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Clinical Advances in Autoimmune Disorders

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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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18 pages, 770 KB

Open AccessArticle

Evaluation of Nailfold Capillaroscopy as a Novel Tool in the Assessment of Eosinophilic Granulomatosis with Polyangiitis

by Gianluca Screm, Ilaria Gandin, Lucrezia Mondini, Rossella Cifaldi, Paola Confalonieri, Chiara Bozzi, Francesco Salton, Giulia Bandini, Giorgio Monteleone, Michael Hughes, Paolo Cameli, Marileda Novello, Rossana Della Porta, Geri Pietro, Marco Confalonieri and Barbara Ruaro

J. Clin. Med. 2025, 14(15), 5311; https://doi.org/10.3390/jcm14155311 - 28 Jul 2025

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Abstract

Background: Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV), including granulomatosis with polyangiitis (GPA), microscopic polyangiitis (MPA), and eosinophilic granulomatosis with polyangiitis (EGPA), represent a spectrum of systemic disorders characterized by necrotizing inflammation of small- to medium-sized vessels. Nailfold videocapillaroscopy (NVC) is a validated, non-invasive technique routinely employed in the assessment of microvascular involvement in systemic sclerosis and in the differential diagnosis of Raynaud’s phenomenon; its application in the context of AAV, particularly EGPA, has not been investigated yet. The present study aims to assess the presence and the possible pattern of microcirculatory abnormalities detected by NVC in EGPA patients, and to explore potential correlations between capillaroscopic findings and disease activity status. Methods: A total of 29 patients with EGPA (19 women and 10 men), aged between 51 and 73 years, and 29 age- and sex-matched healthy controls were retrospectively enrolled between October 2023 and April 2025, after providing informed consent and meeting the inclusion and exclusion criteria. NVC was conducted in both groups to assess various morphological parameters, and mean capillary density was also calculated. Results: This study observed the presence of capillaroscopic alterations in the EGPA group, including decreased capillary density (38%), neoangiogenesis (72%), rolling (100%), pericapillary stippling (66%), and inverted capillary apex (52%). Overall, when comparing healthy controls with EGPA patients, microcirculatory abnormalities were significantly more prevalent in the latter. Specifically, scores for neoangiogenesis, capillary rolling, pericapillary stippling, and inverted capillary apex showed p-values < 0.001. Conclusions: Our study demonstrates a higher prevalence of four nailfold videocapillaroscopic abnormalities in patients with EGPA compared to healthy controls. However, the identification of these capillaroscopic alterations as specific to EGPA requires further confirmation. Ongoing studies aim to explore the potential role of NVC as a diagnostic marker and to investigate its correlation with the clinical manifestations of EGPA. Full article

(This article belongs to the Special Issue Clinical Advances in Autoimmune Disorders)

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14 pages, 1411 KB

Open AccessArticle

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

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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 Autoimmune Disorders)

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