Clinical Advances and New Insights in Systemic Sclerosis

A special issue of Sclerosis (ISSN 2813-3064).

Deadline for manuscript submissions: 20 December 2025 | Viewed by 1759

Special Issue Editor


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Guest Editor
Department of Microbiology & Immunology, College of Medicine, Drexel University, Philadelphia, PA 19129, USA
Interests: systemic sclerosis (scleroderma); fibrosis; inflammasome signaling; collagen export from the endoplasmic reticulum; small molecule therapeutic development

Special Issue Information

Dear Colleagues,

Systemic sclerosis (SSc), or scleroderma, is a rare autoimmune disease marked by widespread fibrosis of the skin and organs, vascular abnormalities, and immune dysregulation. Despite progress in our understanding of its mechanisms, effective treatments remain limited.

This Special Issue, "Clinical Advances and New Insights in Systemic Sclerosis", aims to highlight recent clinical breakthroughs, research developments, and emerging therapies for SSc. We invite submissions addressing early diagnosis, enhanced management, and patient-specific treatments, with the aim of offering a comprehensive overview of current and future SSc care. Submissions may cover a range of topics including, but not limited to, the following:

  • Pathophysiology: The mechanisms driving fibrosis, immune dysfunction, and vascular changes.
  • Early Diagnosis: New tools and biomarkers for the early detection of SSc.
  • Clinical Subtypes: SSc phenotypes and personalized treatment strategies.
  • Treatments: Advances in biologics, immunosuppression, and non-pharmacologic approaches.
  • Long-Term Care: Managing complications and improving patient outcomes.
  • Future Research: Novel therapies, biomarkers, and personalized medicine.

Dr. Carol M. Artlett
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 100 words) can be sent to the Editorial Office for announcement on this website.

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-blind peer-review process. A guide for authors and other relevant information for submission of manuscripts is available on the Instructions for Authors page. Sclerosis is an international peer-reviewed open access quarterly 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 1000 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

  • systemic sclerosis
  • scleroderma
  • fibrosis
  • biomarkers
  • personalized treatment

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Published Papers (2 papers)

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Research

17 pages, 2445 KB  
Article
The Use of 18F-FDG-PET in Systemic Sclerosis with Myocardial Involvement: The Scleroderma Heart Study
by Jamie Sin Ying Ho, Thomas Wagner, Christopher Denton, John Gerry Coughlan, Daniel Knight, Tushar Kotecha and Benjamin Schreiber
Sclerosis 2025, 3(3), 31; https://doi.org/10.3390/sclerosis3030031 - 18 Sep 2025
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Abstract
Objectives: Cardiac involvement in scleroderma due to myocardial inflammation and fibrosis is associated with poor outcomes, but there is lack of consensus on its investigation and treatment. In this prospective pilot study, we aimed to assess the cardiac uptake of 18F-FDG-PET/CT in [...] Read more.
Objectives: Cardiac involvement in scleroderma due to myocardial inflammation and fibrosis is associated with poor outcomes, but there is lack of consensus on its investigation and treatment. In this prospective pilot study, we aimed to assess the cardiac uptake of 18F-FDG-PET/CT in suspected scleroderma cardiomyopathy. Methods: The Scleroderma Heart study involved 16 patients with cardiac scleroderma but no coronary artery disease. 18F-FDG-PET/CTs were performed, and the patients with a positive scan were offered a second 18F-FDG-PET/CT scan after 6–9 months. The clinical characteristics and clinical outcomes (all-cause mortality) were compared between the patients with positive and negative 18F-FDG-PET/CT scans. Results: Of the 16 included patients, 8 (50%) had positive myocardial uptake on the 18F-FDG-PET/CT, 2 of whom showed a pattern consistent with cardiac involvement in scleroderma, while 6 patients more likely had physiological uptake. Over a mean follow-up of 603.3 days, all-cause mortality occurred in six patients (37.5%), and the mortality was similar between the two groups. Five patients with repeat 18F-FDG-PET/CTs showed stable or increased FDG uptake despite immunosuppression. Conclusions: To the best of our knowledge, this is the first study to investigate 18F-FDG-PET/CT in scleroderma patients with suspected cardiac involvement. The cardiac PET showed limited clinical utility due to frequent physiological uptake and lack of correlation with the treatment response. Further studies with larger cohorts and standardised interpretation criteria are needed before cardiac PET can be recommended for routine clinical use in scleroderma cardiomyopathy. Full article
(This article belongs to the Special Issue Clinical Advances and New Insights in Systemic Sclerosis)
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13 pages, 405 KB  
Article
Identifying Patients with Systemic Sclerosis and Progressive Pulmonary Fibrosis in a Real-World Setting: Data from UK Tertiary Rheumatology and ILD Centres
by Rosalind Benson, Mahin Ahmad, Lisa G. Spencer, Freddy Frost, Madhu Paravasthu and Theresa Barnes
Sclerosis 2025, 3(3), 25; https://doi.org/10.3390/sclerosis3030025 - 1 Jul 2025
Viewed by 754
Abstract
Objectives: Systemic sclerosis-related interstitial lung disease (SSc-ILD) has high associated morbidity and mortality. With early diagnosis and treatment, we can improve clinical outcomes with immunosuppression. Some patients develop progressive pulmonary fibrosis (PPF) and are eligible for anti-fibrotic therapy. There are limited data on [...] Read more.
Objectives: Systemic sclerosis-related interstitial lung disease (SSc-ILD) has high associated morbidity and mortality. With early diagnosis and treatment, we can improve clinical outcomes with immunosuppression. Some patients develop progressive pulmonary fibrosis (PPF) and are eligible for anti-fibrotic therapy. There are limited data on the incidence and prevalence of PPF in the SSc ILD cohort to guide case finding. We investigated this using data from UK tertiary Rheumatology and ILD centres. Methods: Patients with systemic sclerosis across two UK rheumatology units were identified using electronic records searched from 2021 to 2023 and were compared against established PPF diagnostic criteria. Results: 255 patients were identified. Prevalence of PPF was 5.49% and in those with established SSc-ILD, 23%. Median time to development of PPF was 5 years. In 64% of patients with PPF diagnosis, they had had systemic sclerosis for over 10 years. Incidence of PPF in patients with SSc was 3.9% and in those with known SSc-ILD 16.%. Only 50% of patients who met criteria for PPF had been referred to respiratory for consideration of antifibrotic initiation. Patients with a predominantly fibrotic baseline radiological pattern (UIP) had a trend towards development of PPF (p = 0.07). No patient with a predominantly inflammatory baseline pattern developed PPF (p = 0.021). Conclusions: Real world data have shown a prevalence of PPF in the SSc-ILD cohort of 23% with a median time of 5 years to development from diagnosis of SSc. Our data show active case finding may be incomplete and rheumatologists must be cognisant of PPF when evaluating patients with SSc. Full article
(This article belongs to the Special Issue Clinical Advances and New Insights in Systemic Sclerosis)
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