Dupilumab in chronic rhinosinusitis with nasal polyposis and concomitant cystic fibrosis: a real-life experience.

Abstract

Purpose: Chronic rhinosinusitis (CRS) has been distinguished in primary CRS, a primary inflammatory disorder limited to airways and secondary CRS, in which the sinonasal pathology is caused by a systemic disease or a local pathologic condition. Primary CRS is in turn classified in Type 2 and Non-type 2 on the basis of the endotype and of the pattern of the immune response. Advance in the knowledge of CRS has led to new therapeutic options, among which Dupilumab (anti-IL4R). We report the clinical response to Dupilumab in two patients with cystic fibrosis and nasal polyposis, in which the coexistence of a primary and secondary CRS could not be excluded.

Methods: Nasal endoscopy, smell and quality of life of the patients were evaluated at each follow-up.

Results: In the first case, increased blood eosinophils, allergy to inhalants and NSAIDs intolerance supported the suspect of primary CRS with type 2 inflammatory pattern, in addition to cystic fibrosis and the therapy was effective. In the second case the patient did not show atopy or peculiar blood test and even if the phenotype could suggest a primary CRS combined with a secondary one, the treatment was ineffective and it was suspended.

Conclusion: Even though classifications can be helpful, they can be reductive in cases where different aetiologies overlap. The presence of a concomitant primary CRS must not be excluded a priori in patients affected by secondary CRS. Each patient must be investigated to identify endotype characteristics and select the most appropriate therapeutic option.