# Evolving Cystic Fibrosis Care: Lung Immunology and Emerging Health Challenges in the Era of CFTR Modulators

**Authors:** Giuseppe Fabio Parisi, Maria Papale, Giulia Pecora, Santiago Presti, Monica Tosto, Enza Mulé, Vittorio Ornato, Donatella Aloisio, Salvatore Leonardi

PMC · DOI: 10.3390/biom15101460 · Biomolecules · 2025-10-16

## TL;DR

CFTR modulators are changing cystic fibrosis care, extending life expectancy but introducing new health challenges that require integrated management.

## Contribution

This review highlights the evolving health landscape in cystic fibrosis due to CFTR modulators and the need for comprehensive care strategies.

## Key findings

- CFTR modulators reduce traditional pulmonary complications by mitigating inflammation and infection.
- Longer life expectancy increases risks of cancers, diabetes, and liver disease in CF patients.
- Integrated care strategies are essential to address both respiratory improvements and emerging health risks.

## Abstract

The introduction of CFTR modulators has dramatically shifted the clinical management of cystic fibrosis (CF) from a life-limiting pediatric condition to a chronic disease with broader health implications. This review explores the impact of these advancements on lung immunology and the emerging spectrum of health challenges. While these modulators have reduced traditional pulmonary complications by mitigating inflammation and infection, they also introduce new considerations for long-term health management. As patients experience longer lives, issues such as the increased risk of certain cancers and other systemic complications like CF-related diabetes and liver disease are gaining attention. Understanding the interplay between CFTR modulators, immune response, and the development of these conditions is essential for optimizing patient outcomes. This review highlights the importance of integrated care strategies that address both the respiratory improvements and emerging health risks associated with longer life expectancy in CF patients. By fostering a comprehensive approach, we aim to enhance the overall quality of life and address the complex needs of individuals navigating CF in the modern therapeutic landscape.

## Linked entities

- **Proteins:** CFTR (CF transmembrane conductance regulator)
- **Diseases:** cystic fibrosis (MONDO:0009061), CF-related diabetes (MONDO:7770003), liver disease (MONDO:0005154)

## Full-text entities

- **Genes:** CFTR (CF transmembrane conductance regulator) [NCBI Gene 1080] {aka ABC35, ABCC7, CF, CFTR/MRP, MRP7, TNR-CFTR}
- **Diseases:** cancers (MESH:D009369), inflammation (MESH:D007249), pulmonary complications (MESH:D008171), diabetes (MESH:D003920), CF (MESH:D003550), infection (MESH:D007239), liver disease (MESH:D008107)
- **Species:** Homo sapiens (human, species) [taxon 9606]

## Full text

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## Figures

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## References

67 references — full list in the complete paper: https://tomesphere.com/paper/PMC12564248/full.md

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Source: https://tomesphere.com/paper/PMC12564248