The prevalence, burden of disease, and healthcare utilization of patients with eosinophilic granulomatosis with polyangiitis in Japan: a retrospective, descriptive cohort claims database study

Abstract

Objectives To estimate eosinophilic granulomatosis with polyangiitis (EGPA) prevalence and disease burden in patients with newly diagnosed EGPA in Japan. Methods This retrospective descriptive cohort study (GSK ID: 209751, HO-18-19652) used administrative claim data from patients (aged ≤74 years) with EGPA (study period: January 1, 2005–December 31, 2017), identified from their first ICD-10 code for EGPA (index). Data were examined during the 12 months before (baseline) and 12 months following the index date (follow-up). EGPA prevalence, respiratory comorbidities, all-cause healthcare utilization, and oral corticosteroid (OCS) use were assessed. Results EGPA prevalence (95%CI) increased from 4.2 (0,23.7)/million people (2005) to 38.0 (31.8,45.1)/million people (2017), was generally more common in females versus males, and increased with age. Of the 45 patients with newly diagnosed EGPA, 57.8% had acute bronchitis and 42.2% had upper respiratory tract infections during baseline. During follow-up, 60.0% of patients were hospitalized at least once and 77.8% used OCS (OCS dependent [≥80% of days]: 73.1%). Conclusions In Japan, EGPA prevalence increased over time, was generally more common in females, and increased with patient age. EGPA burden was high; respiratory comorbidities were common, and most patients required hospitalization and OCS use. Our data suggest additional EGPA treatment options are needed.

Characteristics and Disease Burden of Patients With Eosinophilic Granulomatosis With Polyangiitis Initiating Mepolizumab in the United States

“…As expected, based on the diagnostic criteria for EGPA, which includes obstructive airway disease and nasal polyps, 22 the most common manifestations for patients in the current study were asthma, chronic sinusitis, and allergic rhinitis, largely consistent with previous real-world studies 6,7,9 . Despite the frequency of manifestations, particularly the strong link to ear, nose, and throat manifestations, 23 the median DCI score for this study's population was 1.0 out of 6.…”

“…Given the toxicity profile associated with long-term OCS, 13,14,25,26 immunosuppressants are frequently used as OCS-sparing agents in EGPA. In the current study, the most commonly used immunosuppressant therapies in the year before mepolizumab initiation were azathioprine, methotrexate, and mycophenolate mofetil, although each of these treatments was used by fewer than 10% to 20% of patients; cyclophosphamide was used by approximately 5% of patients, consistent with previous database analyses 6,10 . Based on the ACR 2021 guideline recommendations, 12 these results could suggest that most patients in this study had nonsevere disease.…”

“…The real-world characteristics and disease burden of patients with EGPA have previously been characterized 5–7,9,10 . Following recent approval of mepolizumab for EGPA (2017 in the United States and 2021 in Europe), 17,18 to the best of our knowledge, this is the first study characterizing the disease burden for patients with EGPA initiating mepolizumab treatment.…”

“…1,2 These features can affect multiple organ systems and ultimately result in organ impairment and injury. 3,4 Despite the rarity of EGPA, with a global prevalence of 2.0 to 38.0 per million people, [5][6][7][8] the range of organ systems involved and the relapsing-remitting nature of the disease exert a large burden on both patients and health care systems. [5][6][7]9,10 In addition, underrecognition of EGPA can further add to the burden.…”

“…3,4 Despite the rarity of EGPA, with a global prevalence of 2.0 to 38.0 per million people, [5][6][7][8] the range of organ systems involved and the relapsing-remitting nature of the disease exert a large burden on both patients and health care systems. [5][6][7]9,10 In addition, underrecognition of EGPA can further add to the burden. 11 Treatment of EGPA typically includes oral corticosteroids (OCSs) in combination with immunosuppressive therapies or biologics 12 ; however, OCS and immunosuppressive therapies are associated with substantial adverse effects.…”