Chronic Rhinosinusitis in Cystic Fibrosis: Diagnosis and Medical Management.

Chetan Safi,Emily Dimango,David Gudis,Zhong Zheng,Claire Keating

doi:10.3390/medsci7020032

Abstract

Chronic rhinosinusitis (CRS) is nearly ubiquitous in patients with cystic fibrosis (CF). CF CRS is a challenging entity to define, diagnose, and treat, as patients often have severe refractory sinus disease in addition to complex medical comorbidities. The purpose of this article is to review the literature on the medical management of CF CRS and determine how to best identify, diagnose, and manage CF CRS. Ultimately, the treatment of these patients requires a multi-disciplinary approach involving the pulmonologist and otolaryngologist.

Highlights

Cystic fibrosis (CF) is an autosomal recessive genetic disorder characterized by poor chloride ion (Cl- ) transport across cell membranes due to mutations in the cystic fibrosis transmembrane conductance (CFTR) gene
In considering a unified airway model, some investigators have demonstrated that in CF patients, the paranasal sinuses can serve as a reservoir for virulent bacteria that can lead to pulmonary exacerbations [4,5,6]
Et al demonstrated that CF patients with and without subjective symptoms of sinus disease had similar Lund–Kennedy nasal endoscopy (LKNE) scores which were overall worse than the general population without Chronic rhinosinusitis (CRS)

Summary

Introduction

Cystic fibrosis (CF) is an autosomal recessive genetic disorder characterized by poor chloride ion (Cl- ) transport across cell membranes due to mutations in the cystic fibrosis transmembrane conductance (CFTR) gene. The pulmonary morbidity of CF is due to this thickened mucus that results in poor mucociliary clearance, secondary bacterial colonization, and recurrent infectious exacerbations that decrease overall lung function [2]. This continuous process of mucosal inflammation and infection can affect the paranasal sinuses, leading to chronic rhinosinusitis [3]. Collaboration with a CF pulmonologist is essential to diagnose and treat chronic rhinosinusitis in these complex patients. Our aim is to identify the best strategies to diagnose cystic fibrosis chronic rhinosinusitis (CF CRS) as well as review the medical therapies available to treat these patients. The indications and benefits of surgical therapy are outside the scope of this review

Diagnosis

Quality of Life Metrics

Treatment

Findings

Conclusions