. Thirteen (43%) stopped OMA because of severe infusion reaction in one and refractory disease in 12, and 4 (13%) patients receiving OMA experienced adverse events. Three (8%) patients stopped MEPO because of adverse events in 2 (one severe infusion reaction and one because of paresthesia), because of pregnancy in one. Seven (18%) additional patients receiving MEPO experienced adverse events, mainly asthenia. <h3>Conclusion:</h3> The results suggest that RTX may be effective in 50% of patients with vasculitis relapses related to EGPA, with an acceptable safety profile, while MEPO is highly effective with a good GCs-sparing effect and safety profile in patients with steroid-dependant asthma. <h3> Disclosure of Interests:</h3> Alice Canzian: None declared, Nils Venhoff: None declared, Silvia Sartorelli: None declared, Anne Marie Ruppert: None declared, Matthieu Groh: None declared, Camille Taille: None declared, Virginie Rieu: None declared, Perrine Smets: None declared, Francois Maurier: None declared, Nicolas Girszyn: None declared, Maxime Samson: None declared, Claire De Moreuil: None declared, Gregory Pugnet: None declared, Xavier Delbrel: None declared, Jean-Emmanuel Kahn: None declared, Xavier Puéchal: None declared, Giacomo Emmi: None declared, Loïc Guillevin: None declared, Lorenzo Dagna Consultant for: Prof Lorenzo Dagna received consultation honoraria from Abbvie, Amgen, Biogen, Bristol-Myers Squibb, Celltrion, Novartis, Pfizer, Sanofi-Genzyme, and SOBI., Jens Thiel: None declared, Augusto Vaglio: None declared, Benjamin Terrier: None declared