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Sjogren’s Syndrome: Diagnosis and Treatment during the Precision Medicine Era
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Sjogren’s Syndrome: Diagnosis and Treatment during the Precision Medicine Era

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

Deadline for manuscript submissions: closed (16 August 2022) | Viewed by 5156

Special Issue Editors

Dr. Onorina Berardicurti

E-Mail Website
Guest Editor
Division of Rheumatology, Department of Biotechnological and Applied Clinical Sciences, University of L'Aquila, L'Aquila, Italy
Interests: Sjogren’s syndrome; comorbidities; interferons; autoinflammatory diseases; systemic sclerosis; precision medicine
Dr. Valentina Pucino

E-Mail Website
Guest Editor
Rheumatology Research Group, Institute for Inflammation and Ageing, College of Medical and Dental Sciences, University of Birmingham, Queen Elizabeth Hospital, Birmingham, UK
Interests: Sjogren’s syndrome; inflammatory arthritis; immunometabolism

Special Issue Information

Dear Colleagues,

Sjögren’s syndrome is a chronic autoimmune disorder characterized by lymphoplasmacytic infiltration of the exocrine glands. It affects mainly salivary and lacrimal glands, causing sicca syndrome. Sjögren’s syndrome mainly affects middle-aged women, and its incidence ranges between 3 and 11 per 100,000 individuals per year. Beyond sicca symptoms, more than half of the affected patients develop systemic involvement. In fact, chronic fatigue, Raynaud’s phenomenon, musculoskeletal complaints, renal, liver, and neurological related issues often occur. In severe cases, the excess of mortality is mainly related to the development of B cell lymphoma and visceral involvement, such as interstitial lung disease, renal failure, hypokalemic paralysis, and severe cryoglobulinemic vasculitis. Precision medicine is medical care designed to optimize efficiency or therapeutic benefit for particular groups of patients with shared characteristics, especially using genetic or molecular profiling. Despite the progress in understanding underlying pathogenetic mechanisms, effective therapeutic strategies are limited so far for both local and systemic disease manifestations. Furthermore, given the wide spectrum of clinical manifestations, patient stratification before treatment is difficult and needs to be improved to optimize a tailored treatment approach.

In view of these challenges, we are seeking articles that provide new insights into the underlying mechanisms of Sjögren’s syndrome, including molecular and cellular mechanisms involved in the initiation and perpetuation of autoimmunity, new diagnostic tools/biomarkers, and new therapeutic options.

Dr. Onorina Berardicurti
Dr. Valentina Pucino
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. Journal of Clinical Medicine 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

  • Sjogren’s syndrome
  • pathogenesis
  • diagnosis
  • clinical manifestations
  • therapy
  • patient stratification
  • precision medicine

Published Papers (2 papers)

