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Clinical Diagnosis, Treatment and Management of Systemic Sclerosis
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Clinical Diagnosis, Treatment and Management of 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: closed (15 October 2022) | Viewed by 4050

Special Issue Editor

Prof. Serena GUIDUCCI

E-Mail Website
Guest Editor
AOU Careggi, University of Florence, Division of Rheumatology, Florence, Italy
Interests: systemic sclerosis, autoimmune diseases, rheumatic diseases
Special Issues, Collections and Topics in MDPI journals

Special Issue Information

Dear Colleagues,

Systemic Sclerosis (SSc) is a chronic connective tissue disease characterized by microvascular alteration, the activation of autoimmunity with the production of specific auto-antibodies, and the fibrosis of skin and internal organs. In the last decade, many efforts have been made in the identification of patients in the early phase of the disease, and a breakthrough in the management of SSc was made with the 2013 ACR/EULAR classification criteria, which allowed the identification of SSc patients in the early phase of the disease. Organ involvement may be present from the early phases, often being subclinical and underdiagnosed. Pulmonary involvement and pulmonary arterial hypertension represent the most feared complications and the main causes of SSc-related death. Digital ulcers together with gastrointestinal involvement have severe impacts on the quality of life of SSc patients. SSc represents a heavy socio-economic burden and a challenge for rheumatology due to its high clinical heterogeneity.

Prof. Serena GUIDUCCI
Guest Editor

Manuscript Submission Information

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Keywords

  • Systemic sclerosis
  • Vascular damage
  • Heart involvement
  • Kidney involvement
  • Gastrointestinal involvement
  • Interstitial lung disease
  • SSc-specific autoantibodies
  • Clinical heterogeneity
  • Digital ulcers
  • Classification criteria

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Published Papers (2 papers)

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Research

15 pages, 1127 KiB  
Article
Plasma and Urine Levels of Glycosaminoglycans in Patients with Systemic Sclerosis and Their Relationship to Selected Interleukins and Marker of Early Kidney Injury
by Kornelia Kuźnik-Trocha, Katarzyna Winsz-Szczotka, Katarzyna Komosińska-Vassev, Agnieszka Jura-Półtorak, Adrian Miara, Przemysław Kotyla and Krystyna Olczyk
J. Clin. Med. 2022, 11(21), 6354; https://doi.org/10.3390/jcm11216354 - 27 Oct 2022
Cited by 2 | Viewed by 1395
Abstract
Systemic sclerosis (SSc) is a chronic connective tissue disease characterized by immune system dysfunction, vasculopathy, and progressive fibrosis of the skin and internal organs, resulting from excessive accumulation of extracellular matrix (ECM) elements, including collagen and proteoglycans (PGs). An uncontrolled PG proliferation, caused [...] Read more.
Systemic sclerosis (SSc) is a chronic connective tissue disease characterized by immune system dysfunction, vasculopathy, and progressive fibrosis of the skin and internal organs, resulting from excessive accumulation of extracellular matrix (ECM) elements, including collagen and proteoglycans (PGs). An uncontrolled PG proliferation, caused by disturbances in their metabolism in tissues, is most likely reflected in the quantitative changes of their components, i.e., glycosaminoglycans (GAGs), in body fluids. Therefore, the aim of this study was to quantify the different types of GAGs in the blood and urine of systemic sclerosis patients. Chondroitin/dermatan sulfates (CS/DS) and heparan sulfates/heparin (HS/H) were quantified by hexuronic acid assay and electrophoretic fractionation, while hyaluronic acid (HA) and keratan sulfates were evaluated using ELISA tests. In turn, individual urinary GAGs were determined using the Blyscan™ Sulfated Glycosaminoglycan Assay Kit. Our results showed that the plasma concentrations of CS/DS, HS/H, HA, and KS in systemic sclerosis patients were significantly higher compared with those in healthy subjects. In the case of urine measurements, we have found that in SSc patients, CS/DC concentrations were significantly higher, while HA concentrations were significantly lower compared with the values observed in the urine of healthy subjects. Importantly, the found by us correlations between plasma keratan sulfate levels and both the duration of the disease and the severity of skin lesions, as expressed by the Rodnan scale, seems to suggest this GAG as a potential marker in assessing disease progression and activity. In addition, a level of urinary excretion of all types of GAGs due to their high positive correlation with uACR, may be a valuable complementary test in the diagnosis of early renal dysfunction in the course of SSc. Full article
(This article belongs to the Special Issue Clinical Diagnosis, Treatment and Management of Systemic Sclerosis)
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14 pages, 1783 KiB  
Article
Association between Routine Laboratory Parameters and the Severity and Progression of Systemic Sclerosis
by Liticia Chikhoune, Thierry Brousseau, Sandrine Morell-Dubois, Meryem Maud Farhat, Helene Maillard, Emmanuel Ledoult, Marc Lambert, Cecile Yelnik, Sebastien Sanges, Vincent Sobanski, Eric Hachulla and David Launay
J. Clin. Med. 2022, 11(17), 5087; https://doi.org/10.3390/jcm11175087 - 30 Aug 2022
Cited by 14 | Viewed by 2196
Abstract
(1) Background: Systemic sclerosis (SSc) is a heterogeneous connective tissue disease with a high mortality and morbidity rate. Identification of biomarkers that can predict the evolution of SSc is a key factor in the management of patients. The aim of this study was [...] Read more.
(1) Background: Systemic sclerosis (SSc) is a heterogeneous connective tissue disease with a high mortality and morbidity rate. Identification of biomarkers that can predict the evolution of SSc is a key factor in the management of patients. The aim of this study was to assess the association of routine laboratory parameters, widely used in practice and easily available, with the severity and progression of SSc. (2) Methods: In this retrospective monocentric cohort study, 372 SSc patients were included. We gathered clinical and laboratory data including routine laboratory parameters: C-reactive-protein (CRP), erythrocyte sedimentation rate (ESR), complete blood count, serum sodium and potassium levels, creatinin, urea, ferritin, albumin, uric acid, N-terminal pro-brain natriuretic peptide (NTproBNP), serum protein electrophoresis, and liver enzymes. Associations between these routine laboratory parameters and clinical presentation and outcome were assessed. (3) Results: Median (interquartile range) age was 59.0 (50.0; 68.0) years. White blood cell, monocyte, and neutrophil absolute counts were significantly higher in patients with diffuse cutaneous SSc and with interstitial lung disease (ILD) (p < 0.001). CRP was significantly higher in patients with ILD (p < 0.001). Hemoglobin and ferritin were significantly lower in patients with pulmonary hypertension (PH) including pulmonary arterial hypertension and ILD associated PH (p = 0.016 and 0.046, respectively). Uric acid and NT pro BNP were significantly higher in patients with PH (<0.001). Monocyte count was associated with ILD progression over time. (4) Conclusions: Overall, our study highlights the association of routine laboratory parameters used in current practice with the severity and progression of SSc. Full article
(This article belongs to the Special Issue Clinical Diagnosis, Treatment and Management of Systemic Sclerosis)
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