EGPA diagnosis delays, steroid reliance seen in England real-world study
Median time from first recorded major manifestation to diagnosis was 44 months
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People with eosinophilic granulomatosis with polyangiitis (EGPA) waited a median of nearly four years from their first recorded major sign of the disease to diagnosis, and most received prescriptions for oral glucocorticoids and/or immunosuppressants, a real-world study in England shows.
Also, in a 486-patient group followed from diagnosis, more than one-quarter experienced a new EGPA manifestation within six months, “reflecting challenges in achieving early disease control,” the researchers wrote.
“This study emphasizes the substantial clinical burden and reliance on glucocorticoids in EGPA, highlighting the need for improved diagnosis” and “[oral glucocorticoid]-sparing strategies to reduce toxicity risk,” they added.
Study points to need for earlier EGPA diagnosis
The study, “Epidemiology, clinical management and outcomes in patients with eosinophilic granulomatosis with polyangiitis in England: A retrospective observational cohort study,” was published in the Journal of Allergy and Clinical Immunology: Global by an international team of researchers. The study was sponsored by AstraZeneca, which markets Fasenra (benralizumab), a therapy approved in the U.S. for adults with EGPA. Several authors were employees or contractors of the company at the time the study was conducted.
EGPA is a rare type of ANCA-associated vasculitis, a group of diseases whose causes involve an abnormal immune response tied to self-reactive antibodies, called ANCAs, that can lead to inflammation and damage in small blood vessels.
This rare form is marked by the formation of immune cell clumps, called granulomas, and high levels of eosinophils, a type of immune cell, in the blood. EGPA typically affects the lungs and upper respiratory tract, but may also involve other organs, including the heart, kidneys, gastrointestinal tract, and nervous system — contributing to a substantial clinical burden.
However, “real-world data on EGPA epidemiology and clinical burden are limited, underscoring the need for further studies,” the researchers wrote. Epidemiology refers to the study of the distribution, patterns, and determinants of health and disease conditions in defined populations.
To learn more, the team retrospectively analyzed real-world data from EGPA patients diagnosed between January 2006 and February 2019 who were part of a database in England.
Most clinical findings came from 486-patient cohort
A total of 2,349 patients were identified, of whom 729 were included to estimate EGPA prevalence (total number of cases), and 486 were included in the incident EGPA group, meaning those with a first recorded EGPA diagnosis during the study period. Incidence refers to the number of new cases over a given period.
Most clinical characteristics, treatment patterns, and outcomes were assessed in the 486-patient incident EGPA cohort. Patients’ mean age at diagnosis was 57.9 years, and they were followed for a mean of 5.4 years.
EGPA incidence was 3.04 new cases per million person-years (PY) — a measure that combines the number of patients and their observation time. This rate did not vary significantly across the study period or between sexes.
The overall disease prevalence was 2.7 cases per 100,000 people, increasing steadily from 1.9 in 2006 to 3.1 in 2015, and remaining stable thereafter.
Most patients in the incident EGPA group had at least one coexisting condition. The most common was obstructive airway disease (85.2%), which included asthma (79.8%).
The median time from the first recorded major disease manifestation to EGPA diagnosis was 44 months, or nearly four years. These manifestations included asthma diagnosis or nasal polyps, which are noncancerous growths inside the nose or surrounding air-filled cavities.
Most people (86.6%) had at least one specialist visit in the year before EGPA diagnosis, most commonly with respiratory medicine specialists (46.9%).
At diagnosis, 54.3% of the patients were classified as having stable disease, while the others were either in remission, meaning little to no symptoms, or experiencing a relapse, meaning symptoms had returned after remission. “Over time, stable disease remained the most common state, while fewer patients achieved or maintained remission,” the researchers wrote.
New EGPA manifestations seen after diagnosis
In the first six months after diagnosis, more than one-quarter of patients (26.1%) experienced a new EGPA manifestation. The proportion of participants with asthma exacerbations, or sudden worsening, dropped from 13% in the six months before diagnosis to 6.2% in the six months after diagnosis, remaining stable thereafter.
The proportion of patients with recorded persistent damage in at least one organ increased from 2.9% in the six months after diagnosis to 28.6% in the four to five years after diagnosis.
The most frequent recorded treatment was oral glucocorticoids (86.2%), followed by immunosuppressants (53.7%). Among patients prescribed oral glucocorticoids, 27% were successfully tapered, while 43.3% “were categorized as not tapered or having unsuccessful tapering outcomes,” the researchers wrote.
The disease prognosis was evaluated using the Five-Factor Score (FFS), in which higher scores indicate a poorer prognosis. While most patients (76.3%) were considered to have a low risk of poor outcomes according to the 1996 FFS version, only 26.1% were classified as having such a low risk with the updated 2009 version.
Also, 20.2% of patients died after a median follow-up of 4.8 years, corresponding to a death rate of 37.1 per 1,000 person-years. The most common causes of death included EGPA (11.2%), chronic ischemic heart disease (a condition where the heart muscle is deprived of oxygen; 6.1%), lung cancer (6.1%), and heart attack (5.1%).
Together, these findings suggest that many people with EGPA experience substantial diagnostic delays and rely heavily on glucocorticoid treatment. “As such, there is an urgent and critical need for improved diagnostic pathways and more effective new treatments,” the team concluded.
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