RATIONALE: Eosinophilic granulomatosis with polyangiitis (EGPA) is a rare, immune-inflammatory disorder characterized by necrotizing vasculitis and late-onset asthma. We investigated the degree of pulmonary involvement and damage in patients with newly diagnosed EGPA, using a retrospective analysis of US administrative health insurance claims data (Merative™ MarketScan® databases).
METHODS: Patients with newly diagnosed EGPA (2017–2021) with ≥12 months of continuous pre-diagnosis health plan enrollment and ≥1 inpatient or ≥2 outpatient EGPA-related diagnoses (≥90 days apart, ICD-10-CM code M30.1) were included. Follow-up was from first observed EGPA diagnosis (index date; ID) until health plan disenrollment or database end. We assessed the prevalence of pulmonary involvement at any time on or before ID; the number of patients with ≥1 pulmonary-related EGPA symptom ≤6 months before ID (‘existing symptoms’) and at any time after ID (‘new symptoms’), and the number of patients with persistent pulmonary damage (caused by EGPA or another cause; assessed using components of the vasculitic damage index) at ID and 1 year after ID. Data were stratified by patient age (<65 and ≥65 years) and sex.
RESULTS: 236 patients with EGPA were identified (136 [57.6%] women; at ID, 208 [88.1%] <65 years, mean [SD] age, 50.4 [14.5] years; mean [SD] follow-up from ID, 21.7 [14.6] months). Pulmonary involvement was present across women and men aged <65 and ≥65 years (Table). A substantial number of patients had obstructive airway diseases (75.8%), throat or chest pain (46.2%), obstructive sleep apnea (19.5%), and other pulmonary manifestations or comorbidities. Most existing and new pulmonary-related symptoms were similar between groups. Notably, most pulmonary-related symptoms occurred considerably more often ≤6 months before ID than after ID, regardless of age and sex, particularly respiratory failure, lung nodules or cavities and pleural effusion or pleurisy/pleuritis, but new events continued to occur after ID (Table). Persistent pulmonary damage was reported across all patients, with approximately 80% having asthma, chronic breathlessness, or impaired lung function at and 1 year after ID. In the year before and after ID, only 33% and 36% of patients identified, respectively, had ≥1 outpatient or office visit with a pulmonologist; among these, the mean (SD) number of claims for visits was 3.4 (3.4) in the year after ID.
CONCLUSIONS: Both men and women, and younger and older patients, had substantial pulmonary involvement, recent and continuing pulmonary-related symptoms, and persistent pulmonary damage, highlighting the importance of involvement of pulmonologists in the management of EGPA.
