Concurrent Use of Mepolizumab and Rituximab for Eosinophilic Granulomatosis With Polyangiitis and Multisystem Involvement

Abstract

Eosinophilic granulomatosis with polyangiitis (EGPA), formerly Churg-Strauss, is an anti-neutrophil cytoplasmic antibody (ANCA)-associated autoimmune vasculitis, involving small- and medium-sized arteries, which could involve several organs. This rare syndrome can present with a myriad of symptoms, which may make diagnosis challenging. It has been suggested that there are variants of EGPA, which may respond differently to available modes of treatment. Multiple and different mechanisms may be at play in each case of EGPA. This may influence the decision of clinicians to combine treatment strategies as done in this case. The addition of immunosuppressive agents other than high-dose steroids may mitigate end-organ damage, facilitate faster recovery, and prevent relapse. Rituximab among others has been seen to provide better outcomes, including a lower incidence of relapse. Mepolizumab was approved by the Food and Drug Administration (FDA) in 2017 for the treatment of EGPA. Administered at a higher dose than approved for severe eosinophilic asthma, it has been shown to lengthen remission in EGPA. The optimal dose and duration of therapy with mepolizumab remain unclear. The rarity alone of EGPA creates room for further investigation regarding pathogenesis, outcome over time, and treatment strategies, which may vary depending on how an individual case presents. This case describes the course of a 55-year-old woman who presented with respiratory symptoms, pauci-immune necrotizing granulomatous nephropathy, and neuropathy secondary to P-ANCA-positive EGPA who was successfully treated with rituximab and mepolizumab, in addition to glucocorticoids.

Switching from omalizumab to mepolizumab therapy improved extra‐pulmonary abdominal and cutaneous vasculitis symptoms in a patient with eosinophilic granulomatosis with polyangiitis

“…Recently, Menzella et al described remarkable improvements in asthma and ear–nose–throat symptoms in an EGPA patient after switching from mepolizumab to benralizumab, which was also consistent with the results reported by Bormioli et al 27,28 These might result from a better ability of benralizumab to reduce the number of eosinophils in various tissues by 90%–100% 10,27 . Moreover, some studies suggest that concurrent use of mepolizumab and rituximab may be more beneficial for the treatment of ANCA‐positive EGPA patients 11,29,30 . Therefore, the relative efficacy of biologics in each patient may be influenced by the multiple and different mechanisms involved 30 …”

“… 10 , 27 Moreover, some studies suggest that concurrent use of mepolizumab and rituximab may be more beneficial for the treatment of ANCA‐positive EGPA patients. 11 , 29 , 30 Therefore, the relative efficacy of biologics in each patient may be influenced by the multiple and different mechanisms involved. 30 …”

“… 11 , 29 , 30 Therefore, the relative efficacy of biologics in each patient may be influenced by the multiple and different mechanisms involved. 30 …”

Clinical Features of Patients with Active Eosinophilic Granulomatosis with Polyangiitis Successfully Treated with Mepolizumab

“…Other published studies support the use of mepolizumab for induction and maintenance of remission in refractory, relapsing, or glucocorticoid-dependent EGPA [36-39]. There have been 2 reports of the simultaneous administration of RTX and mepolizumab in refractory cases [40, 41], but in those reports, it was difficult to evaluate the effect of mepolizumab. Moreover, it has not been conclusively determined whether mepolizumab treatment is effective against various vasculitis signs in EGPA or in ANCA-positive cases [42].…”

Sequential rituximab and mepolizumab in eosinophilic granulomatosis with polyangiitis (EGPA): a European multicentre observational study

“…Based on the idea that combining treatments with complementary mechanisms of action might induce and maintain remission of both disease components,6 7 we investigated the efficacy and safety of a regimen based on sequential rituximab and mepolizumab for the control of EGPA.…”