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Idiopathic Pulmonary Fibrosis (IPF): Current Perspectives and Future Directions

A special issue of Journal of Clinical Medicine (ISSN 2077-0383). This special issue belongs to the section "Respiratory Medicine".

Deadline for manuscript submissions: 20 January 2026 | Viewed by 461

Special Issue Editor


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Guest Editor
1. Department of Translational Medical Sciences, University of Campania L. Vanvitelli, 80131 Naples, Italy
2. Unit of Respiratory Medicine “L. Vanvitelli”, A.O. dei Colli, Monaldi Hospital, 80131 Naples, Italy
Interests: interstitial lung diseases; lung cancer; idiopathic pulmonary fibrosis; chronic respiratory disorders

Special Issue Information

Dear Colleagues,

Idiopathic pulmonary fibrosis (IPF) represents a chronic fibrotic pulmonary disease that is characterized by a rapidly progressive decline in lung function and is associated with a poor prognosis, given that the median survival time ranges from 3 to 5 years after diagnosis. The pathogenesis of IPF is undoubtedly complex and only partly understood.

Recent advances have led to the approval of new antifibrotic drugs, pirfenidone and nintedanib, which have led to a remarkable improvement in patient outcomes. However, patients with IPF continue to succumb to severe end-stage respiratory failure.

For this Special Issue, we are seeking original research and review articles focusing on current perspectives and future directions in the field of IPF, from its pathogenesis to future therapeutic approaches. We warmly welcome a broad range of submissions, including original research papers and reviews, exploring the state of the art in this pressing topic.

Dr. Vito D’Agnano
Guest Editor

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Keywords

  • pulmonary fibrosis
  • IPF
  • ILD
  • antifibrotics
  • EMT
  • respiratory physiology
  • metabolism

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Published Papers (1 paper)

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10 pages, 226 KB  
Case Report
Case Series on the Combined Therapy with Elexacaftor/Tezacaftor/Ivacaftor During Pregnancy in Women with Severe Cystic Fibrosis: A Retrospective Report from an Italian Centre
by Paola Iacotucci, Jolanda Somma, Lorenza Ferrillo, Assunta Celardo, Valeria Conti, Costantino Di Carlo, Giuseppe Rengo, Graziamaria Corbi and Vincenzo Carnovale
J. Clin. Med. 2025, 14(18), 6520; https://doi.org/10.3390/jcm14186520 - 16 Sep 2025
Viewed by 206
Abstract
In patients with cystic fibrosis (CF), infertility is a common issue in men, while women often experience subfertility. The introduction of CF transmembrane conductance regulator (CFTR) modulators has improved disease progression and enhanced quality of life, consequently leading to an increase in unplanned [...] Read more.
In patients with cystic fibrosis (CF), infertility is a common issue in men, while women often experience subfertility. The introduction of CF transmembrane conductance regulator (CFTR) modulators has improved disease progression and enhanced quality of life, consequently leading to an increase in unplanned pregnancies. This article describes six cases of pregnancies in five patients diagnosed with severe CF who were treated with the combined therapy of elexacaftor/tezacaftor/ivacaftor (ETI). All women were under regular clinical and instrumental monitoring at the Regional CF Center for Adults at the University of Naples Federico II. The reported pregnancies were spontaneous, and all patients were followed throughout their pregnancies. Two pregnancies were carried to term by the same patient. All five patients with a severe CF phenotype were able to experience pregnancy without stopping their ETI treatment without any complications. In two cases, the patients chose to continue ETI therapy while breastfeeding, and there were no adverse events reported. A cesarean delivery was preferred in all cases to prevent potential respiratory distress. These five patients represent some of the few cases in Italy where pregnancy was achieved without interrupting treatment with ETI. However, the lack of more reliable data necessitates that doctors and patients carefully evaluate the risks and benefits of continuing or discontinuing treatment with CFTR modulators. In conclusion, the increasing number of pregnancies and the desire for children expressed by women with CF highlight the need for more data on the long-term effects of CFTR modulators. Full article
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