D1.453 - Pregnancy in Eosinophilic Granulomatosis with Polyangiitis (EGPA): a case report and literature review

Background

Eosinophilic Granulomatosis with Polyangiitis (EGPA) is a rare ANCA-associated vasculitis characterized by asthma, chronic rhinosinusitis with nasal polyps and (hyper)eosinophilia. Evidence on pregnancy management is limited and derived from case reports and small case series. With the increasing use of anti–IL-5/IL-5R biologics, understanding their safety and disease course during pregnancy is a growing clinical need. We report, to our knowledge, the first case of a patient with EGPA treated with mepolizumab throughout pregnancy. Additionally, we performed a literature review on EGPA and pregnancy.

Written informed consent for publication was obtained from the patient.

Case Report

A 34-year-old woman with ANCA-negative EGPA diagnosed in 2011 presented with adult-onset asthma, persistent eosinophilia, pulmonary infiltrates with bronchoalveolar lavage eosinophilia, chronic rhinosinusitis, gastrointestinal involvement and bronchiectasis. For ten years, she was treated with low-dose oral corticosteroids and inhaled therapies, experiencing recurrent exacerbations.

In June 2021, subcutaneous mepolizumab 300 mg every 4 weeks was started, leading to marked clinical improvement, reduced relapses and complete steroid discontinuation by May 2023. In September 2024, while in remission, she discontinued contraception to pursue pregnancy. Pregnancy was confirmed in October 2024. Given limited safety data on biologics, therapy was adjusted to mepolizumab 100 mg every 4 weeks plus 2 mg daily of methylprednisolone.

Pregnancy was complicated by worsening nasal obstruction due to rhinosinusitis with initial polypoid formation, managed with temporary glucocorticoid escalation (4 mg daily) and intranasal budesonide. No asthma exacerbations or systemic vasculitic manifestations occurred. At 39 weeks, she delivered a healthy 3800-g infant vaginally without complications. Postpartum, mepolizumab 300 mg was resumed with steroid tapering.

Methods and Results

A PubMed search using “pregnancy AND eosinophilic granulomatosis with polyangiitis” OR “Churg–Strauss syndrome” identified 29 publications, comprising 54 patients and 78 pregnancies (56 with individual data). EGPA was diagnosed before pregnancy in 68.5%, during pregnancy in 14.8% and postpartum in 16.7%. Flares occurred in 36.7% of pregnancies and 14.3% postpartum. Cardiac involvement (16.1%) accounted for maternal deaths (3.7%). Corticosteroids were used in 85.4% of cases. Compared with the general population, higher rates of preterm birth and very low birth weight were reported, particularly with active disease.

Conclusions

Pregnancy in EGPA is feasible in sustained remission. Disease activity is the main predictor of complications. Steroids remain central to management, while anti–IL-5/IL-5R agents appear promising without major safety concerns. Preconception counselling, ≥6 months of remission and close multidisciplinary monitoring are recommended.