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Advances in Systemic Sclerosis: From Pathogenetic Pathways toward Novel Therapeutic...
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Advances in Systemic Sclerosis: From Pathogenetic Pathways toward Novel Therapeutic Targets: 2nd Edition

A special issue of Life (ISSN 2075-1729). This special issue belongs to the section "Medical Research".

Deadline for manuscript submissions: closed (30 July 2023) | Viewed by 1967

Special Issue Editors

Dr. Eloisa Romano

E-Mail Website
Guest Editor
Department of Experimental and Clinical Medicine, Division of Rheumatology, University of Florence, Largo Brambilla 3, 50134 Florence, Italy
Interests: angiogenesis; cellular and molecular mechanisms of tissue fibrosis; pathogenesis of autoimmune diseases; chronic inflammatory and connective tissue diseases; systemic sclerosis; scleroderma; endothelial cell biology
Special Issues, Collections and Topics in MDPI journals
Dr. Irene Rosa

E-Mail Website
Guest Editor
Department of Experimental and Clinical Medicine, Section of Anatomy and Histology, University of Florence, Largo Brambilla 3, 50134 Florence, Italy
Interests: anatomy; histology; morphological and functional aspects of stromal cells and endothelial cells; angiogenesis; tissue fibrosis; systemic sclerosis; scleroderma
Special Issues, Collections and Topics in MDPI journals
Prof. Dr. Mirko Manetti

E-Mail Website
Guest Editor
Department of Experimental and Clinical Medicine, Section of Anatomy and Histology, University of Florence, Largo Brambilla 3, 50134 Florence, Italy
Interests: anatomy; histology; morphological and functional aspects of stromal cells; endothelial cell biology; angiogenesis; cellular and molecular mechanisms of tissue fibrosis; pathogenesis of autoimmune, chronic inflammatory and connective tissue diseases; animal models of human disorders; systemic sclerosis; scleroderma
Special Issues, Collections and Topics in MDPI journals

Special Issue Information

Dear Colleagues,

The editors are grateful to the many researchers who contributed to the success of the first volume of this issue (https://www.mdpi.com/journal/life/special_issues/scleroderma). We are very pleased to announce the second volume of our Special Issue “Advances in Systemic Sclerosis: From Pathogenetic Pathways toward Novel Therapeutic Targets".

Systemic sclerosis (SSc, or scleroderma) is a complex multifaceted connective tissue disease identified by early microvascular involvement, perivascular tissue inflammation and autoimmune reactions, evolving into progressive fibrosis of the skin and internal organs. Microangiopathy clinically presents as Raynaud’s phenomenon, abnormal nailfold capillaries, digital ulcers, pulmonary arterial hypertension and scleroderma renal crisis, together with myofibroblast-orchestrated untreatable fibrosis, often leading to organ failure, accounting for the high rate of morbidity and mortality.

In recent years, there have been substantial advances in our understanding of SSc pathophysiology, especially in deciphering the pathogenetic mechanisms underlying endothelial cell damage, uncontrolled fibroblast activation and dysfunctional immune response, as well as in the early diagnosis and clinical management of patients. Expanding our knowledge on the cellular and molecular signatures underlying the development and progression of SSc is crucial to gain insights into their potential clinical relevance as biomarkers and/or therapeutic targets and, hence, to make important strides towards precision medicine for this currently incurable disease.

Outstanding experts interested in this Special Issue are invited to submit original manuscripts and reviews dealing with any of the abovementioned aspects of SSc.

Dr. Eloisa Romano
Dr. Irene Rosa
Prof. Dr. Mirko Manetti
Guest Editors

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. Life is an international peer-reviewed open access monthly 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 2600 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
  • pathogenetic mechanisms
  • fibrosis
  • fibroblasts
  • myofibroblasts
  • vasculopathy
  • angiogenesis
  • endothelial cells
  • immune cells
  • autoantibodies
  • biomarkers
  • molecular pathways
  • therapeutic targets

Published Papers (1 paper)

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Research

12 pages, 1712 KiB  
Article
Treatment of Scleroderma-Related Microstomia Using Hyaluronic Acid: An Interventional Study
by Ofir Shir-az, Ariel Berl, Din Mann, Biader Samih Bilal, Yair Levy and Avshalom Shalom
Life 2023, 13(11), 2176; https://doi.org/10.3390/life13112176 - 7 Nov 2023
Cited by 1 | Viewed by 1619
Abstract
Systemic sclerosis (SSc) or scleroderma is a rare, systemic, autoimmune connective tissue disease. It causes increased collagen synthesis, leading to multi-organ sclerosis, including the skin and joints. Patients’ overall health and quality of life are harmed dramatically. Involvement of the face and, especially, [...] Read more.
Systemic sclerosis (SSc) or scleroderma is a rare, systemic, autoimmune connective tissue disease. It causes increased collagen synthesis, leading to multi-organ sclerosis, including the skin and joints. Patients’ overall health and quality of life are harmed dramatically. Involvement of the face and, especially, the oral opening can limit patients’ ability to speak and eat, oral hygiene, and cosmetic appearance. Profhilo® (NAHYCO®) is an over-the-counter product consisting of pure hyaluronic acid. It is used to improve skin quality by increasing collagen production and adipocyte vitality. This interventional study evaluated the results of perioral injections of hyaluronic acid in terms of improved skin quality, elasticity, and increased oral opening. Patients diagnosed with SSc received an injection of one syringe of Profhilo® (2 mL of hyaluronic acid) at each of two clinic visits at one-month intervals. The oral opening was measured between the upper and lower central incisors before and after treatment. Quality of life was assessed using the modified Rodnan Skin Score and Health Assessment Questionnaire-Disability Index. A total of 14 patients received the first treatment, and 11 received the second treatment. The mean oral opening increased from 31.6 mm (range 17–50 mm) prior to therapy to 35.8 mm (range 21–56) 2 months following the second injection. Statistical analysis showed that there was a significant increase in the oral opening as observed one week (36.2 mm, p = 0.011), one month (36.2 mm, p = 0.007), and three months (31.6 mm, p = 0.023) after the second injection, at the 5-month follow-up. Treatment of SSc patients’ perioral area with Profhilo® can result in significant improvements in oral opening and quality of life. Full article
(This article belongs to the Special Issue Advances in Systemic Sclerosis: From Pathogenetic Pathways toward Novel Therapeutic Targets: 2nd Edition)
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