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Systemic Autoimmune Disorder with Pulmonary Involvement: Novel Insights from Underlying Mechanisms of Drug Development and Intervention

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

Deadline for manuscript submissions: 30 September 2025 | Viewed by 716

Special Issue Editor

Special Issue Information

Dear Colleagues,

Rheumatic diseases include a wide range of disorders with a notable impact on quality of life, as well as significant morbidity and mortality worldwide. Pulmonary involvement is overall frequent, representing a prognostic factor of negative outcomes in patients with connective tissue disorders. During the last few decades, several studies have addressed the immune-mediated mechanisms of lung involvement in patients with systemic autoimmune disorders (SARDs). However, several pathogenetic mechanisms are still far from being clarified, and there is a general lack of knowledge about the heterogeneity of lung involvement in patients with SARDs.

The clinical breakthrough of novel antifibrotic agents and their interconnection with different immunomodulatory agents have led to significant changes in the therapeutic scenario, boosting the development of pharmacological research for these patients.

In this Special Issue, we aim to include original research and review articles focused on early diagnosis, and innovative treatments for patients with pulmonary involvement of systemic autoimmune disorders.

Dr. Fabio Perrotta
Guest Editor

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Keywords

  • rheumatic diseases
  • pulmonary involvement
  • connective tissue disorders
  • systemic autoimmune disorders (SARDs)
  • diagnosis
  • innovative treatments

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Published Papers (1 paper)

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Review

18 pages, 1275 KiB  
Review
A Simple Ratio in a Complex Disease: Exploring the Neutrophil-to-Lymphocyte Ratio in Idiopathic Pulmonary Fibrosis
by Giorgio Monteleone, Luca Passantino, Jacopo Simonetti, Bruno Iovene, Francesco Varone, Paolo Cameli, Giacomo Sgalla and Luca Richeldi
J. Clin. Med. 2025, 14(14), 5100; https://doi.org/10.3390/jcm14145100 - 18 Jul 2025
Viewed by 527
Abstract
The neutrophil-to-lymphocyte ratio (NLR) is a simple, inexpensive and easily accessible inflammatory biomarker that reflects the balance between innate and adaptive immunity. In recent years, NLR has emerged as a potential prognostic and disease severity marker for different diseases, including idiopathic pulmonary fibrosis [...] Read more.
The neutrophil-to-lymphocyte ratio (NLR) is a simple, inexpensive and easily accessible inflammatory biomarker that reflects the balance between innate and adaptive immunity. In recent years, NLR has emerged as a potential prognostic and disease severity marker for different diseases, including idiopathic pulmonary fibrosis (IPF), a progressive and fatal interstitial lung disease with a highly variable course and poor prognosis. Several studies have highlighted that NLR can be associated with several clinical outcomes such as lung function decline, increased risk of hospitalization, acute exacerbation of IPF, and mortality over time. It might also correlate with overall survival in the course of antifibrotic therapy and validated prognostic score as a gender–age–physiology score. Despite these findings, the clinical use of NLR remains limited due to its non-specific nature, the lack of standardized cut-off values, and high variability related to demographic factors, comorbidities and medications. Hence, NLR may display the underlying immune dysregulation in IPF and could be exploited as a non-invasive tool for risk stratification and disease monitoring. Further studies are needed to confirm and validate its use in IPF and to establish reliable cut-off values in clinical applications. Full article
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