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RMD Open 2019To evaluate current evidence on the efficacy and safety of topical and systemic medications in patients with primary Sjögren syndrome (SjS) to inform European League... (Meta-Analysis)
Meta-Analysis Review
OBJECTIVE
To evaluate current evidence on the efficacy and safety of topical and systemic medications in patients with primary Sjögren syndrome (SjS) to inform European League Against Rheumatism treatment recommendations.
METHODS
The MEDLINE, EMBASE and Cochrane databases were searched for case-control/prospective cohort studies, randomised controlled trials (RCTs) and systematic reviews.
RESULTS
Current evidence in primary SjS patients fulfilling the 2002 criteria is based on the data from 9 RCTs, 18 prospective cohort studies and 5 case-control studies. Two Cochrane systematic literature reviews (SLRs) have reported that topical treatments for dry mouth and dry eye are safe and effective. Ocular cyclosporine A was safe and effective in two RCTs including 1039 patients with dry eye syndrome. Two Cochrane SLRs on serum tear drops and plugs showed inconsistency in possible benefits, both for symptoms and objective measures. Five RCTs reported significant improvements in oral dryness and salivary flow rates for pilocarpine and cevimeline. An RCT showed no significant placebo-differences for hydroxychloroquine 400 mg/day for the primary outcome (visual analogue scale (VAS) composite of dryness, fatigue and pain). We identified seven RCTs carried out in primary SjS patients. RCTs using infliximab, anakinra and baminercept found no placebo-differences for the primary outcomes. The two largest RCTs randomised 255 patients to receive rituximab or placebo and reported no significant results in the primary outcome (VAS composite), while prospective studies suggested efficacy in systemic disease.
CONCLUSION
The current evidence supporting the use of the main topical therapeutic options of primary SjS is solid, while limited data from RCTs are available to guide systemic therapies.
Topics: Clinical Trials as Topic; Combined Modality Therapy; Disease Management; Humans; Sjogren's Syndrome; Treatment Outcome
PubMed: 31749986
DOI: 10.1136/rmdopen-2019-001064 -
Chest Feb 2021Pulmonary disease is a potentially serious yet underdiagnosed complication of Sjögren's syndrome, the second most common autoimmune rheumatic disease. Approximately...
BACKGROUND
Pulmonary disease is a potentially serious yet underdiagnosed complication of Sjögren's syndrome, the second most common autoimmune rheumatic disease. Approximately 16% of patients with Sjögren's demonstrate pulmonary involvement with higher mortality and lower quality of life.
RESEARCH QUESTION
Clinical practice guidelines for pulmonary manifestations of Sjögren's were developed by the Sjögren's Foundation after identifying a critical need for early diagnosis and improved quality and consistency of care.
STUDY DESIGN AND METHODS
A rigorous and transparent methodology was followed according to American College of Rheumatology guidelines. The Pulmonary Topic Review Group (TRG) developed clinical questions in the PICO (Patient, Intervention, Comparison, Outcome) format and selected literature search parameters. Each article was reviewed by a minimum of two TRG members for eligibility and assessment of quality of evidence and strength of recommendation. Guidelines were then drafted based on available evidence, expert opinion, and clinical importance. Draft recommendations with a clinical rationale and data extraction tables were submitted to a Consensus Expert Panel for consideration and approval, with at least 75% agreement required for individual recommendations to be included in the final version.
RESULTS
The literature search revealed 1,192 articles, of which 150 qualified for consideration in guideline development. Of the original 85 PICO questions posed by the TRG, 52 recommendations were generated. These were then reviewed by the Consensus Expert Panel and 52 recommendations were finalized, with a mean agreement of 97.71% (range, 79%-100%). The recommendations span topics of evaluating Sjögren's patients for pulmonary manifestations and assessing, managing, and treating upper and lower airway disease, interstitial lung disease, and lymphoproliferative disease.
INTERPRETATION
Clinical practice guidelines for pulmonary manifestations in Sjögren's will improve early identification, evaluation, and uniformity of care by primary care physicians, rheumatologists, and pulmonologists. Additionally, opportunities for future research are identified.
