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Diagnostic Delay in Eosinophilic Granulomatosis With Polyangiitis: Clinical Predictors and Implications for Earlier
Vani Mulkareddy1, Kalena Liu2, Shreya Mandloi3
1Division of Pulmonary, Allergy & Critical Care, Thomas Jefferson University Hospital, Philadelphia, Pennsylvania, USA.
Background:
Eosinophilic Granulomatosis with Polyangiitis (EGPA), previously known as Churg-Strauss syndrome, is a rare antineutrophil cytoplasmic antibody (ANCA) vasculitis, with multiorgan involvement. Due to the variable presentation, diagnosis is often challenging and delayed.
Objective:
We aimed to assess diagnostic delay among patients with EGPA and identify clinical characteristics associated with delayed recognition.
Methods:
A single-institution retrospective chart review was performed in adult patients over 18 diagnosed with EGPA. Data was collected on demographics, clinical features, laboratory data, imaging results, biopsy results, treatment, and outcomes.
Results:
A total of 36 patients were included, with an average time to diagnosis from onset of symptoms of 4.82 years (SD = 5.44). Mean age at diagnosis was 52.5 years and 47.2% were female. Overall, asthma (80.5%) and chronic rhinosinusitis (CRS) (80.5%) were the most prevalent features, and [VERIFY: abstract currently reports 5.5% CRS surgery; Results report 19/29] of those with CRS required surgery prior to diagnosis. Lung nodules or infiltrates were identified in 77.8%, nasal polyps in 52.8%, and 27.8% had received a biologic prior to diagnosis. In this exploratory analysis, diagnostic delay > 3 years was associated with female sex (OR 6.7, 95% CI 1.66-31.9), elevated C-ANCA (yes), which was associated with lower odds of diagnostic delay (OR 0.11, 95% CI 0.005-0.716, p = 0.049), and prior biologic treatment (OR 7.1, 95% CI 1.42-54.4). Renal involvement was associated with a lower likelihood of diagnostic delay (p = 0.037). Given the small cohort and wide confidence intervals, these findings should be interpreted as hypothesis-generating.
Conclusion:
Diagnosing EGPA presents a clinical challenge given its rarity and multiorgan presentation. A high clinical suspicion is required to ensure earlier recognition of this rare disease, including a thorough review of systems and appropriate laboratory workup, particularly in patients with treatment-resistant asthma, CRS, or eosinophilia.