Diagnosis and Treatment of Sjogren’s Syndrome

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

Deadline for manuscript submissions: closed (20 March 2024) | Viewed by 9098

Special Issue Editor


E-Mail Website
Guest Editor
Department of Rheumatology, Sasebo City General Hospital, Sasebo, Nagasaki, Japan
Interests: Sjögren's syndrome; lupus; autoimmune diseases

Special Issue Information

Dear Colleagues,

Recent advances in diagnostic imaging and molecular methods have changed the management of rheumatic diseases; Sjögren's syndrome (SS) is not an exception. Ultrasound, which is a tool used to detect synovitis of rheumatoid arthritis, is reported to be also useful for the diagnosis of SS. Expressions of noncoding RNAs in patients with SS have been investigated as disease biomarkers. New immunosuppressive drugs such as biological agents and janus kinase inhibitors have been studied as potential therapeutic options. In this Special Issue, we welcome authors to submit papers on the advances in the diagnosis and treatment of SS in terms of both molecular methods and clinical practice.

Dr. Yoshiro Horai
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. 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

  • biological agents
  • JAK inhibitors
  • noncoding RNA
  • Sjögren's syndrome
  • ultrasound

Published Papers (5 papers)

Order results
Result details
Select all
Export citation of selected articles as:

Research

Jump to: Review

10 pages, 518 KiB  
Article
Pulmonary Involvement in Sjögren’s Syndrome: Correlations with Biomarkers of Activity and High-Resolution Computer Tomography Findings
by Deiana Roman, Stela Iurciuc and Alexandru Caraba
J. Clin. Med. 2024, 13(4), 1100; https://doi.org/10.3390/jcm13041100 - 15 Feb 2024
Cited by 1 | Viewed by 744
Abstract
(1) Background: Sjögren’s syndrome (SS) represents a systemic autoimmune disease whose pathophysiology has yet to be elucidated, though it is known that the inflammatory process encountered in SS is of a systemic nature, with cytokines representing the main mediators for tissue damage. (2) [...] Read more.
(1) Background: Sjögren’s syndrome (SS) represents a systemic autoimmune disease whose pathophysiology has yet to be elucidated, though it is known that the inflammatory process encountered in SS is of a systemic nature, with cytokines representing the main mediators for tissue damage. (2) Aim of the study: The aim of the present study is to further the understanding of the link between interleukin serum levels, cytokine serum levels, HRCT findings and the Warrick score (as tools for the evaluation of pulmonary involvement) in patients with pSS. (3) Methods: The present study is a retrospective, observational one aimed at ascertaining the link between SS activity and its clinical implications, as well as how interleukin and TNF-α levels correlate with systemic changes. The study enrolled 112 patients with pSS and 56 healthy subjects, matched for age and gender, as a control group. pSS activity was assessed using the ESSDAI. Cytokine levels and leukocyte and lymphocyte counts were measured in both groups. The focus score was calculated for each patient, HRCT was performed to assess lung function, and the Warrick score was calculated. (4) Conclusions: HRCT revealed NSIP in 13 patients (59.09%) and UIP in 9 patients. The strongest positive correlation was identified upon analyzing the relation between IL-8 and the Warrick score (r = 0.9156, p < 0.00001), followed by a positive correlation between the score and IL-6 levels (r = 0.5738, p < 0.0052). Unsurprisingly, the degree and severity of pulmonary involvement was also positively correlated with the degree of disease activity (r = 0.4345, p = 0.0433). Full article
(This article belongs to the Special Issue Diagnosis and Treatment of Sjogren’s Syndrome)
Show Figures

Figure 1

16 pages, 3073 KiB  
Article
Three Signs to Help Detect Sjögren’s Syndrome: Incidental Findings on Magnetic Resonance Imaging and Computed Tomography
by Yukinori Takagi, Ikuo Katayama, Sato Eida, Miho Sasaki, Toshimasa Shimizu, Shuntaro Sato, Kunio Hashimoto, Hiroki Mori, Mitsunobu Otsuru, Masahiro Umeda, Yoshihiko Kumai, Ryo Toya, Atsushi Kawakami and Misa Sumi
J. Clin. Med. 2023, 12(20), 6487; https://doi.org/10.3390/jcm12206487 - 12 Oct 2023
Cited by 1 | Viewed by 1281
Abstract
This study aimed to retrospectively investigate the prevalence of Sjögren’s syndrome (SS) among patients with ranulas, parotid cysts, or parotid calcifications; identify the characteristic magnetic resonance imaging (MRI) or computed tomography (CT) findings of the lesions associated with SS; and compare the SS [...] Read more.
This study aimed to retrospectively investigate the prevalence of Sjögren’s syndrome (SS) among patients with ranulas, parotid cysts, or parotid calcifications; identify the characteristic magnetic resonance imaging (MRI) or computed tomography (CT) findings of the lesions associated with SS; and compare the SS disease stages among SS patients with the three lesion types. A total of 228 patients with the lesions were classified into SS, possible SS, and non-SS groups. The prevalence of SS among patients with ranulas, parotid cysts, or parotid calcifications was 16%, 24%, and 40%, and the rates of either SS or possible SS were 25%, 41%, and 64%, respectively. SS was associated with (i) ranulas: ≤17 mm; (ii) parotid cysts: bilateral and multiple; and (iii) parotid calcifications: in females, bilateral, multiple, parenchymal, and no coexisting calcifications in other tissues. SS patients with ranulas were significantly younger and had lower submandibular gland stage scores on MRI/CT than those with other lesions. Additionally, in 58% and 15% of SS patients with ranulas and parotid calcifications, respectively, detection of the lesions led to the diagnosis of primary SS. Therefore, recognizing the prevalence of SS among patients with these lesions and the findings associated with SS can help detect undiagnosed SS. Full article
(This article belongs to the Special Issue Diagnosis and Treatment of Sjogren’s Syndrome)
Show Figures

