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Diagnostics
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Diagnosis and Management of Systemic Sclerosis in 2023
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Diagnosis and Management of Systemic Sclerosis in 2023

A special issue of Diagnostics (ISSN 2075-4418). This special issue belongs to the section "Pathology and Molecular Diagnostics".

Deadline for manuscript submissions: closed (1 May 2023) | Viewed by 8560

Special Issue Editor

Dr. Mohammed S. Osman

E-Mail Website
Guest Editor
Division of Rheumatology, Department of Medicine, University of Alberta Hospital, University of Alberta, Edmonton, AB T6G 2R3, Canada
Interests: systemic sclerosis; biomarkers; genomic instability; microvascular disease

Special Issue Information

Dear Colleagues, 

Systemic sclerosis is a devastating life-threatening autoimmune disease characterized by progressive vasculopathy, fibrosis, and immune dysregulation. Recently, experts in the field have utilized point-of-care tools as ways to diagnose patients with early systemic sclerosis, and to potentially monitor responses to treatment. With this, many experts have paid close attention to patients with very early diagnosis of systemic sclerosis (VeDoSS). There have also been various groups that have utilized combination therapies to treat VeDoSS patients.  In this Special Issue, we would like to invite submissions that discuss various point-of-care tools that are utilized for risk stratifying VeDoSS patients, with an emphasis on how these tools are used to measure certain aspects of the pathogenesis. Similarly, we would also like to invite submissions that discuss the utility of combination therapies in SSc and how treatment using point of care tools is monitored, with an emphasis on how these approaches are utilized to improve and detect the various aspects driving the pathogenesis of SSc.

Dr. Mohammed S. Osman
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. Diagnostics is an international peer-reviewed open access semimonthly 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

  • diagnosis
  • early systemic sclerosis
  • combination therapies
  • biomarkers

Published Papers (4 papers)

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Research

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12 pages, 508 KiB  
Article
Self-Reported Systemic Sclerosis-Related Symptoms Are More Prevalent in Subjects with Raynaud’s Phenomenon in the Lifelines Population: Focus on Pulmonary Complications
by Saskia Corine van de Zande, Amaal Eman Abdulle, Yehya Al-Adwi, Alja Stel, Karina de Leeuw, Elisabeth Brouwer, Suzanne Arends, Christiaan Tji Gan, Harry van Goor and Douwe Johannes Mulder
Diagnostics 2023, 13(13), 2160; https://doi.org/10.3390/diagnostics13132160 - 25 Jun 2023
Viewed by 904
Abstract
Puffy fingers and Raynaud’s phenomenon (RP) are important clinical predictors of the development of systemic sclerosis (SSc). We aim to assess the prevalence of SSc-related symptoms, explore pulmonary symptoms, and test the usefulness of skin autofluorescence (SAF) as a non-invasive marker for Advanced [...] Read more.
Puffy fingers and Raynaud’s phenomenon (RP) are important clinical predictors of the development of systemic sclerosis (SSc). We aim to assess the prevalence of SSc-related symptoms, explore pulmonary symptoms, and test the usefulness of skin autofluorescence (SAF) as a non-invasive marker for Advanced Glycation Endproducts (AGEs). Subjects from the Lifelines Cohort Study with known connective tissue disease (CTD) were excluded. Patient characteristics, SAF, self-reported pulmonary symptoms, and spirometry were obtained. Subjects (n = 73,948) were categorized into definite RP (5.3%) with and without SSc-related symptoms and non-RP. Prevalence of at least one potential SSc-related symptom (other than RP) was 8.7%; 23.5% in subjects with RP and 7.1% without RP (p < 0.001). Subjects with RP and additional SSc-related symptoms more frequently reported dyspnea at rest, dyspnea after exertion, and self-reported pulmonary fibrosis, and had the lowest mean forced vital capacity compared to the other groups (RP without SSc-related symptoms and no RP, both p < 0.001). In multivariate regression, dyspnea at rest/on exertion remained associated with an increased risk of SSc-related symptoms in subjects with RP (both p < 0.001). SAF was higher in subjects with RP and SSc-related symptoms compared to the other groups (p < 0.001), but this difference was not significant after correction for potential confounders. The prevalence of SSc-related symptoms was approximately three-fold higher in subjects with RP. Pulmonary symptoms are more prevalent in subjects with RP who also reported additional potential SSc-related symptoms. This might suggest that (suspected) early SSc develops more insidiously than acknowledged. According to this study, SAF is no marker for early detection of SSc. Full article
(This article belongs to the Special Issue Diagnosis and Management of Systemic Sclerosis in 2023)
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Review