Topics: Consensus; Humans; Lung Diseases; Quality of Life; Sjogren's Syndrome
PubMed: 33075377
DOI: 10.1016/j.chest.2020.10.011 -
Autoimmunity Reviews Aug 2021Relevant reviews highlight the association between dysfunctional mitochondria and inflammation, but few studies address the contribution of mitochondria and... (Review)
Review
Relevant reviews highlight the association between dysfunctional mitochondria and inflammation, but few studies address the contribution of mitochondria and mitochondria-endoplasmic reticulum (ER) contact sites (MERCs) to cellular homeostasis and inflammatory signaling. The present review outlines the important role of mitochondria in cellular homeostasis and how dysfunctional mitochondrion can release and misplace mitochondrial components (cardiolipin, mitochondrial DNA (mtDNA), and mitochondrial formylated peptides) through multiple mechanisms. These components can act as damage-associated molecular patterns (DAMPs) and induce an inflammatory response via pattern recognition receptors (PRRs). Accumulation of damaged ROS-generating mitochondria, accompanied by the release of mitochondrial DAMPs, can activate PRRs such as the NLRP3 inflammasome, TLR9, cGAS/STING, and ZBP1. This process would explain the chronic inflammation that is observed in autoimmune diseases such as systemic lupus erythematosus (SLE), rheumatoid arthritis (RA), type I diabetes (T1D), and Sjögren's syndrome. This review also provides a comprehensive overview of the importance of MERCs to mitochondrial function and morphology, cellular homeostasis, and the inflammatory response. MERCs play an important role in calcium homeostasis by mediating the transfer of calcium from the ER to the mitochondria and thereby facilitating the production of ATP. They also contribute to the synthesis and transfer of phospholipids, protein folding in the ER, mitochondrial fission, mitochondrial fusion, initiation of autophagosome formation, regulation of cell death/survival signaling, and regulation of immune responses. Therefore, alterations within MERCs could increase inflammatory signaling, modulate ER stress responses, cell homeostasis, and ultimately, the cell fate. This study shows severe ultrastructural alterations of mitochondria in salivary gland cells from Sjögren's syndrome patients for the first time, which could trigger alterations in cellular bioenergetics. This finding could explain symptoms such as fatigue and malfunction of the salivary glands in Sjögren's syndrome patients, which would contribute to the chronic inflammatory pathology of the disease. However, this is only a first step in solving this complex puzzle, and several other important factors such as changes in mitochondrial morphology, functionality, and their important contacts with other organelles require further in-depth study. Future work should focus on detecting the key milestones that are related to inflammation in patients with autoimmune diseases, such as Sjögren´s syndrome.
Topics: DNA, Mitochondrial; Endoplasmic Reticulum; Humans; Inflammation; Mitochondria; Sjogren's Syndrome
PubMed: 34118452
DOI: 10.1016/j.autrev.2021.102867 -
Clinical and Experimental Rheumatology 2020Primary Sjögren's syndrome (pSS) is a complex and heterogeneous disorder characterised by a wide spectrum of glandular and extra-glandular features. The discovery of... (Review)
Review
Primary Sjögren's syndrome (pSS) is a complex and heterogeneous disorder characterised by a wide spectrum of glandular and extra-glandular features. The discovery of novel biomarkers allowed to characterise the disease not only phenotypically on the basis of clinical presentation, but also on the basis of the endotype. Moreover, a better stratification of patients has important value in the evaluation of mechanisms underlying the risk of lymphoproliferative disorders in these patients. Finally, novel targeted therapies may open new possibilities for the application of personalised medicine in pSS.
Topics: Biomarkers; Comorbidity; Humans; Sjogren's Syndrome
PubMed: 32940212
DOI: No ID Found -
Gastroenterology Clinics of North... Dec 2021The gastrointestinal tract is the second largest organ system in the body and is often affected by connective tissue disorders. Scleroderma is the classic rheumatologic... (Review)
Review
The gastrointestinal tract is the second largest organ system in the body and is often affected by connective tissue disorders. Scleroderma is the classic rheumatologic disease affecting the esophagus; more than 90% of patients with scleroderma have esophageal involvement. This article highlights esophageal manifestations of scleroderma, focusing on pathogenesis, clinical presentation, diagnostic considerations, and treatment options. In addition, this article briefly reviews the esophageal manifestations of other key connective tissue disorders, including mixed connective tissue disease, myositis, Sjogren syndrome, systemic lupus erythematosus, fibromyalgia, and Ehlers-Danlos syndrome.