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Research

15 pages, 1889 KiB  
Article
The Predictive Role of Neutrophil-to-Lymphocyte Ratio (NLR), Platelet-to-Lymphocyte Ratio (PLR), Monocytes-to-Lymphocyte Ratio (MLR) and Gammaglobulins for the Development of Cutaneous Vasculitis Lesions in Primary Sjögren’s Syndrome
by Ancuta Mihai, Ana Caruntu, Daniela Opris-Belinski, Ciprian Jurcut, Alina Dima, Constantin Caruntu and Ruxandra Ionescu
J. Clin. Med. 2022, 11(19), 5525; https://doi.org/10.3390/jcm11195525 - 21 Sep 2022
Cited by 7 | Viewed by 3463
Abstract
Background: In primary Sjögren’s Syndrome (pSS), cutaneous vasculitis lesions (CVL) are extraglandular manifestations with an important clinical and prognostic impact and their early detection might contribute to the improvement of disease control and even patients’ survival. The aim of this study was to [...] Read more.
Background: In primary Sjögren’s Syndrome (pSS), cutaneous vasculitis lesions (CVL) are extraglandular manifestations with an important clinical and prognostic impact and their early detection might contribute to the improvement of disease control and even patients’ survival. The aim of this study was to evaluate the predictive potential of hematological elements in the development of CVL in pSS patients. Methods: In this single center, retrospective study, a total of 245 participants were included (124 pSS patients and 121 healthy controls). Complete blood count, inflammatory and immunological parameters were determined at the initial visit. pSS patients underwent a periodical follow-up program, when disease progression and response to therapy was monitored, including the emergence of CVL. Results: In pSS, leucocytes, lymphocyte, neutrophil, monocyte, erythrocyte and platelet counts are significantly decreased compared to healthy subjects (p < 0.001), whereas cellular ratios: NLR, PLR, MLR, and immunological and inflammatory parameters are significantly increased (p < 0.001). A total of 34 patients with pSS (27.41%) developed CVL during the follow-up period. The occurrence of CVL was positively correlated with neutrophil and platelet counts (p < 0.001), while for lymphocytes the correlation was negative (p < 0.001). Cellular ratios: NLR, PLR and MLR, and gammaglobulins also revealed significant positive correlations with the emergence of CVL in pSS (p < 0.001). The multivariate analysis confirmed the independent predictive character for CVL emergence in pSS for NLR (CI95% 0.053–0.2, p < 0.002), PLR (CI95% 0.001–0.003, p < 0.003), MLR (CI95% 0.086–0.935, p < 0.019), and gammaglobulins (CI95% 0.423–0.688, p < 0.001). Conclusions: Standard hematological parameters, widely used in the assessment of pSS patients, such as NLR, PLR, MLR and gammaglobulins could become valid elements that might be used for the early detection of patients at risk for the development of CVL. Full article
(This article belongs to the Special Issue Sjogren’s Syndrome: Diagnosis and Treatment during the Precision Medicine Era)
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8 pages, 258 KiB  
Article
Interferon Gamma Targeted Therapy: Is It Justified in Primary Sjögren’s Syndrome?
by Agata Sebastian, Marta Madej, Paweł Gajdanowicz, Maciej Sebastian, Anna Łuczak, Magdalena Zemelka-Wiącek, Marek Jutel and Piotr Wiland
J. Clin. Med. 2022, 11(18), 5405; https://doi.org/10.3390/jcm11185405 - 14 Sep 2022
Cited by 2 | Viewed by 1206
Abstract
Background: The pathomechanism of primary Sjögren syndrome (pSS) is multifactorial. Many cytokines take part in this process, including interferon. The study aimed to quantify certain cytokines involved in the pathomechanism of primary Sjögren syndrome (IL2, IL5, IL6, IL10, IL13, TNFα, IFNγ) and determine [...] Read more.
Background: The pathomechanism of primary Sjögren syndrome (pSS) is multifactorial. Many cytokines take part in this process, including interferon. The study aimed to quantify certain cytokines involved in the pathomechanism of primary Sjögren syndrome (IL2, IL5, IL6, IL10, IL13, TNFα, IFNγ) and determine their common clinical correlation. On this basis, we discuss the potential use of anti-cytokine drugs in pSS therapy. Methods: The study group consisted of adult patients with a confirmed diagnosis of pSS. Results: The most frequently detected cytokines were IFNγ (82% of patients), TNFα (70%), IL6 (50%), and IL2 (42.5%). In all patients, except for one patient, IFNγ was found in the presence of other specific cytokines. There was no difference in clinical symptoms, age, and laboratory test results between the group of patients with IL-6 + TNFα + IFNγ positive cytokine, and the group of patients in whom they were not detected. There was no correlation between the presence of IL5, IL13, IL2, IL6, IL10, TNFα and musculoskeletal symptoms, skin lesions, glandular domains, pulmonary neurological, lymphadenopathy, biological and hematological domains in ESSDAI (p > 0.05). Conclusions: IFNγ most likely plays a central role in the pathomechanism of the disease. We have not noticed a clinical correlation between the three most common cytokines (IL6, IFNγ and TNFα), preliminary research results open up the possibility of searching for new treatments for pSS. The lower percentage of patients with detectable levels of TNFα and IL6 may explain the ineffectiveness of drugs targeting cytokines in clinical trials to date. Full article
(This article belongs to the Special Issue Sjogren’s Syndrome: Diagnosis and Treatment during the Precision Medicine Era)
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