Allergy, Immunology and Rheumatology
Allergy, Immunology, and Rheumatology
David Van, MD (he/him/his)
Louisiana State University School of Medicine in New Orleans
New Orleans, Louisiana, United States
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.