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Purpose <p>With the advent of CFTR modulators for cystic fibrosis (CF) like Trikafta®, robust, radiation-sparing imaging strategies are urgently needed for monitoring therapeutic effects in both adult and pediatric patients. We investigated whether low-dose and ultra-low-dose lung CT could serve as innovative tool for short-term monitoring of treatment response in adults and children, respectively.</p> Methods <p>A total of 30 CF patients (15 adults, 15 paediatric) initiated Trikafta® and underwent baseline CT and 12–18-month follow-up scans. Adults and children were imaged with low- and ultra-low-dose protocols, respectively. Disease severity and extent were quantified using Brody score through both qualitative and quantitative analysis. Pre- and post-treatment CT data were compared (paired t-tests), and results were correlated with spirometry and sweat chloride values.</p> Results <p>Remarkably, low and ultra-low-dose protocols maintained high diagnostic quality while reducing radiation exposure (effective dose 2.4 and 0.56&#xa0;mSv respectively). The Brody score showed significant improvements across all patients, demonstrating substantial decreases in mucous plugging (73%) and bronchial thickening (51%). Each patient exhibited a drop in Brody score after therapy, paralleled by better lung function (<i>r</i><sub>s</sub> = − 0.71, <i>p</i> &lt; 0.0001) and sweat test outcomes (<i>r</i><sub>s</sub> = 0.7, <i>p</i> &lt; 0.0001).</p> Conclusions <p>Low- and ultra-low-dose lung CT protocols represent an important advancement in cystic fibrosis imaging, providing sufficient detail to evaluate disease status while substantially reducing cumulative radiation exposure. By enabling reliable short-term assessment of CFTR modulator efficacy through established disease scoring systems, these protocols fill a critical gap in the routine follow-up of patients who require frequent imaging.</p>

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Short-term monitoring of CFTR modulator therapy in adults and children with cystic fibrosis using low and ultra-low-dose lung CT

  • 期刊论文

  • 出版模式：

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  • 发表日期：

    2026年2月25日

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  • Francesca Maccioni,
  • Giuseppe Cimino,
  • Alessandro Longhi,
  • Ludovica Busato,
  • Alessandra Valenti,
  • Lorenza Bottino,
  • Mariateresa Rutigliano,
  • Roberto Alessandrelli,
  • Nicholas Landini,
  • Carlo Catalano

摘要

Purpose

With the advent of CFTR modulators for cystic fibrosis (CF) like Trikafta®, robust, radiation-sparing imaging strategies are urgently needed for monitoring therapeutic effects in both adult and pediatric patients. We investigated whether low-dose and ultra-low-dose lung CT could serve as innovative tool for short-term monitoring of treatment response in adults and children, respectively.

Methods

A total of 30 CF patients (15 adults, 15 paediatric) initiated Trikafta® and underwent baseline CT and 12–18-month follow-up scans. Adults and children were imaged with low- and ultra-low-dose protocols, respectively. Disease severity and extent were quantified using Brody score through both qualitative and quantitative analysis. Pre- and post-treatment CT data were compared (paired t-tests), and results were correlated with spirometry and sweat chloride values.

Results

Remarkably, low and ultra-low-dose protocols maintained high diagnostic quality while reducing radiation exposure (effective dose 2.4 and 0.56 mSv respectively). The Brody score showed significant improvements across all patients, demonstrating substantial decreases in mucous plugging (73%) and bronchial thickening (51%). Each patient exhibited a drop in Brody score after therapy, paralleled by better lung function (rs = − 0.71, p < 0.0001) and sweat test outcomes (rs = 0.7, p < 0.0001).

Conclusions

Low- and ultra-low-dose lung CT protocols represent an important advancement in cystic fibrosis imaging, providing sufficient detail to evaluate disease status while substantially reducing cumulative radiation exposure. By enabling reliable short-term assessment of CFTR modulator efficacy through established disease scoring systems, these protocols fill a critical gap in the routine follow-up of patients who require frequent imaging.