Triple therapy improves chronic rhinosinusitis outcomes in cystic fibrosis

Key takeaways:

• Sinus CT scan scores dropped after 1 year of triple therapy, signaling improvement in chronic rhinosinusitis.
• Fewer patients had Pseudomonas aeruginosa and Staphylococcus aureus after this treatment.

Patients with cystic fibrosis had improved chronic rhinosinusitis outcomes after 1 year of elexacaftor/tezacaftor/ivacaftor therapy, according to results published in JAMA Otolaryngology-Head & Neck Surgery.

Shahid Sheikh

“During this study period (1 year), none of the patients with cystic fibrosis (CF) required sinus surgery, intravenous antibiotic therapy or hospitalization for worsening sinus disease,” Shahid Sheikh, MD, professor of pediatrics at The Ohio State University College of Medicine, told Healio. “This gives confidence to clinicians that with this therapy CF-related sinus disease will improve, which decreases morbidity and probably will also improve quality of life (although this parameter was not monitored in our study).”

In a prospective, single-center, longitudinal cohort study, Sheikh and colleagues assessed 64 patients (median age, 18.5 years; 60.9% girls; 100% white) with CF treated with elexacaftor/tezacaftor/ivacaftor (ETI; Trikafta, Vertex Pharmaceuticals) therapy to find out how this treatment impacts chronic rhinosinusitis outcomes.

Researchers found changes in chronic rhinosinusitis by evaluating sinus CT scans taken at baseline (during the first month of ETI) against scans taken during the first month after 1 year of the therapy. Physicians scored each scan using the Lund-Mackay scoring system, as well as the CF-specific Sheikh-Lind scoring system created by the researchers of this study.

Both scoring systems showed that 1 year of ETI therapy was linked to a lower median total score for chronic rhinosinusitis vs. baseline (Lund-Mackay, 3.3 vs. 5.8; Sheikh-Lind, 2.2 vs. 3.8).

Notably, fewer patients had a mean Lund-Mackay score above five, which indicates severe disease and poorer surgical outcomes, after 1 year of ETI treatment compared with baseline (75% vs. 98.4%).

Between baseline and 1 month of ETI therapy, researchers observed a reduction in sweat chloride (mean difference, 48 mEq/L; 95% CI, 41-54), which they measured as a secondary outcome.

Researchers also looked at how percent-predicted FEV₁, BMI and microbiologic data changed after ETI therapy. From baseline to 1 year, median percent-predicted FEV₁ increased from 67% to 86%, and median BMI increased from 21 kg/m² to 23 kg/m².

In terms of microbiologic data, researchers found that after patients received ETI therapy for 1 year, there was a decrease from baseline in oropharyngeal cultures positive for Pseudomonas aeruginosa (53.1% vs. 22.3%; OR = 0.03; 95% CI, 0.01-0.15) and methicillin-resistant Staphylococcus aureus (29.7% vs. 17.7%; OR = 0.06; 95% CI, 0.01-0.54).

Further, fewer pulmonary exacerbations occurred during the year of ETI therapy vs. the year before ETI therapy (median, 3.5 vs. 0).

“Now we have two sinus CT scoring systems of which one is CF-specific; thus, future studies may also be benefited with a CF-specific sinus CT scoring system (Sheikh-Lind) for chronic rhinosinusitis,” Sheikh told Healio.

“More studies for longer duration in people with CF especially in younger age groups on CFTR modulator therapies will be needed to confirm our findings,” Sheikh added.

For more information:

Shahid Sheikh, MD, can be reached at shahid.sheikh@nationwidechildrens.org.