Background/Purpose: To evaluate the efficiency and safety of IFN-alpha in induction and maintenance of remission in patients with EGPA and to describe its effects on lung function tests and corticosteroid (CS) tapering
Methods: Retrospective, single-center cohort study in patients with EGPA (according to ACR criteria), who were insufficiently controlled with CS and other immunosuppressant therapies (AZA, MTX, MMF). Patients were treated with Peg-IFN-alpha (135µg per week) or IFN-alpha2b (3 x 9 million units per week) for induction and maintenance of remission. Remission was defined as a Birmingham Vasculitis Activity Score (BVAS) of 0. After achieving remission, time to first relapse and total occurrence of relapses were recorded. Patients received lung-functioning tests before treatment and at time of remission. Adverse events were recorded.
Results: 25 patients, age of 50 (±9.5) years were evaluated for induction of remission and in 21 patients IFN was continued for maintenance of remission. Five-factor-score (FFS) at initiation of treatment was 0 in 18 (72%) and ≥1 in 7 (28%) in patients. Mean BVAS at initiation of treatment was 8.3 (2-21). Previous therapies were CYC (N=4), AZA (N=3), Omalizumab (N=3), MTX (N=1), MMF (N=1), Rituximab (N=1).
Treatment was discontinued in 18 of 25 (72%) patients and 7 (28%) were still on IFN after a mean time of treatment of 31 (2-131) months. Of 25 patients, 21 (84%) achieved remission and mean time to remission was 5 (3-12) months. CS were tapered from 18.75 (5-50) mg per day to 5 (0-30) mg per day at time of remission (p<0.001).
FEV1 increased from 74.8 (25-102) % to 89.1 (50-133) % at time of remission (p=0.001).
Four patients discontinued therapy before remission was achieved due to side effects (N=3) and treatment failure (N=1). Of 21 patients treated for maintenance of remission, 8 (38%) were still in remission after a median time of 21 (4-57) months without relapsing. 13 (62%) suffered a total of 15 relapses (4 major and 11 minor) after a median of 12 (4-42) months. Most relapses were associated with rapid CS-withdrawal.
Adverse events were frequent and forced discontinuation of treatment in 11 of 18 patients: depression (N=4), polyneuropathy (N=3), autoimmune hepatitis, toxic liver damage, anemia, alopecia and chronic nausea (each N=1). Other adverse events occurred as flu-like-symptoms (76%), leucopenia (44%) and thrombopenia (36%) but were transient and did not require a change in treatment regime. 4 of 18 patients IFN discontinued treatment due to lack of efficacy in remission induction (N=1) or prevention of relapse (N=3).
Conclusion: IFN is effective in both induction of remission and maintenance of remission while depression is the main side effect that limits therapy. As IFN showed good effect on CS-tapering and on asthma in particular, it can be considered as an alternate treatment in EGPA patients without life- or organ-threatening manifestations.
Disclosure:
B. Seeliger,
None;
M. Foerster,
None;
A. Moeser,
None;
J. Happe,
None;
C. Kroegel,
None;
T. Neumann,
None.
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ACR Meeting Abstracts - https://acrabstracts.org/abstract/efficacy-and-safety-of-ifn-alpha-in-induction-and-maintenance-of-remission-in-patients-with-eosinophilic-granulomatosis-with-polyangiitis-egpa-single-center-observational-study/