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Survey of Ophthalmology 2020Primary Sjögren syndrome is an autoimmune disease that mainly affects exocrine glands such as the salivary and lacrimal glands. In addition, systemic involvement is... (Review)
Review
Primary Sjögren syndrome is an autoimmune disease that mainly affects exocrine glands such as the salivary and lacrimal glands. In addition, systemic involvement is common. Primary Sjögren syndrome is of particular interest to ophthalmologists as it constitutes an important differential diagnosis in conditions with dry eye disease. In addition, ocular tests for more precisely diagnosing and monitoring primary Sjögren syndrome have become increasingly important, and new therapeutics for local and systemic treatment evolve as a result of increased understanding of immunological mechanisms and molecular pathways in the pathogenesis of primary Sjögren syndrome. We provide an update of interest to ophthalmologists regarding pathogenesis, diagnosis, investigative procedures, and treatment options.
Topics: Animals; Autoimmune Diseases; Autoimmunity; Biopsy; Dry Eye Syndromes; Humans; Lacrimal Apparatus; Sjogren's Syndrome
PubMed: 31634487
DOI: 10.1016/j.survophthal.2019.10.004 -
Clinical and Experimental Rheumatology Dec 2022Primary Sjögren's syndrome (pSS) is a complex disabling systemic autoimmune disorder. The hallmark of pSS is the T-cell-mediated hyperactivation of B-cells, evolving... (Review)
Review
Primary Sjögren's syndrome (pSS) is a complex disabling systemic autoimmune disorder. The hallmark of pSS is the T-cell-mediated hyperactivation of B-cells, evolving from asymptomatic conditions to systemic complications and lymphoma development. On tissue level, the typical feature is the lymphocytic infiltration of the salivary gland by B-, T- and antigen presenting cells, as mirrored by the diagnostic cornerstone role of minor salivary gland (MSG) biopsy. B-cells show multiple possible roles in disease pathogenesis, from autoantibody production, to antigen presentation, and cytokine production. B-cells hyperactivation is supported by genetic risk factors, T-cell dependent and independent mechanisms, and the presence of different pathogenic B-cell subsets must be reminded.Many aspects have been investigated in the last year regarding genetic and epigenetics, B- and T-cell role in pSS pathogenesis, their interaction with salivary gland epithelial cells (SGECs) and in their direct or indirect use as biomarkers and predictors of disease development, activity, and lymphomagenesis.In this review, following the others of this series, we will summarise the most recent literature on pSS pathogenesis and clinical features focusing in particular on new insights into pSS molecular stratification and therapeutic advances in the era of precision medicine.
Topics: Humans; Sjogren's Syndrome; Salivary Glands; Salivary Glands, Minor; B-Lymphocytes; Biomarkers
PubMed: 36541236
DOI: 10.55563/clinexprheumatol/43z8gu -
Ugeskrift For Laeger Jul 2021Xerostomia and salivary gland hypofunction impact oral health and quality of life and are mainly caused by the intake of medications, chronic disorders like Sjögren's... (Review)
Review
Xerostomia and salivary gland hypofunction impact oral health and quality of life and are mainly caused by the intake of medications, chronic disorders like Sjögren's syndrome, and head and neck radiation. Other aetiologies may be local diseases of the salivary glands including infections, cancer, or obstructive diseases of the salivary ducts. Management strategies are primarily alleviating symptoms. Current investigations show promising results in stem cell treatment. In this review, we want to provide comprehension of the aetiologies, evaluation, and management of xerostomia and hyposalivation.
Topics: Humans; Neoplasms; Quality of Life; Salivary Glands; Sjogren's Syndrome; Xerostomia
PubMed: 34219641
DOI: No ID Found -
Journal Der Deutschen Dermatologischen... Jul 2022Sjögren's syndrome (SjS) is an autoimmune disease characterized by the triad of sicca symptoms, fatigue and pain. This diagnosis is usually made in women at the average...
Sjögren's syndrome (SjS) is an autoimmune disease characterized by the triad of sicca symptoms, fatigue and pain. This diagnosis is usually made in women at the average age of 60 years. Diagnosis is made when sicca symptoms persist for more than three months, after the exclusion of possible differential diagnoses, and using the ACR/EULAR 2016 classification criteria for SjS. Many organs can be affected in the course of this disease. Xerosis cutis and pruritus are the most common skin manifestations, followed by leukocytoclastic vasculitis and subacute cutaneous lupus erythematosus. In addition, SjS patients often have myoarthralgia and neuropsychiatric symptoms. In the long term, attention must be paid to the increased risk of cardiovascular disease and lymphoma. Due to the multiorgan involvement in SjS patients, interdisciplinary care is required.
Topics: Diagnosis, Differential; Female; Humans; Lupus Erythematosus, Cutaneous; Lymphoma; Middle Aged; Sjogren's Syndrome
PubMed: 35775593
DOI: 10.1111/ddg.14823 -
Deutsches Arzteblatt International May 2017Sjögren's syndrome is one of the more common inflammatory rheumatological diseases, with a prevalence of at least 0.4% in Germany. (Review)
Review
BACKGROUND
Sjögren's syndrome is one of the more common inflammatory rheumatological diseases, with a prevalence of at least 0.4% in Germany.
