Therapeutic advances in eosinophilic granulomatosis with polyangiitis

Abstract

Purpose of reviewIn recent years, therapeutic advances in eosinophilic granulomatosis with polyangiitis (EGPA) have changed our treatment paradigm. This review will summarize and discuss updates in management of EGPA, with a particular focus on biologic therapies. Recent findingsThe anti-interleukin (IL)-5 agent mepolizumab (the first FDA-approved drug specifically for EGPA) is effective in induction and maintenance of remission particularly in patients with predominantly asthma and allergic manifestations, though efficacy in ANCA-positive, vasculitic disease is unclear; additional anti-IL-5 agents are under study. Rituximab is currently recommended for remission induction in severe disease, particularly in ANCA-positive patients with vasculitic manifestations, though the supportive evidence is mostly observational. Evidence supporting use of traditional DMARDs and other biologic agents such as omalizumab remains limited and observational.

Biologics for eosinophilic granulomatosis with polyangiitis

“…In other words, ACR guidelines suggest a very careful approach to mepolizumab treatment, by restricting its recommended use to specific diseases phases and severity grades. This probably reflects the lack of strong evidence supporting its relevance as a single treatment in inducing EGPA remission, as well as the need to consider in the acute phase the coexistence of autoimmune mechanisms besides the eosinophilic driven inflammation which deserve to be specifically treated by immunosuppressant drugs [7 ▪ ,30]. The real-word evidence, as discussed below, also provides quite controversial data in terms of mepolizumab dose, timing, and clinical profile of best responder patients.…”

The new indications for biologicals in type 2 diseases: perspectives

“…Taken together, the evidence coming from the trial by Wechsler and colleagues supports MEP as a valuable treatment option for EGPA; on the other side, a not negligible proportion of patients in the active arm did not reach the identified outcomes as per protocol. The complexity of EGPA immunological background, the intra-individual variability of clinical manifestation and burden related to the specific disease phase as well as the inter-individual variability in terms of EGPA pathophysiologic features and clinical presentation might certainly account for the controversial issues emerging from the mentioned trial [68,70]. Under that perspective, a clustered subanalysis of responder and nonresponders could be helpful, but at the moment, it is not provided.…”

Successful treatment with spinal cord stimulation for pain due to eosinophilic granulomatosis with polyangiitis: a case report

“…The clinical manifestations tend to segregate into eosinophilic disorder and vasculitis disorder, depending on the ANCA status [1,2], with the former being more prevalent in the ANCA-negative population and the latter more prevalent in the ANCA-positive population [1,2]. The manifestations are wide-ranging, both those induced by eosinophilic non-vasculitis, such as asthma, rhinosinusitis, peripheral and tissue eosinophilia, and cardiomyopathy, and that induced by vasculitis, such as purpura, glomerulonephritis, and mononeuritis multiplex [3]. The prevalence of peripheral neuropathy in EGPA patients is reportedly between 46 and 77% [4,5].…”

“…Patients with EGPA have historically been treated by cyclophosphamide, glucocorticoids, and sometimes antirheumatic drugs, such as azathioprine, methotrexate, and mycophenolate mofetil, depending on the severity [3]. Mepolizumab, an antiinterleukin-5 agent, was the first FDA-approved agent, and other biologic agents have since been developed for the treatment of EGPA [3].…”

“…Patients with EGPA have historically been treated by cyclophosphamide, glucocorticoids, and sometimes antirheumatic drugs, such as azathioprine, methotrexate, and mycophenolate mofetil, depending on the severity [3]. Mepolizumab, an antiinterleukin-5 agent, was the first FDA-approved agent, and other biologic agents have since been developed for the treatment of EGPA [3]. Although most patients experience a reduction in pain and improvement in their strength within several weeks to months of the initiation of immunotherapies, some suffer from residual neuropathic symptoms for a long time, even after EGPA remission [5].…”