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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: closed (20 March 2026) | Viewed by 4676

Editor

Dr. Fabio Perrotta

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Guest Editor
Department of Traslational Medical Sciences, Univerisity of Campania L. Vanvitelli, 80145 Naples, Italy
Interests: COVID-19; interstitial lung diseases; metabolic dysfuntion in chronic respiratory disorders
Special Issues, Collections and Topics in MDPI journals

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

Manuscript Submission Information

Manuscripts should be submitted online at www.mdpi.com by registering and logging in to this website. Once you are registered, click here to go to the submission form. Manuscripts can be submitted until the deadline. All submissions that pass pre-check are peer-reviewed. Accepted papers will be published continuously in the journal (as soon as accepted) and will be listed together on the special issue website. Research articles, review articles as well as short communications are invited. For planned papers, a title and short abstract (about 250 words) can be sent to the Editorial Office for assessment.

Submitted manuscripts should not have been published previously, nor be under consideration for publication elsewhere (except conference proceedings papers). All manuscripts are thoroughly refereed through a single-anonymized peer-review process. A guide for authors and other relevant information for submission of manuscripts is available on the Instructions for Authors page. Journal of Clinical Medicine is an international peer-reviewed open access semimonthly journal published by MDPI.

Please visit the Instructions for Authors page before submitting a manuscript. The Article Processing Charge (APC) for publication in this open access journal is 2600 CHF (Swiss Francs). Submitted papers should be well formatted and use good English. Authors may use MDPI's English editing service prior to publication or during author revisions.

Keywords

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

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Published Papers (2 papers)

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Review

11 pages, 581 KB  
Review
Nerandomilast in Autoimmune-Associated Interstitial Lung Diseases: Translating Evidence from Progressive Pulmonary Fibrosis Studies
by Fabio Perrotta, Domenica Francesca Mariniello, Giulia M. Stella, Raffaella Pagliaro, Filippo Scialò, Vasiliki Liakouli, Giulio Forte, Francesco Ciccia, Andrea Bianco and Vito D’Agnano
J. Clin. Med. 2026, 15(6), 2166; https://doi.org/10.3390/jcm15062166 - 12 Mar 2026
Cited by 2 | Viewed by 1466
Abstract
Systemic autoimmune rheumatic disease-associated interstitial lung disease (SARD-ILD) comprises a heterogeneous group of fibrosing lung disorders frequently complicated by progressive pulmonary fibrosis, a phenotype associated with accelerated lung function decline and increased mortality. Although antifibrotic therapies have improved clinical outcomes, significant unmet needs [...] Read more.
Systemic autoimmune rheumatic disease-associated interstitial lung disease (SARD-ILD) comprises a heterogeneous group of fibrosing lung disorders frequently complicated by progressive pulmonary fibrosis, a phenotype associated with accelerated lung function decline and increased mortality. Although antifibrotic therapies have improved clinical outcomes, significant unmet needs remain, particularly regarding treatment tolerability and integration with background immunosuppressive strategies. Preferential phosphodiesterase-4B (PDE4B) inhibition has emerged as a novel therapeutic approach targeting both inflammatory and fibrotic pathways through modulation of intracellular cyclic adenosine monophosphate signaling. This narrative review summarizes the biological rationale and emerging clinical evidence supporting nerandomilast, an oral preferential PDE4B inhibitor, in autoimmune-associated interstitial lung diseases. Preclinical data indicate that PDE4B inhibition may attenuate fibroblast activation, inflammatory signaling, and extracellular matrix deposition. Clinical trials conducted in progressive pulmonary fibrosis populations have demonstrated a reduction in lung function decline, with subgroup analyses suggesting potential benefit in autoimmune-related diseases, although evidence remains limited. The safety profile appears mainly characterized by gastrointestinal adverse events, with ongoing evaluation of neuropsychiatric safety and drug interactions in complex autoimmune populations. Overall, nerandomilast represents a promising investigational strategy bridging antifibrotic and immunomodulatory mechanisms, warranting further dedicated studies in SARD-ILD. Full article
(This article belongs to the Special Issue Systemic Autoimmune Disorder with Pulmonary Involvement: Novel Insights from Underlying Mechanisms of Drug Development and Intervention)
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18 pages, 1275 KB  
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
Cited by 5 | Viewed by 2472
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
(This article belongs to the Special Issue Systemic Autoimmune Disorder with Pulmonary Involvement: Novel Insights from Underlying Mechanisms of Drug Development and Intervention)
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