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13 pages, 1211 KiB  
Review
Methods of Assessing Nailfold Capillaroscopy Compared to Video Capillaroscopy in Patients with Systemic Sclerosis—A Critical Review of the Literature
by Zechen Ma, Douwe Johannes Mulder, Robert Gniadecki, Jan Willem Cohen Tervaert and Mohammed Osman
Diagnostics 2023, 13(13), 2204; https://doi.org/10.3390/diagnostics13132204 - 28 Jun 2023
Cited by 3 | Viewed by 1488
Abstract
Introduction: Nailfolds of patients with systemic sclerosis (SSc) provide an opportunity to directly visualize microvascular remodeling in SSc. Nailfold video capillaroscopy (NVC) remains the gold standard for assessing nailfold capillaroscopy (NFC). However, access to NVC is limited by expense and expertise. This review [...] Read more.
Introduction: Nailfolds of patients with systemic sclerosis (SSc) provide an opportunity to directly visualize microvascular remodeling in SSc. Nailfold video capillaroscopy (NVC) remains the gold standard for assessing nailfold capillaroscopy (NFC). However, access to NVC is limited by expense and expertise. This review aims to synthesize current research on other NFC devices compared to NVC. Methods: The literature search included the primary research of adult patients with SSc as defined by the 2013 ACR/EULAR criteria. Methods of assessing NFC included stereomicroscopy/wide-field microscopy, ophthalmoscopy, dermatoscopy, smartphone devices, and digital USB microscopy. Primary outcomes included both qualitative (normal vs. abnormal nailfolds, overall pattern recognition, presence/absence of giant capillaries, hemorrhages, and abnormal morphology) and quantitative (capillary density and dimension) measures. Results: The search yielded 471 studies, of which 9 were included. Five studies compared NVC to dermatoscopy, two compared it to widefield/stereomicroscopy, one to smartphone attachments, and one to USB microscopy. In dermatoscopy studies, NVC had a higher percentage of images that were interpretable (63–77% vs. 100%), classifiable (70% vs. 84%), or gradable (70% vs. 79.3%) across three studies. Dermatoscopy had a lower sensitivity (60.2% vs. 81.6%) and higher specificity (92.5% vs. 84.6%) compared to NVC. One stereomicroscopy study found a significant difference between methods in capillary density in limited cutaneous SSc, while another found correlations in all parameters between stereomicroscopy and NVC. One smartphone lens had good agreement with NVC on abnormal capillary morphology and density. USB microscopy was able to differentiate between SSc and healthy controls using mean capillary width but not by capillary density. Discussion: A dermatoscope may serve as a more portable and affordable screening tool to identify a normal “scleroderma pattern”, and images that need further corroboration by NVC. NFC parameters reported are heterogenous and the standardization of these parameters is important, especially in non-gold-standard devices. Full article
(This article belongs to the Special Issue Diagnosis and Management of Systemic Sclerosis in 2023)
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15 pages, 310 KiB  
Review
Advanced Autoantibody Testing in Systemic Sclerosis
by Kholoud Almaabdi, Zareen Ahmad and Sindhu R. Johnson
Diagnostics 2023, 13(5), 851; https://doi.org/10.3390/diagnostics13050851 - 23 Feb 2023
Cited by 5 | Viewed by 3436
Abstract
Systemic sclerosis is a systemic autoimmune rheumatic disease characterized by immune abnormalities, leading to vasculopathy and fibrosis. Autoantibody testing has become an increasingly important part of diagnosis and prognostication. Clinicians have been limited to antinuclear antibody (ANA), antitopoisomerase I (also known as anti-Scl-70) [...] Read more.
Systemic sclerosis is a systemic autoimmune rheumatic disease characterized by immune abnormalities, leading to vasculopathy and fibrosis. Autoantibody testing has become an increasingly important part of diagnosis and prognostication. Clinicians have been limited to antinuclear antibody (ANA), antitopoisomerase I (also known as anti-Scl-70) antibody, and anticentromere antibody testing. Many clinicians now have improved access to an expanded profile of autoantibody testing. In this narrative review article, we review the epidemiology, clinical associations, and prognostic value of advanced autoantibody testing in people with systemic sclerosis. Full article
(This article belongs to the Special Issue Diagnosis and Management of Systemic Sclerosis in 2023)
11 pages, 469 KiB  
Review
Quality Measures in Systemic Sclerosis
by Aos Aboabat, Zareen Ahmad, Amanda Steiman and Sindhu R. Johnson
Diagnostics 2023, 13(4), 579; https://doi.org/10.3390/diagnostics13040579 - 4 Feb 2023
Viewed by 1685
Abstract
Quality improvement is an emerging field, that applies principles of improvement science and utilizes measurement methods with the aim of improving patient care. Systemic sclerosis (SSc) is a systemic autoimmune rheumatic disease associated with increased healthcare burden, cost, morbidity, and mortality. Gaps in [...] Read more.
Quality improvement is an emerging field, that applies principles of improvement science and utilizes measurement methods with the aim of improving patient care. Systemic sclerosis (SSc) is a systemic autoimmune rheumatic disease associated with increased healthcare burden, cost, morbidity, and mortality. Gaps in delivering care to patients with SSc have been consistently observed. In this article, we introduce the discipline of quality improvement and its use of quality measures. We summarize and comparatively evaluate three sets of quality measures that have been proposed to evaluate the quality of care of patients with SSc. Finally, we highlight the areas of unmet needs and indicate future directions for quality improvement and quality measures in SSc. Full article
(This article belongs to the Special Issue Diagnosis and Management of Systemic Sclerosis in 2023)
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