Figure 1

22 pages, 625 KiB  
Article
The Comparison between the Composition of 100% Autologous Serum and 100% Platelet-Rich Plasma Eye Drops and Their Impact on the Treatment Effectiveness of Dry Eye Disease in Primary Sjogren Syndrome
by Dominika Wróbel-Dudzińska, Agata Przekora, Paulina Kazimierczak, Agnieszka Ćwiklińska-Haszcz, Ewa Kosior-Jarecka and Tomasz Żarnowski
J. Clin. Med. 2023, 12(9), 3126; https://doi.org/10.3390/jcm12093126 - 25 Apr 2023
Cited by 5 | Viewed by 2842
Abstract
Purpose: The aim of the study was to compare the difference in composition between 100% autologous serum (AS) and 100% platelet-rich plasma (PRP) eye drops and assess their impact on the clinical outcomes after the treatment of severe dry eye (DE) in primary [...] Read more.
Purpose: The aim of the study was to compare the difference in composition between 100% autologous serum (AS) and 100% platelet-rich plasma (PRP) eye drops and assess their impact on the clinical outcomes after the treatment of severe dry eye (DE) in primary Sjogren Syndrome patients (pSS). Materials and Methods: This is an interventional, non-randomized, comparative, three-month study. 22 patients with severe DE in pSS were treated with 100% AS (22 eyes) and 100% PRP (22 eyes) eye drops 5 times per day in monotherapy mode. The quantifications of growth factors (GFs) such as fibroblast growth factor (FGF), epidermal growth factor (EGF), vascular endothelial growth factor (VEGF), platelet-derived growth factor (PDGF), nerve growth factor (NGF), transforming growth factor (TGF-b), insulin-like growth factor (IGF), fibronectin, and substance p in hemoderivates were done. The main outcome measures were: Ocular Surface Disease Index (OSDI), Best Corrected Visual Acuity (BCVA), the Schirmer test, tear break-up time (TBUT), corneal and conjunctival staining according to the Oxford scale, conjunctival hyperaemia, and Meibomian gland parameters. The results were compared at baseline, 1 month, and 3 months following the treatment. The clinical results were correlated with the concentration of GFs in the biological tear substitutes. Results: Significant differences were observed in the concentration of FGF (4.42 ± 0.86 vs. 15.96 ± 7.63, p < 0.0001), EGF (4.98 ± 0.97 vs. 39.06 ± 20.18, p < 0.0001), fibronectin (929.6 ± 111.5 vs. 823.64 ± 98.49, p = 0.0005), VEGF (175.45 ± 65.93 vs. 717.35 ± 488.15, p < 0.0001), PDGF AB (619.6 ± 117.30 vs. 349.66 ± 79.82, p < 0.0001), NGF (85.22 ± 23.49 vs. 8.29 ± 9.06, p < 0.0001), PDGF (935.38 ± 434.26 vs. 126.66 ± 54.41, p < 0.0001), substance p (112.58 ± 27.28 vs. 127.51 ± 26.56, p = 0.0125) in PRP compared to AS. The level of TGF-β was undoubtedly higher in AS than in PRP (1031.37 ± 330.23 vs. 726.03 ± 298.95, p = 0.0004). No significant differences between AS and PRP were observed in the concentration of IGF. Therapy with blood products relieved the signs and symptoms in pSS DE patients. There was a statistically significant improvement in BCVA, the Schirmer test, TBUT, Meibomian gland parameters, and the reduction of the OSDI scores, Oxford staining, and conjunctiva hyperaemia in each of the groups. However, the clinical changes were more significant in the PRP group. There were numerous correlations between the level of GFs and the mean change in clinical outcomes. No adverse events were reported. Conclusions: Despite the fact that blood derivatives differ in composition, they seem to be effective and safe in the treatment of severe DE in pSS patients. The signs and symptoms of DE were reduced in both groups, but only the mean change in OSDI was statistically significant. A greater reduction in OSDI scores was observed in the PRP group. The obtained results and the composition of haemoderivates may indicate the superiority of PRP in relieving the symptoms of DE in pSS patients compared to AS. Full article
(This article belongs to the Special Issue Diagnosis and Treatment of Sjogren’s Syndrome)
Show Figures

