381 - Dupilumab Use in a Pediatric Patient with Severe Allergic Fungal Rhinosinusitis (AFRS)

Sunday, April 30, 2023

3:30 PM – 6:00 PM ET

Poster Number: 381
Publication Number: 381.301

David Van, Louisiana State University School of Medicine in New Orleans, New Orleans, LA, United States; Andrew Abreo, Louisiana State University School of Medicine in New Orleans, New Orleans, LA, United States

Presenting Author(s)

David Van, MD (he/him/his)

Resident
Louisiana State University School of Medicine in New Orleans
New Orleans, Louisiana, United States

Background: Allergic fungal rhinosinusitis (AFRS) is a subtype of chronic rhinosinusitis with nasal polyposis (CRSwNP). The mainstay of treatment involves endoscopic sinus surgery and medical management, including systemic and topical glucocorticoids. Case reports have illustrated the positive effect of dupilumab in adult patients with refractory AFRS; however, there is no available literature in the pediatric population.

Objective:

We describe the case of a pediatric patient with severe AFRS treated with dupilumab.

Design/Methods: Single subject case study.

Results: A 12-year-old female presented with one year of progressive exophthalmos and hypertelorism associated with nasal obstruction. Computed tomography (CT) results revealed extreme sinonasal polyposis, pansinusitis, and bony expansion into the orbits. Otolaryngology performed an endoscopic sinus surgery. Eosinophilic mucin was removed, and fungal cultures grew Curvularia species. She was treated with saline rinses, intranasal glucocorticoids, and oral glucocorticoids post-operatively. Serum immunoglobulin E (IgE) levels showed sensitization to multiple molds. She was unable to start allergen immunotherapy due to hospital distance. Dupilumab was started on a compassionate use basis given the risks of long-term systemic glucocorticoid use in a pediatric patient. CT imaging obtained 7 months after initiation showed significant improvement in paranasal sinus opacification. She has tolerated dupilumab for one year with improved nasal symptoms and no disease recurrence.

Conclusion(s): AFRS is caused by a type 2 inflammatory response against sinus fungal colonization. Dupilumab inhibits type 2 inflammation by blocking interleukin-4 (IL-4) and interleukin-13 (IL-13) signaling. This case highlights the potential role of dupilumab for the treatment of AFRS in pediatric patients; however, randomized controlled trials are needed to further evaluate the safety and efficacy.