xRead - Nasal Obstruction (September 2024) Full Articles

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International consensus statement on rhinosinusitis

Oral and topical steroids Contrary to CRS patients without CF, there is a paucity of evidence for or against the use of topical corticosteroids in CF patients with CRS for CF. One double-blind RCT showed that topical betamethasone reduced the size of NPs, albeit without concomitant improvement in nasal symptoms. 2380 Nonetheless, a 2019 study reported that 88.6% of pediatric otolaryngologists advocate for use of INCS for CRS in CF, 2381 which may be partly due to the low side effect profile. 1083 Comprehensive studies regard ing the use of oral corticosteroids in the treatment of CRS in CF are also lacking. Anti-inflammatory agents While transient resolution of NP was observed with high dose ibuprofen in a 2007 retrospective study, its adoption as a treatment option for NP in CF has been limited due to its side effect profile, findings of polyp recurrence, and the likelihood of requiring eventual endoscopic surgery despite treatment. 2382 DNAse mucolytics (Dornase alfa) Mucolytic agents such as Dornase alfa reduce the vis cosity of sinonasal mucus by cleaving extracellular DNA known to accumulate in CF upper and lower airways due to extensive neutrophil degradation. 2383 A 2018 systematic review showed consistent improvement of sinonasal symp tom scores with topical dornase alfa compared to topical saline alone. 1211 However, the drug’s impact on pulmonary function and endoscopic scores was variable, leading the authors to suggest the need for larger studies. CFTR modulators Ivacaftor, a potentiator that prolongs the open time of the CFTR channel and increases the liquid component of respiratory mucus, has shown significant long-term improvements in pulmonary disease in certain CF patients with gating (G551D) or residual function mutations. 2384 Lumacaftor and tezacaftor, 2 additional CFTR modula tors, are used in combination with ivacaftor to target addi tional mutations of CF. With the US FDA approval of triple combination (TC) CFTR therapy (elexacaftor-tezacaftor ivacaftor) in October 2019, 90% of individuals with CF ≥ 12 years of age have clinical access to highly effective mod ulator therapy based on genotype. 2385

With respect to CRS in CF, a 2019 study of ivacaftor ana lyzed multicenter prospective data originally collected in 2013. It showed improvements out to 6 months in the rhi nologic, psychological, and sleep domains of the SNOT-20 outcomes tool, albeit without a control arm and in young patients with limited CRS severity. 2386 TC CFTR therapy, which targets the most common mutation in CF, F508del, is anticipated to lead to improvements in CF-CRS, beyond substantial pulmonary effects. 2387 Despite the substantial cost (USD$300,000 / year), 2388 CFTR modulators show substantial promise in the treatment of CRS in CF. Surgical Treatment Recommendations It has been reported that approximately 25% to 60% of patients with CF and CRS fail appropriate medical therapy and require surgical intervention. 2389,2390 Stud ies have consistently shown a benefit of ESS on QoL outcomes, 2391,2392 but have mixed results with respect to pulmonary function tests (PFTs), antibiotic use, and pul monary exacerbations. 2393,2394 Additionally, no data exist regarding the outcomes of ESS in the expanding era of highly effective CFTR modulator therapy. In CF patients who undergo ESS following lung transplant, studies have shown no significant improvement in PFTs, but demon strated a significant improvement in total pulmonary related hospitalizations. 2395,2396 With respect to surgical technique, sinus hypoplasia and anatomic variants can make complete ESS difficult, which is especially important in CF as inspissated secre tions may be trapped in partially removed partitions or unopened cells. Therefore, careful pre-operative review of CT anatomy remains crucial. 2397 While extended surgi cal procedures such as endoscopic medial maxillectomy and Draf 3 procedures have shown favorable long-term sinonasal outcomes, 1984,2398 comparative studies are lack ing, and therefore should be considered on a case by case basis based on the degree of disease and mechanism of fail ure in the case of revision ESS. XIV.B Chronic Granulomatous Diseases Chronic granulomatous diseases (CGD) include granulo matosis with polyangiitis (GPA, formerly Wegener’s gran ulomatosis), eosinophilic granulomatosis with polyangi itis (EGPA, formerly Churg-Strauss syndrome), and sar coidosis. CGD produces hallmark perivascular or perilym phatic non-caseating granulomas. GPA and EGPA cause systemic, necrotizing, ANCA-associated vasculitis, while sarcoidosis produces a chronic inflammatory disease of uncertain etiology.