Topics: Connective Tissue Diseases; Esophagus; Humans; Lupus Erythematosus, Systemic; Mixed Connective Tissue Disease; Sjogren's Syndrome
PubMed: 34717878
DOI: 10.1016/j.gtc.2021.08.005 -
Ugeskrift For Laeger Aug 2021Sjögren's syndrome (SS) is a common autoimmune disease with a prevalence of 1%. SS affects primarily women between the age of 30 and 50 years. The classic... (Review)
Review
Sjögren's syndrome (SS) is a common autoimmune disease with a prevalence of 1%. SS affects primarily women between the age of 30 and 50 years. The classic manifestations are sicca symptoms, musculoskeletal pain and fatigue but the disease can affect all organs. SS is associated with the antibody anti-SSA antibodies. The patients have a 15-20 times higher risk of lymphoma and an increased risk of spontaneous abortion and AV-block in life-born children. In this review, we share the diagnostic process and risk stratification and outline the treatments available from private practice.
Topics: Adult; Child; Fatigue; Female; Humans; Middle Aged; Prevalence; Sjogren's Syndrome
PubMed: 34378522
DOI: No ID Found -
Journal of Stomatology, Oral and... Oct 2022This systematic review aimed to evaluate complications and survival rates of dental implants placed in patients suffering from autoimmune diseases.
PURPOSE
This systematic review aimed to evaluate complications and survival rates of dental implants placed in patients suffering from autoimmune diseases.
MATERIALS AND METHODS
A systematic review was conducted following Preferred Reporting Items for Systematic Reviews and Meta-Analyses systematic review guidelines (PRISMA), using Google scholar and PubMed electronic databases with a stop date of September 2021. The eligibility criteria included all full text human studies in the English language literature reporting on patients with autoimmune diseases treated with dental implants.
RESULTS
Fifty-five studies reporting on nine distinct autoimmune diseases were analyzed: 17 on Sjögren's syndrome (SS), 11 on oral lichen planus (OLP), 8 on Type 1 diabetes, 6 on rheumatoid arthritis (RA), 4 on systemic scleroderma (SSc), 3 on Crohn's disease (CD), 3 on systemic lupus erythematosus (SLE), 2 on mucous membrane pemphigoid (MMB) and 1 on pemphigus vulgaris (PV). Despite the heterogeneity and methodological limitations of most of the studies, results showed that dental implant survival rates were comparable to those reported in the general population. However, patients with secondary SS or erosive OLP were more susceptible to developing peri-mucositis and increased marginal bone loss.
CONCLUSION
This review suggested that dental implants may be considered as a safe and viable therapeutic option in the management of edentulous patients suffering from autoimmune diseases. Nevertheless, scrupulous maintenance of oral hygiene and long-term follow-up emerge as being the common determinants for uneventful dental implant treatment.
Topics: Dental Implants; Humans; Lichen Planus, Oral; Sjogren's Syndrome
PubMed: 35033725
DOI: 10.1016/j.jormas.2022.01.005 -
Arquivos Brasileiros de Oftalmologia 2021To evaluate the concentration of tear lysozyme in individuals with Sjogren´s syndrome, meibomian gland dysfunction, and non-dry-eye disease.
PURPOSE
To evaluate the concentration of tear lysozyme in individuals with Sjogren´s syndrome, meibomian gland dysfunction, and non-dry-eye disease.
METHODS
Ninety subjects were recruited for this study, including 30 with Sjogren´s syndrome, 30 with meibomian gland dysfunction, and 30 with non-dry-eye disease. All subjects were referred to participate in the study based on a "dry eye" investigation. They underwent a complete ocular surface ophthalmic examination encompassing ocular surface disease index, biomicroscopy, tear break-up time, Schirmer test type I, conjunctival vital staining with fluorescein and lissamine green, tear lysozyme concentration, and impression cytology.
