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Cognitive Function in Primary Sjogren's Syndrome: A Systematic Review.

ABSTRACT: BACKGROUND:Cognitive disorders are reported to be common in patients with primary Sjogren's syndrome (pSS). In some cases, they are the first clinical manifestation, preceding the diagnosis of pSS by two years on average. AIM:A systematic review was conducted to explore cognitive impairment in pSS, with reference to diagnostic methods and their relationship with laboratory data and clinical manifestations. MATERIALS AND METHODS:According to the PRISMA 2009 checklist, we carried out a comprehensive literature search in the three main bibliographic databases: MEDLINE, EMBASE, and PsycINFO (NICE HDAS interface). The following main search terms were used: primary Sjogren syndrome, neurological manifestations, fatigue, cognitive functions, psychiatric manifestations, mild cognitive impairment, dementia, and neurocognitive disorder. The search was made on 14 September, 2018. References from all selected studies were also examined. Inclusion criteria were: all studies and case-reports published in any language from 2002 that assessed the association of pSS (according to classification criteria proposed by the 2002 American/European collaborative group (AECG)) with all types of cognitive impairment (including dementia). Exclusion criteria were: reviews, abstracts, secondary Sjögren's syndrome (SS), and all articles in which other classification criteria were used. RESULTS:The initial search yielded 352 articles, of which 253 were excluded based on the title and abstract review. A total of 54 articles underwent a full-length review, and 32 articles were excluded. Data were extracted from 18 studies and three case-reports involving a total of 6196 participants. In most cases, cognitive dysfunction was a brain fog or a mild cognitive impairment (MCI). Occasionally, an autoimmune dementia was present. The relationship between pSS and degenerative dementias, such as Alzheimer's disease (AD), was a controversial issue, even if some investigators hypothesized that pSS could be a risk factor. Several unmet needs were highlighted. First, some of the included studies had not reported the severity of pSS; hence, few correlations between disease severity and cognitive function were possible. Secondly, the evaluation of the pathogenetic role of comorbid diseases was often absent. The lack of information on the type of dementia represented a third critical point in the majority of the included studies. CONCLUSIONS:This systematic review confirmed that adequate studies on cognitive function in pSS are scarce, mostly performed on small-sized samples, and often conflicting. The routine assessment of cognitive function in patients with pSS seems advisable and it will help to elucidate some of the unmet needs highlighted by this review in future appropriately designed studies.

SUBMITTER: Manzo C

PROVIDER: S-EPMC6523842 | biostudies-literature | 2019 Apr

REPOSITORIES: biostudies-literature

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Cognitive Function in Primary Sjögren's Syndrome: A Systematic Review.

Manzo Ciro C Manzo Ciro C Martinez-Suarez Eva E Kechida Melek M Isetta Marco M Serra-Mestres Jordi J

Brain sciences 20190415 4

<h4>Background</h4>Cognitive disorders are reported to be common in patients with primary Sjogren's syndrome (pSS). In some cases, they are the first clinical manifestation, preceding the diagnosis of pSS by two years on average.<h4>Aim</h4>A systematic review was conducted to explore cognitive impairment in pSS, with reference to diagnostic methods and their relationship with laboratory data and clinical manifestations.<h4>Materials and methods</h4>According to the PRISMA 2009 checklist, we car ...[more]

