Background/Purpose: Eosinophilic Granulomatosis with Polyangiitis (EGPA) is a rare systemic small-vessel necrotizing vasculitis presenting with asthma and eosinophilia. Anti-neutrophil cytoplasmic antibodies (ANCA) association may influence disease presentation, treatment and outcomes. Rituximab (RTX) is effective in ANCA-associated vasculitis (AAV), but specific potential benefits in EGPA are less clear. Difficult-to-treat or glucocorticoid (GC) dependent asthma is one of the main challenges in EGPA management. Our aim was to characterize asthma control and GC dependency in EGPA patients treated with RTX.

Methods: Retrospective cohort study of patients diagnosed with EGPA between 2000 and 2017 presenting with GC dependent asthma and treated with RTX for remission induction in a single center. Standardised data collection was performed, including disease activity assessment, RTX treatment, asthma characterization and control.

Results: A total of 17 patients (52.9% men) were included. According to BVAS/WG, lower respiratory tract was most commonly affected at presentation (16, 94.1%), followed by nervous system (15, 88.2%) and ENT (14, 82.4%). There were no differences in disease extent index (DEI). Five Factor Score was≥ 1 in 11 patients (64.7%). At diagnosis, the majority were myeloperoxidase (MPO)-ANCA positive (13, 76.5%). Non-controlled asthma symptoms were present in 13 (76.5%) patients. Atopy was present in 13 (76.5%) and radiographic changes in 8 (47.1%).

RTX was used in three different clinical scenarios: for initial remission induction (5, 29.4%) in patients with new onset of severe disease (BVAS/WG>3), after failed remission induction attempts with other immunosuppressants (12, 70.6%) or for remission maintenance (9, 52.9%). GC were used in all maintenance regimens at a median dose of 25 (16.25 – 37.5) mg/day.

Median time of follow-up since EGPA diagnosis was 38 (17-107) months. Three patients (17.6%) relapsed after RTX and 6 (35.3%) were refractory to RTX. As maintenance treatment in 9 (52.9%) patients, RTX choice was guided mainly by B-cell reconstitution during the follow-up (7, 41.2%). At the end of follow-up, remission was achieved in 15 (88.2%) patients. After induction with RTX, more than one asthma exacerbation occurred in 8 (47.1%) patients but the use of bronchodilators was not significantly different after the relapse. Mepolizumab was used for refractory symptomatic asthma in 2 patients (11.8%), after RTX effect worn off.

At the end of follow-up, median serum eosinophils and C-reactive protein were lower than before RTX treatment (0.10 vs. 1.06 x10⁹/L, p=0.012; 5 vs. 27 mg/dL, p=0.001; respectively). Pulmonary function tests (PFTs) remained unchanged. Induction treatment with RTX allowed a more predictable GC tapering: to a median dose of 5 mg/day at 12 months and a rise from 12 to 24 months was seen (no GC pattern pre RTX treatment). However, in the patients on maintenance treatment with RTX, asthma symptom control was not dependent on GC at 12 months.

Conclusion: RTX seems a safe drug with some GC sparing efficacy in EGPA. Improvement in inflammatory markers and eosinophilia was observed, but not in PFTs. RTX deserves more detailed studies in prospective randomized controlled clinical trials in EGPA.

« Back to 2018 ACR/ARHP Annual Meeting

ACR Meeting Abstracts - https://acrabstracts.org/abstract/asthma-in-eosinophilic-granulomatosis-with-polyangiitis-treated-with-rituximab/