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Interstitial Lung Disease Secondary to Autoimmune Disorders: Recent Advances and Novel Perspectives

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

Deadline for manuscript submissions: 25 November 2025 | Viewed by 366

Special Issue Editors


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Guest Editor
Rheumatology Unit, Department of Clinical and Experimental Medicine, University of Pisa, Via Roma 67, 56126 Pisa, Italy
Interests: interstitial lung disease; autoimmune systemic diseases; Sjögren’s disease; systemic vasculitis; lung involvement

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Guest Editor
Department of Medicine and Surgery, University of Enna “Kore”, 94100 Enna, Italy
Interests: interstitial pneumonia with autoimmune features (IPAF); interstitial lung diseases associated with systemic autoimmune diseases; rheumatic diseases
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Guest Editor
Rheumatology Unit, Department of Clinical and Experimental Medicine, University of Pisa, Via Roma 67, 56126 Pisa, Italy
Interests: interstitial lung disease; systemic vasculitis; autoimmune systemic diseases; Sjögren’s disease; lung involvement; COVID-19 pneumonia

Special Issue Information

Dear Colleagues,

Interstitial lung disease (ILD) has a high prevalence across a wide range of autoimmune systemic disorders (AIDs). ILD development carries a high burden of mortality for AID patients, often representing the leading organ involvement and influencing therapeutic strategies. On the other hand, there is growing awareness that ILD may be the first (and sometimes the sole) clinical manifestation of an AID, posing a significant diagnostic challenge.

AID-related ILD encompasses a wide spectrum of pulmonary phenotypes, ranging from chronic fibrosing conditions to rapidly progressive, highly inflammatory pictures. Anticipating the trajectory of ILD and discriminating between ongoing fibrosis and inflammation may be key for the choice of the most appropriate treatment regimen.

In this complex scenario, the aim of this Special Issue is to collect new evidence regarding the diagnosis, phenotyping, and outcome prediction of lung involvement secondary to autoimmune etiologies, as well as studies investigating the complex pathogenetic pathways underlying ILD development and new potential therapeutic targets.

Original studies, brief reports on original data, and systematic and narrative reviews are welcome. Submitted studies may include (but are not necessarily limited to) the following topics:

  • Diagnostic and multidisciplinary approach to new-onset ILD patients;
  • Phenotypic stratification of AID-ILD;
  • Novel biomarkers for ILD prediction, diagnosis, and prognostic stratification in AID patients;
  • New imaging techniques for ILD assessment including lung ultrasound and HRCT analysis with AI-based software;
  • Traditional and novel therapeutic approaches to AID-ILD;
  • Cellular and molecular pathogenetic mechanisms underlying ILD development and progression.

We would be glad to receive your valuable contributions.

Dr. Gaetano La Rocca
Prof. Dr. Gianluca Sambataro
Dr. Francesco Ferro
Guest Editors

Manuscript Submission Information

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Keywords

  • interstitial lung disease
  • lung involvement
  • connective tissue diseases
  • rheumatoid arthritis
  • systemic vasculitis
  • radiomics
  • lung ultrasound
  • biomarkers
  • pathogenetic immunological pathways
  • therapeutic targets

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Published Papers (1 paper)

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13 pages, 5919 KiB  
Brief Report
Co-Occurrence of Anti-Synthetase Syndrome and Sjögren Disease: A Case-Based Review
by Andrea Pilato, Giorgio D’Avanzo, Francesca Di Nunzio, Annalisa Marino, Alessia Gallo, Irene Genovali, Letizia Pia Di Corcia, Chiara Taffon, Giuseppe Perrone, Vasiliki Liakouli, Luca Navarini, Roberto Giacomelli, Onorina Berardicurti and Raffaele Antonelli Incalzi
J. Clin. Med. 2025, 14(15), 5395; https://doi.org/10.3390/jcm14155395 (registering DOI) - 31 Jul 2025
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Abstract
Background: Anti-synthetase Syndrome (ASyS) is an idiopathic inflammatory myopathy characterized by muscle weakness and inflammatory infiltrates in muscles. Sjogren’s disease (SD) is an autoimmune condition primarily affecting exocrine glands. Both these conditions may present lung involvement. We describe a female patient with [...] Read more.
Background: Anti-synthetase Syndrome (ASyS) is an idiopathic inflammatory myopathy characterized by muscle weakness and inflammatory infiltrates in muscles. Sjogren’s disease (SD) is an autoimmune condition primarily affecting exocrine glands. Both these conditions may present lung involvement. We describe a female patient with anti-synthetase/SD overlap syndrome and review the literature to identify published cases describing this overlap, aiming to better define its clinical, radiological, and serological features. Methods: The case description was based on a retrospective collection of clinical, laboratory, and imaging data related to the patient’s diagnostic process and clinical course. Data were anonymized and handled in accordance with the competent territorial Ethics Committee. A literature review was performed using the MEDLINE and Scopus databases by combining the keywords “Anti-Synthetase syndrome”, “Sjögren disease”, “Sjögren syndrome”, “Myositis”, and “Interstitial lung disease” (ILD). Published cases were selected if they met the 2016 EULAR/ACR criteria for SD and at least one of the currently proposed classification criteria for ASyS. Results: The described case concerns a 68-year-old woman with rapidly progressive ILD. The diagnosis of anti-synthetase/SD overlap syndrome was based on clinical, serological (anti-Ro52 and anti-PL7 antibodies), histological, and radiological findings. Despite immunosuppressive and antifibrotic treatment, the clinical course worsened, leading to a poor outcome. In addition, six relevant cases were identified in the literature. Clinical presentations, autoantibody profiles, radiological findings, and outcomes were highly heterogeneous. Among the reported cases, no standardized treatment protocols were adopted, reflecting the lack of consensus in managing this rare condition. Conclusions: In anti-synthetase/SD overlap syndrome, ILD may follow a rapidly progressive course. Early recognition can be challenging, especially in the absence of muscular involvement. This case-based review highlights the need for more standardized approaches to the diagnosis and management of this rare and complex overlap syndrome. Full article
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