EGPA linked to high disease burden, irrespective of clinical setting
Burden similar for those treated at private practices, academic centers
Eosinophilic granulomatosis with polyangiitis (EGPA) patients treated at private practices in the U.S. have high disease burden associated with multiple symptoms and simultaneous health conditions, a study showed, mirroring findings reported for patients treated at academic settings.
Patients treated in private practice demonstrated variable responses to standard treatments, with some patients continuing to experience uncontrolled disease and relapses.
“Understanding the disease burden across multiple clinical settings will allow the opportunity for earlier recognition of disease, potentially shortening delays to diagnosis and allowing earlier involvement of multidisciplinary team support, while also mitigating ongoing risk of relapse and organ damage,” the researchers wrote.
The study, “Eosinophilic Granulomatosis With Polyangiitis: Patient Profiles From a Large US Allergy Practice,” was published in the Journal of Allergy and Clinical Immunology: Global. The researchers said it was the first “to assess the characteristics and burden of patients with EGPA outside of academic referral centers.”
EGPA is a rare form of ANCA-associated vasculitis, a group of autoimmune conditions that cause inflammation in small blood vessels. This rare type is typically marked by asthma and high levels of eosinophils, a type of immune cell. EGPA may affect the lungs, gastrointestinal tract, nervous system, as well as ears, nose, and throat and other organs.
Private-practice experience not widely studied
Previous studies have indicated the disease is associated with a high burden regarding treatment and the use of healthcare resources, but “little is known about how EGPA is diagnosed and managed in private practice and whether patient presentation and burden of disease differ from academic centers,” the researchers wrote.
The team retrospectively analyzed data from 52 EGPA patients receiving treatment through the Allergy Partners network, a specialty care provider in allergy and immunology in the U.S.
Sixty-three percent of the patients were female, 65% were from southern U.S. states, and 56% had commercial health insurance. Some 75% received a diagnosis outside the specialized clinic and were referred by primary care providers, pulmonologists, or another allergist or immunologist.
Patients had a mean age of 50.2 at diagnosis and 53.1 at the time of evaluation in private practice, and were followed for a mean of 3.7 years.
Each patient received care from a median of four specialists, with the most common being allergists/immunologists (100%), followed by those specialized in conditions affecting the ears, nose, and throat (60%).
The most common simultaneous conditions among patients included diseases of the circulatory system (98%), the respiratory system, including asthma (96%), and gastrointestinal system (40%).
Patients self-reported a mean of about 18 distinct symptoms. All reported symptoms affecting the nose and related air-filled spaces, rash, and chest pain, while 98% noted fatigue and itching.
Treatment mainly included oral corticosteroids (85%) and the EGPA-targeted therapy Nucala (mepolizumab; 60%). Fasenra (benralizumab), a more recently approved EGPA-targeted treatment, was given to 12% of the patients. Twenty-five percent were treated with immunosuppressive agents, most commonly azathioprine.
Results showed that 75% of patients responded to treatment — defined as physician-reported symptom lessening or control — after a median of 5.6 months. The first response lasted for a median of 19.5 months (about 1.5 years) before the next relapse or end of follow-up.
Most of the responders had been treated with corticosteroids (87%) and Nucala (64%).
Nearly half of the patients (46%) achieved a controlled disease status — meaning the symptoms were controlled after being active, worsened, or unchanged — at a median of 16.2 months and for a median of 9.7 months. More than one-third of these patients (38%) subsequently experienced a relapse.
Fifteen percent of the patients were hospitalized at least once, for a mean of 4.6 days per hospitalization. All patients had outpatient visits, and 4% required at least one emergency care. Similar results were seen for all-cause or EGPA-related visits.
“Results reveal complex patient journeys, marked by a significant disease burden featuring high [rates of simultaneous conditions] and numerous symptoms, with [variable] response to standard treatments, and underutilized targeted therapies,” the researchers wrote.
When compared with data from prior retrospective EGPA studies in academic settings, “patients with EGPA experience high disease burden irrespective of clinical setting,” the team wrote.
The study “highlights the continued need for earlier disease recognition and management, and optimized and individual treatment strategies within a coordinated multidisciplinary team approach,” the researchers concluded.
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