The management of cystic fibrosis chronic rhinosinusitis: An evidenced‐based review with recommendations

Abstract

Background: Cystic fibrosis (CF) chronic rhinosinusitis (CRS) has emerged as a distinct diagnostic entity, unique from other endotypes of CRS in its presentation, pathophysiology, diagnosis, treatment, and outcomes. As the sinonasal health of this patient population may have broad effects on pulmonary health and quality of life, a comprehensive understanding of the diagnostic and therapeutic approach to CF CRS is essential. In recognizing recent scientific advances and unique treatment modalities specific to this challenging patient population, in this review we systematically evaluate the scientific literature and provide an evidenced-based review with recommendations (EBRR) for fundamental management principles of CF CRS. Methods:A systematic review of the literature was performed. Studies evaluating interventions for the management of CF CRS were included. An iterative review process was implemented in accordance with EBRR guidelines. A treatment recommendation was generated based on an assessment of the benefits, harms, and the overall grade of evidence. Results:We evaluated the published literature on 5 unique topics. Each of the following therapeutic categories was investigated explicitly for treatment outcomes in patients with CF CRS: (1) nasal saline; (2) intranasal corticosteroids (INCS); (3) topical antibiotics; (4) cystic fibrosis transmembrane conductance regulator (CFTR) modulator therapy; and (5) endoscopic sinus surgery (ESS).

Effects of CFTR-modulator triple therapy on sinunasal symptoms in children and adults with cystic fibrosis

“…Therapeutic options for CRS in CF are limited and a recent Cochrane review found a lack of evidence regarding therapy [ 7 ]. There is some evidence for beneficial effects of nasal saline, endoscopic sinus surgery, and intranasal corticosteroids as well as topical antibiotics [ 1 , 8 ]. CFTR-modulator therapy has been shown to improve lung function, exacerbation rate, body mass index, quality of life and other aspects of life of patients with cystic fibrosis [ 9 , 10 ].…”

Triple combination CFTR modulator therapy reduces the need for endoscopic sinus surgery in adult patients with cystic fibrosis

“…A recently published Cystic Fibrosis Foundation otolaryngology care multidisciplinary consensus statement recommends ESS for children and adults with CF who have symptomatic chronic rhinosinusitis refractory to appropriate medical therapy, 7 and a recent evidencedbased review stated that nasal saline, ESS, and CFTR modulators are recommended in the management of CF CRS when appropriate. 8 T A B L E 1 Patient characteristics and main result.…”

Antimicrobial Strategies for Cystic Fibrosis

“…The early stages of infection and colonization with P. aeruginosa are often of nonmucoid phenotype and fully susceptible, making antimicrobial therapy much more accessible. 28 29 Sanitizing the sinuses is an important part of eradication, and endoscopic sinus surgery with follow-up nasal saline lavages daily is recommended with intranasal corticosteroids and topical antibiotics as optional add-ons 30 . European antibiotic guidelines recommend either of two regimens: 28 days of inhalations with tobramycin inhalation solution (TIS) or 3 months treatment with inhaled colistin, supplemented by oral ciprofloxacin.…”