RESULTS
Clinical tests yielded the following results: ocular surface disease index Sjogren´s syndrome: 64.5 ± 22.6 meibomian gland dysfunction: 43.5 ± 21.4, non-dry-eye disease: 6.7 ± 4.3 (p=0.02 between groups); Schirmer I test (mm/5 min): Sjogren´s syndrome: 4.95 ± 2.25, meibomian gland dysfunction: 13.28 ± 1.53, non-dry-eye disease 13.70 ± 1.39 (p<0.01 Sjogren´s syndrome vs. non-dry-eye disease and p<0.01 meibomian gland dysfunction vs. non-dry-eye disease); tear break-up time (seconds): Sjogren´s syndrome: 3.97 ± 1.47, meibomian gland dysfunction: 3.95 ± 0.86, non-dry-eye disease: 7.25 ± 1.90 (p<0.01 Sjogren´s syndrome vs. non-dry-eye disease and p<0.01 meibomian gland dysfunction vs. non-dry-eye disease); Lissamine green score: Sjogren´s syndrome-dry-eye: 6.18 ± 2.14, meibomian gland dysfunction-dry-eye: 5.27 ± 1.27, non-dry-eye disease: 1.52 ± 0.97 (p<0.01 Sjogren´s syndrome vs. non-dry-eye disease and p<0.01 meibomian gland dysfunction vs. non-dry-eye disease); impression cytology score: Sjogren´s syndrome: 1.88 ± 0.92, meibomian gland dysfunction: 1.67 ± 0.56, non-dry-eye: 0.45 ± 0.44 (p<0.01 Sjogren´s syndrome vs. non-dry-eye disease and p<0.01 meibomian gland dysfunction vs. non-dry-eye disease) and; tear lysozyme concentration (µg/mL): Sjogren´s syndrome: 751.25 ± 244.73, meibomian gland dysfunction: 1423.67 ± 182.75, non-dry-eye disease: 1409.90 ± 188.21 (p<0.01 Sjogren´s syndrome vs. non-dry-eye disease and p<0.01 Sjogren´s syndrome vs. meibomian gland dysfunction).
CONCLUSION
The concentration of lysozyme in the tears was lower in Sjögren's syndrome patients than in meibomian gland dysfunction and non-dry-eye disease groups. Hence, the lacrimal lysozyme could be considered as a simple, non-invasive, and economical biomarker to differentiate between Sjögren's syndrome dry eye disease and meibomian gland dysfunction dry eye disease.
Topics: Dry Eye Syndromes; Humans; Meibomian Gland Dysfunction; Meibomian Glands; Muramidase; Sjogren's Syndrome; Tears
PubMed: 34431892
DOI: 10.5935/0004-2749.20220017 -
Zeitschrift Fur Rheumatologie Aug 2019The prevalence of primary Sjögren's syndrome (pSS) is between 1:100 and 1:1000 and it is therefore the most common connective tissue disease. Nevertheless, it can be... (Review)
Review
The prevalence of primary Sjögren's syndrome (pSS) is between 1:100 and 1:1000 and it is therefore the most common connective tissue disease. Nevertheless, it can be difficult to diagnose pSS as the symptoms are frequently unspecific and diagnostic markers are lacking in many patients. In addition, only few controlled therapeutic studies of pSS have been carried out so that the optimal management is not yet clear. Meanwhile, outcome parameters to monitor clinical improvement have been developed and a large number of therapeutic studies are currently being performed. This review article summarizes the current diagnostic and treatment options for pSS.
Topics: Humans; Prevalence; Sjogren's Syndrome
PubMed: 30937527
DOI: 10.1007/s00393-019-0625-8 -
Brain and Nerve = Shinkei Kenkyu No... May 2021Sjögren's syndrome is an autoimmune disease characterized by chronic sialadenitis and keratoconjunctivitis sicca. While dryness of the salivary and lacrimal glands is a...
Sjögren's syndrome is an autoimmune disease characterized by chronic sialadenitis and keratoconjunctivitis sicca. While dryness of the salivary and lacrimal glands is a core symptom, neurological complications are known to occur as part of a variety of extraglandular manifestations. The reported frequency of neurological complications ranges from 1.8% to 60%, with a wide variety of central, peripheral, and muscular manifestations. In 25% of cases, neurological symptoms precede the diagnosis of Sjögren's syndrome. Therefore, in the presence of unexplained neurological symptoms, it is important to exclude Sjogren's syndrome.
Topics: Humans; Sjogren's Syndrome
PubMed: 34006688
DOI: 10.11477/mf.1416201798