PMID: 30991679

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Project description:Objective: The study aimed to assess the efficacy and safety of clinical trials of biologics in improving the salivary gland (SG) function in primary Sjögren's syndrome (pSS), which has not been analyzed critically and systematically. Methods: PubMed, Web of Science, ClinicalTrials.gov, the EU Clinical Trials Register, and the Cochrane Library were searched for clinical trials that reported effects of biological treatment on the SG function and safety in pSS patients. Inclusion criteria were defined following participants, interventions, comparisons, outcome, and study design (PICOS) recommendations. The objective index (the change of unstimulated whole saliva (UWS) flow) and the serious adverse event (SAE) were assessed as main outcome measures. A meta-analysis of the efficacy and safety of the treatment was conducted. Quality assessment, sensitivity analysis, and publication bias were assessed. The effect size together with a 95% confidence interval was used to estimate the efficacy and safety of biological treatment and was plotted as a forest plot. Results: The literature search yielded 6,678 studies, nine of which fulfilled the inclusion criteria, with seven randomized controlled trials (RCTs) and two non-RCT clinical studies. Generally, biologics do not significantly increase UWS from the baseline of pSS patients compared to the control group at a matched time point (p = 0.55; standard mean difference, SMD = 0.05; 95% confidence interval, CI: -0.11 and 0.21). However, pSS patients with shorter disease duration (≤3 years; SMD = 0.46; 95% CI: 0.06 and 0.85) were prone to have a better response to biological treatment by showing higher increased UWS than patients with longer disease duration (> 3 years; SMD = -0.03; 95% CI: -0.21 and 0.15) (p = 0.03). For the meta-analysis of the safety of biological treatment, the SAEs in the biologics group were significantly higher than those of the control group (p = 0.0021; log odds ratio, OR = 1.03; 95% CI: 0.37 and 1.69). Conclusion: Biological intervention during the early course of the disease may benefit pSS patients better than that during the late course. Significantly, more SAEs in the biologics group indicate that the safety of biologics needs to be addressed for future biological clinical trials and treatment.

Project description:Primary Sjögren's syndrome is an autoimmune exocrinopathy related to lymphocytic infiltration of the exocrine glandular epithelia (such as salivary, lacrimal, nasal, and sebaceous glands or vaginal mucosa) with systemic manifestations of an immuno-inflammatory nature, and not associated with any other systemic disease. It is characterized by severe dryness (Sicca syndrome), particularly in mouth and eyes, with potential strong impact on quality of life and could increase the risk of depression in Sjögren's patient. To date, the impairment of taste and olfactory functions related to Sjögren syndrome remains poorly assessed; so is the trigeminal functions which remain sparsely studied in patients with Sjögren disease. However, other factors can also modify chemosensory functions (olfactory or gustatory sensations and trigeminal nerves), in particular the reduction of the masticatory coefficient or halitosis, due to oral saliva flow decrease, and poor dental condition, which are often present in Sjögren patients. Of the 12 articles evaluated after a 22-year literature search of this review, chemosensory disorders (including taste, smell, and trigeminal impairments) are described and evaluated in pSS patients, with mainly poorer performance compared to healthy controls. Diagnostic and therapeutic (including rehabilitation) approaches of chemosensory disorders in pSS are discussed in this review. Clinician should be more attentive to taste as well as olfacto-trigeminal disorders in primary Sjögren's disease, if possible at the earlier stage, in order to take the best care of Sjögren's patients. This review also highlights some lack in knowledge on pSS chemosensory disorders that should provide new research perspectives. Key Points •Chemosensory functions (including taste, smell, and trigeminal functions) are altered in patients with primary Sjögren's syndrome (pSS) due to dryness of the mouth and the nose. •The trigeminal nerve which interacts with olfactory and gustatory nerves contributes to olfactory and taste perception but remains little studied to date. •Chemosensory function should be considered in the daily clinical assessment of patients with pSS. •Chemosensory function treatment is not standardized yet, however symptomatic treatment of Sjögren syndrome-associated dryness transiently would improve taste and smell, and olfactory or gustatory rehabilitation in pSS patients would be useful.

