Living with Eosinophilic Granulomatosis with Polyangiitis (EGPA): Podcast of a Patient-Physician Discussion.
Autumn Smith, Michael E Wechsler
Abstract
Open AccessFor people living with eosinophilic granulomatosis with polyangiitis (EGPA), the journey to diagnosis can be long and complicated. EGPA is a rare inflammatory disorder, typically characterized by high levels of blood eosinophils and vasculitis in the lungs and/or other end organ(s). It is associated with asthma, pulmonary infiltrates, neuropathy, sinus disease and the presence of anti-neutrophil cytoplasmic antibodies. Patients can present with a number of symptoms and complications, which often go unrecognized or misdiagnosed and can result in considerable delays in appropriate treatment and worsening of underlying disease. Following correct diagnosis, patients are often treated with oral corticosteroids and other immunosuppressive drugs. While these treatments can be effective at controlling disease, they are also associated with significant side effects, which can lead to diminished quality of life. The emergence in recent years of anti-interleukin-5/receptor (anti-IL-5/R) therapies, such as benralizumab and mepolizumab, has revolutionized the management of EGPA. Interleukin-5 (IL-5) is a key cytokine in the maturation, proliferation and activation of eosinophils. Anti-IL-5/R therapies work by binding IL-5 or the receptor for IL-5, thereby significantly decreasing eosinophilic inflammation, the key pathogenic driver in EPGA. Not only are anti-IL-5/R therapies effective at controlling disease, but they are also well tolerated and can facilitate corticosteroid tapering. In this podcast, a patient and physician discuss the lived experience of EGPA, including difficulties with diagnosis, impact of disease on daily life and disease management with both traditional treatments and with the newer anti-IL-5/R therapies. The discussion highlights the importance of EGPA awareness and of physicians recognizing seemingly unconnected symptoms and different disease components, as well as the need to minimize corticosteroid use because of long-term risks. Furthermore, access to an experienced and coordinated healthcare team and establishing a strong support network are emphasized as essential to help patients manage this complex and rare disease. Podcast Audio (MP4 168118 KB).