Optimal therapy and prospects for new medicines in eosinophilic granulomatosis with polyangiitis (Churg-Strauss syndrome)

Abstract

At present, EGPA conventional therapy is by default similar to that of other AAVs. Limited, non-severe EGPA can initially be treated with glucocorticoids (GCs) alone. Patients with life-threatening manifestations and/or major organ involvement must receive a combination of GCs and an immunosuppressant, mainly cyclophosphamide. Remission can be achieved in >85% of patients with these first-line treatments, but vasculitis relapses occur in more than one-third of patients, and about 85% cannot stop GC treatment because of GC-dependent asthma and/or ENT manifestations. A few biologic agents, including rituximab or mepolizumab, are now under investigation after interesting preliminary results. Expert commentary: Treatment for EGPA still has several unmet needs. Several biologic agents are now under investigation in randomized controlled trials, but a few others should be considered soon. Their benefit should be demonstrated for devising more EGPA-tailored therapeutic strategies (ideally GC-free).

Co‐morbidities in severe asthma: Clinical impact and management

“…Treatment of EGPA requires systemic corticosteroids, usually in combination with immunosuppressants (e.g. cyclophosphamide, methotrexate or azathioprine) . Furthermore, some patients may benefit from treatment with monoclonal antibodies such as Rituximab and anti‐IL‐5 in particular appears to be a promising future treatment option for these patients …”

“…cyclophosphamide, methotrexate or azathioprine). 120 Furthermore, some patients may benefit from treatment with monoclonal antibodies such as Rituximab and anti-IL-5 in particular appears to be a promising future treatment option for these patients. 120…”

Scheduled rituximab maintenance reduces relapse rate in eosinophilic granulomatosis with polyangiitis

“…Nine patients received RTX-maintenance, the others received RTX only for relapses (a single, 1g infusion). Complete remission was defined as Birmingham Vasculitis Activity Score (BVAS)=0 and prednisone ≤7.5 mg/day,3 partial remission as >50% BVAS reduction and prednisone ≤15 mg/day. Refractory disease was defined as failure to achieve remission, relapse as the recurrence/new onset of clinical manifestations requiring intensification of immunosuppression or increase in glucocorticoid dose >50% 8…”

Eosinophilic granulomatosis with polyangiitis (formerly known as Churg-Strauss syndrome) as a differential diagnosis of hypereosinophilic syndromes

“…In patients with FFS ≥2 or severe organ dysfunction (e.g., heart and central nervous system), intravenous cyclophosphamide plus intravenous high dose glucocorticoids (methylprednisolone 1000 mg for three days) have been proposed [34], [35], [36]. In refractory disease, the use of rituximab (anti-CD20 monoclonal antibody) [37], [38], [39], [40] and mepolizumab (anti-IL-5 monoclonal antibody) has been reported with success [41], [42], [43].…”