Clinical Advances in Systemic Sclerosis

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

Deadline for manuscript submissions: 31 July 2025 | Viewed by 458

Special Issue Editors


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Guest Editor
Department of Translational and Precision Medicine, Sapienza University of Rome, 00185 Rome, Italy
Interests: systemic sclerosis; Raynaud’s phenomenon; autoimmunity; fibrosis

E-Mail Website
Guest Editor
Department of Translational and Precision Medicine, Sapienza University of Rome, 00185 Rome, Italy
Interests: systemic sclerosis; Raynaud’s phenomenon; autoimmunity; fibrosis
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Special Issue Information

Dear Colleagues,

Systemic sclerosis (SSc) is an extremely heterogeneous systemic autoimmune disease, characterized by fibrosis of the skin and internal organs, endothelial dysfunction with mechanical and functional damage to the microcirculation, and the aberrant activation of the immune system with the production of autoantibodies. Among autoimmune diseases, it has the highest mortality rate related to the disease itself due to visceral involvement. Complications include digital ulcers, pulmonary arterial hypertension, interstitial lung disease, and scleroderma renal crisis.

This Special Issue of the Journal of Clinical Medicine will highlight the latest research findings and clinical advancements in the field of SSc and its complications. The aim is to provide a comprehensive overview of the current state of knowledge and to explore innovative therapeutic approaches that could improve patient outcomes and ensure optimal short- and long-term management.

Dr. Chiara Pellicano
Dr. Edoardo Rosato
Guest Editors

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Keywords

  • systemic sclerosis
  • Raynaud’s phenomenon
  • digital ulcers
  • scleroderma renal crisis
  • pulmonary arterial hypertension
  • interstitial lung disease
  • fibrosis

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Published Papers (1 paper)

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Research

14 pages, 1411 KiB  
Article
Cardiac Involvement and Heart Failure Staging in Patients with Systemic Sclerosis Without Pulmonary Arterial Hypertension
by Maria Isilda Oliveira, Bruno Bragança, José Rodrigues Gomes and Mário Santos
J. Clin. Med. 2025, 14(7), 2211; https://doi.org/10.3390/jcm14072211 - 24 Mar 2025
Viewed by 340
Abstract
Background/Objectives: Systemic sclerosis (SSc) is an autoimmune connective tissue disease characterized by fibrosis and vascular damage, significantly increasing the risk of heart failure (HF). Methods: This cross-sectional study included 61 SSc patients (92% female, mean age 63 ± 13 years), excluding [...] Read more.
Background/Objectives: Systemic sclerosis (SSc) is an autoimmune connective tissue disease characterized by fibrosis and vascular damage, significantly increasing the risk of heart failure (HF). Methods: This cross-sectional study included 61 SSc patients (92% female, mean age 63 ± 13 years), excluding those with pulmonary arterial hypertension, referred to a tertiary pulmonary hypertension center. HF stages were classified according to updated guidelines. Clinical, echocardiographic, hemodynamic, and functional capacity data were analyzed in relation to HF stages. Results: A total of 48% of patients had pre-symptomatic HF (5% stage A, 43% stage B), while 38% had symptomatic HF (stage C). Advanced HF stages were significantly associated with older age (p = 0.02) and multiorgan involvement (p = 0.045) but not with SSc subtype or autoantibodies. Structural and functional echocardiographic abnormalities were prevalent (77% and 10%, respectively). Markers of elevated ventricular filling pressure such as left atrial volume (p = 0.011) and E/e’ ratio (p = 0.03) correlated with HF severity. Functional impairment was observed with lower 6 min walk test (6MWT) distance (p = 0.017), reduced VO2 peak (p = 0.015), and increased VE/VCO2 slope (p = 0.002). Resting pulmonary artery wedge pressure did not correlate with HF stage (p = 0.93). VE/VCO2 slope and 6MWT were independently associated with HF severity. Conclusions: Preclinical and symptomatic HF are highly prevalent in SSc patients. HF staging was linked to disease severity, age, and cardiovascular risk factors. Functional capacity tests (6MWT and CPET) serve as valuable tools for HF risk stratification. These findings highlight the critical need for comprehensive cardiovascular assessment and targeted management strategies to mitigate HF progression in SSc patients. Full article
(This article belongs to the Special Issue Clinical Advances in Systemic Sclerosis)
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