Figure 1

Review

Jump to: Research

13 pages, 589 KiB  
Review
Current Views on Pathophysiology and Potential Therapeutic Targets in Sjögren’s Syndrome: A Review from the Perspective of Viral Infections, Toll-like Receptors, and Long-Noncoding RNAs
by Yoshiro Horai, Toshimasa Shimizu, Masataka Umeda, Shin-Ya Nishihata, Hideki Nakamura and Atsushi Kawakami
J. Clin. Med. 2023, 12(18), 5873; https://doi.org/10.3390/jcm12185873 - 10 Sep 2023
Cited by 1 | Viewed by 1283
Abstract
Sjögren’s syndrome (SS) is a rheumatic disease characterized by sicca and extraglandular symptoms, such as interstitial lung disease and renal tubular acidosis. SS potentially affects the prognosis of patients, especially in cases of complicated extraglandular symptoms; however, only symptomatic therapies against xerophthalmia and [...] Read more.
Sjögren’s syndrome (SS) is a rheumatic disease characterized by sicca and extraglandular symptoms, such as interstitial lung disease and renal tubular acidosis. SS potentially affects the prognosis of patients, especially in cases of complicated extraglandular symptoms; however, only symptomatic therapies against xerophthalmia and xerostomia are currently included in the practice guidelines as recommended therapies for SS. Considering that SS is presumed to be a multifactorial entity caused by genetic and environmental factors, a multidisciplinary approach is necessary to clarify the whole picture of its pathogenesis and to develop disease-specific therapies for SS. This review discusses past achievements and future prospects for pursuing the pathophysiology and therapeutic targets for SS, especially from the perspectives of viral infections, toll-like receptors (TLRs), long-noncoding RNAs (lncRNAs), and related signals. Based on the emerging roles of viral infections, TLRs, long-noncoding RNAs and related signals, antiviral therapy, hydroxychloroquine, and vitamin D may lower the risk of or mitigate SS. Janus-kinase (JAK) inhibitors are also potential novel therapeutic options for several rheumatic diseases involving the JAK-signal transducer and activator of transcription pathways, which are yet to be ascertained in a randomized controlled study targeting SS. Full article
(This article belongs to the Special Issue Diagnosis and Treatment of Sjogren’s Syndrome)
Show Figures

Figure 1

16 pages, 19834 KiB  
Review
Primary-Sjögren’s-Syndrome-Related Interstitial Lung Disease: A Clinical Review Discussing Current Controversies
by Gaetano La Rocca, Francesco Ferro, Gianluca Sambataro, Elena Elefante, Silvia Fonzetti, Giovanni Fulvio, Inmaculada C. Navarro, Marta Mosca and Chiara Baldini
J. Clin. Med. 2023, 12(10), 3428; https://doi.org/10.3390/jcm12103428 - 12 May 2023
Cited by 6 | Viewed by 2314
Abstract
Lung involvement, especially interstitial lung disease, is a potentially severe extra-glandular manifestation of Primary Sjogren’s Syndrome (pSS-ILD). ILD can manifest either as a late complication of pSS or anticipate sicca symptoms, likely reflecting two different patho-physiological entities. Presence of lung involvement in pSS [...] Read more.
Lung involvement, especially interstitial lung disease, is a potentially severe extra-glandular manifestation of Primary Sjogren’s Syndrome (pSS-ILD). ILD can manifest either as a late complication of pSS or anticipate sicca symptoms, likely reflecting two different patho-physiological entities. Presence of lung involvement in pSS subjects can remain subclinical for a long time; therefore, patients should be actively screened, and lung ultrasound is currently being investigated as a potential low cost, radiation-free, easily repeatable screening tool for detection of ILD. In contrast, rheumatologic evaluation, serology testing, and minor salivary gland biopsy are crucial for the recognition of pSS in apparently idiopathic ILD patients. Whether the HRCT pattern influences prognosis and treatment response in pSS-ILD is not clear; a UIP pattern associated with a worse prognosis in some studies, but not in others. Many aspects of pSS-ILD, including its actual prevalence, association with specific clinical–serological characteristics, and prognosis, are still debated by the current literature, likely due to poor phenotypic stratification of patients in clinical studies. In the present review, we critically discuss these and other clinically relevant “hot topics” in pSS-ILD. More specifically, after a focused discussion, we compiled a list of questions regarding pSS-ILD that, in our opinion, are not easily answered by the available literature. We subsequently tried to formulate adequate answers on the basis of an extensive literature search and our clinical experience. At the same, we highlighted different issues that require further investigation. Full article
(This article belongs to the Special Issue Diagnosis and Treatment of Sjogren’s Syndrome)
Show Figures

Figure 1

Back to TopTop