Anti‐IgE Monoclonal Antibody (Omalizumab) in Refractory and Relapsing Eosinophilic Granulomatosis With Polyangiitis (Churg‐Strauss): Data on Seventeen Patients

Abstract

Objective. To describe the efficacy and safety of omalizumab, an anti-IgE monoclonal antibody, in patients with refractory and/or relapsing eosinophilic granulomatosis with polyangiitis (Churg-Strauss) (EGPA).Methods. We conducted a nationwide retrospective study including EGPA patients who received omalizumab. Response was defined as the absence of asthma and/or sinonasal exacerbations with a prednisone dosage of £7.5 mg/day (complete response) or >7.5 mg/day (partial response).Results. Seventeen patients (median age 45 years) received omalizumab for severe steroiddependent asthma (88%) and/or sinonasal involvement (18%). After a median follow-up of 22 months, 6 patients (35%) achieved a complete response, 5 patients (30%) achieved a partial response, and 6 patients (35%) had no improvement. The median Birmingham Vasculitis Activity Score decreased from 2.5 at baseline to 0.5 at 12 months. The median number of exacerbations per month decreased from 1 at baseline to 0 at 12 months, and the median forced expiratory volume in 1 second increased from 63% of the percent predicted at baseline to 85% of the percent predicted at 12 months. The median prednisone dosage decreased from 16 mg/day at baseline to 11 mg/day at 6 months and 9 mg/day at 12 months. Omalizumab was discontinued in 8 patients

Biological therapy improves myocarditis and disease activity in eosinophilic granulomatosis with polyangiitis patients

“…Another RTX maintaining therapy with a regimen of 500 mg fortnightly at 6, 12 and 18 months, has also demonstrated a sustained remission with overall survival superior to the azathioprinebased protocol in newly diagnosed or relapsing AAV [28]. Despite the exclusion of EGPA from AAV in above studies, therapeutic effects of RTX at induction in refractory or relapsing EGPA have been shown in 2 larger trials with 1 gram fortnightly and 375 mg/m 2 weekly × 4 regimens [20,29]. Furthermore, a regimen with 1 gram fortnightly every 6 months has been observed to be effective as maintenance treatment [29].…”

“…Case no. 16 acquired OMA under 150 mg bi-weekly subcutaneous injection due to relapsing disease at the age of 12 [20], and Ben with 30 mg quadri-weekly subcutaneous injection at the age of 16 with a 100% inhibition of the baseline eosinophil counts due to disease relapse [12]. For those receiving biologics, there were complete remission in 5 (cases no.…”

Vasculitis research: Current trends and future perspectives

“…Two landmark trials, the RITUXVAS and the RAVE trials, established the comparative efficacy (both short‐ and long‐term) of rituximab‐based regimens vs cyclophosphamide‐based regimens for remission induction in AAV, with a suggestion that rituximab may be a better option for induction of remission in relapsing disease in the short‐ and intermediate‐term, but not in the longer‐term . EGPA remains a subset of AAV that is relatively more difficult to treat, and recent literature has suggested promise with the use of rituximab,, IFN‐α and omalizumab as first‐line or reserve agents in patients with this rarer form of AAV. A recent French multicentric trial, the CHUSPAN study, did not reveal benefit of addition of azathioprine (vs placebo) to corticosteroids for the induction of remission in 95 patients with EGPA, MPA or PAN of lesser severity .…”

Immunotherapy in Eosinophilic Granulomatosis with Polyangiitis: A New Step Forward?

“…The introduction of anti-IgE therapy for asthma inaugurated the era of biological therapies and has been shown to be useful for patients with allergic severe asthma [35,36]. EGPA patients have also been treated with omalizumab with conflicting results [37,38], some patients presenting vasculitis onset after omalizumab therapy [39,40]. As now accepted with leukotriene antagonists, it is likely that omalizumab may have unmasked a preexisting vasculitis in some cases but additional data are necessary before concluding that omalizumab has no role in these complications.…”