Project description:BACKGROUND:Primary Sjögren's Syndrome (pSS) is a systemic autoimmune disease that involves the exocrine glands and internal organs. pSS leads to destruction and loss of secretory function due to intense lymphoplasmacytic infiltration. Therapeutic options include mainly symptomatic and supportive measures, and traditional immunosuppressant drugs have shown no effectiveness in randomized trials. Rituximab (RTX) is a chimeric antibody anti-CD20 that leads to B cell depletion by diverse mechanisms. There is evidence that this drug may be effective for treating pSS. The objective of this systematic review was to evaluate Rituximab effectiveness and safety for treating pSS. METHODS AND FINDINGS:We conducted a systematic review of RCTs published until December 2015, with no language restriction. We registered a protocol on Plataforma Brasil (40654814.6.0000.5505) and developed search strategies for the following scientific databases: MEDLINE, EMBASE, CENTRAL and LILACS. We included adults with established pSS diagnosis and considered the use of Rituximab as intervention and the use of other drugs or placebo as control. Four studies met our eligibility criteria: three with low risk of bias and one with uncertain risk of bias. The total number of participants was 276 (145 RTX, 131 placebo). We assessed the risk of bias of each included study and evaluated the following as primary outcomes: lacrimal gland function, salivary gland function, fatigue improvement and adverse events. We found no significant differences between the groups in the Schirmer test at week 24 meta-analysis (MD 3.59, 95% CI -2.89 to 10.07). Only one study evaluated the lissamine green test and reported a statistically significant difference between the groups at week 24 (MD -2.00, 95% CI -3.52 to -0.48). There was a significant difference between the groups regarding salivary flow rate (MD 0.09, 95% CI 0.02 to 0.16) and improvement in fatigue VAS at weeks 6 (RR 3.98, 95% CI 1.61 to 9.82) and week 16 (RR 3.08, 95% CI 1.21 to 7.80). CONCLUSIONS:According to moderate quality evidence, the treatment with a single RTX course in patients with SSp presents discrete effect for improving lacrimal gland function. Low-quality evidence indicates the potential of this drug for improving salivary flow. According to low quality evidence, no differences were observed in the evaluation after 24 weeks regarding fatigue reduction (30% VAS), serious adverse events occurrence, quality of life improvement and disease activity. With a very low level of evidence, there was no improvement in oral dryness VAS evaluation.

Project description:IntroductionPrimary Sjögren's syndrome (pSS) is a systemic autoimmune disorder that primarily affects the exocrine glands such as the lacrimal and the salivary glands. Dry eye disease (DED) is one of the most prevalent manifestations of pSS and is usually classified into aqueous-deficient dry eye and evaporative dry eye. Sjögren's syndrome dry eye (SSDE) is generally described as aqueous-deficient dry eye. However, as the leading pathophysiological mechanism of evaporative dry eye, meibomian gland dysfunction (MGD) also has influence on SSDE, which has been shown in recent studies. We speculate that SSDE is more than just an aqueous-deficient dry eye. While no related systematic review and meta-analysis has been published, the present study is designed to derive a better understanding of the association between MGD and SSDE.Methods and analysisThe Preferred Reporting items for Systematic Reviews and Meta-Analysis for Protocols 2015 statement was used to prepare this protocol. PubMed, Embase, Web of Science, Cochrane Database, China National Knowledge Infrastructure and Wan Fang Database will be searched from their inception to 31 October 2021, with restrictions to publications in English or Chinese. Two reviewers will independently carry out data extraction and quality assessment. The diagnosis of pSS will meet the standard diagnostic criteria, such as American College of Rheumatology/European League against Rheumatism Classification Criteria (ACR/EULAR) or American-European Consensus Group Classification criteria (AECG), and the definition of MGD and DED will differ between studies. The quality of included studies will be judged using the Newcastle-Ottawa Quality Scale. We will carry out this meta-analysis using RevMan V.5.4.1. The incidence of MGD in patients with SSDE will be indicated as OR with 95% CI.Ethics and disseminationEthical approval is not required as this meta-analysis is performed based on published studies and does not involve human participants. The results will be published in a peer-reviewed journal.Prospero registration numberCRD42021226017.

Dataset Information

Cognitive Function in Primary Sjogren's Syndrome: A Systematic Review.

Publications

Cognitive Function in Primary Sjögren's Syndrome: A Systematic Review.

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