METHODS
This review is based on pertinent articles retrieved by a selective search in PubMed. Special attention is drawn to updated classification criteria and current treatment recommendations.
RESULTS
Sjögren's syndrome has a wide variety of presentations, ranging from the local involvement of exocrine glands with keratoconjunctivitis sicca and xerostomia (the leading signs of the disease) to the systemic, extraglandular involvement of multiple organs. Fatigue also markedly worsens the patients' quality of life. Serologic testing reveals antinuclear auto-antibodies (anti-Ro/ SSA and anti-La/SSB) as well as rheumatoid factors. The histological hallmark of the disease is focal lymphocytic infiltration in otherwise normal-appearing glandular acini. The disease also markedly elevates the risk of non-Hodgkin lymphoma of the B-cell series, which arises in about 5% of patients. Primary Sjögren's syndrome (pSS) differs from the secondary form (sSS), which appears in the setting of another autoimmune disease, particularly systemic lupus erythematosus (15-36%), rheumatoid arthritis (20-32%), and limited or progressive systemic sclerosis (11-24%). Disease-modifying therapy is reserved for patients with systemic involvement; there is limited evidence for its efficacy. Because of the complexity of this disease, some of its clinical manifestations may require interdisciplinary treatment.
CONCLUSION
The main considerations in the interdisciplinary care of patients with Sjögren's disease are measures to improve quality of life, pharmacological and non-pharmacological treatments to keep disease activity in check, and management of the risk of lymphoma. Future therapeutic approaches must take the heterogeneity of the disease into account.
Topics: Antibodies, Antinuclear; Germany; Humans; Lupus Erythematosus, Systemic; Quality of Life; Sjogren's Syndrome
PubMed: 28610655
DOI: 10.3238/arztebl.2017.0354 -
European Journal of Oral Sciences Oct 2018Sjögren's syndrome is a lymphoproliferative disease with autoimmune features characterized by mononuclear cell infiltration of exocrine glands, notably the lacrimal and... (Review)
Review
Sjögren's syndrome is a lymphoproliferative disease with autoimmune features characterized by mononuclear cell infiltration of exocrine glands, notably the lacrimal and salivary glands. These lymphoid infiltrations lead to dryness of the eyes (keratoconjunctivitis sicca), dryness of the mouth (xerostomia), and, frequently, dryness of other surfaces connected to exocrine glands. Sjögren's syndrome is associated with the production of autoantibodies because B-cell activation is a consistent immunoregulatory abnormality. The spectrum of the disease extends from an organ-specific autoimmune disorder to a systemic process and is also associated with an increased risk of B-cell lymphoma. Current treatments are mainly symptomatic. As a result of the diverse presentation of the syndrome, a major challenge remains to improve diagnosis and therapy. For this purpose an international set of classification criteria for primary Sjögren's syndrome has recently been developed and validated and seems well suited for enrolment in clinical trials. Salivary gland biopsies have been examined and histopathology standards have been developed, to be used in clinical trials and patient stratification. Finally, ultrasonography and saliva meet the need of non-invasive imaging and sampling methods for discovery and validation of disease biomarkers in Sjögren's syndrome.
Topics: Biomarkers; Biopsy; Humans; Salivary Glands; Sjogren's Syndrome
PubMed: 30178554
DOI: 10.1111/eos.12536 -
Clinical and Experimental Rheumatology 2020Interstitial lung disease (ILD) is considered the most frequent and serious pulmonary complication in primary Sjögren's syndrome (pSS), with the majority of the studies... (Review)
Review
Interstitial lung disease (ILD) is considered the most frequent and serious pulmonary complication in primary Sjögren's syndrome (pSS), with the majority of the studies indicating a prevalence of about 20%, and resulting in significant morbidity and mortality. Although ILD was historically described as a late manifestation of pSS, more recently, a high variability of the time of onset of pSS-ILD has been observed and from 10 to 51% of patients can develop ILD years before the onset of pSS. Lymphocytic interstitial pneumonia is highly typical for SS, but it occurs only in a few cases, while the most common ILD pattern is nonspecific interstitial pneumonia, followed by usual interstitial pneumonia and organising pneumonia. Multidisciplinary discussion can be necessary in pSS cases with ambiguous clinical findings, when differential diagnosis with IIPs might be very difficult. Up to date, available data do not allow to establish an evidence-based treatment strategy in pSS-ILD. Glucocorticoids are empirically used, usually in association to immunosuppressive drugs, such as cyclophosphamide and mycophenolate mofetil. A better understanding of the molecular mechanisms involved in the pathogenesis of pSS should facilitate the development of new therapies. Recently, a trial showed the efficacy of the antifibrotic drug nintedanib in slowing progression of various interstitial lung diseases, including patients with connective tissue diseases. The aims of this review are to describe clinical features, imaging, pathology, together with diagnostic criteria, prognosis and management of pSS-ILD patients.
Topics: Connective Tissue Diseases; Humans; Immunosuppressive Agents; Lung Diseases, Interstitial; Prognosis; Sjogren's Syndrome
PubMed: 33095142
DOI: No ID Found -
Rheumatic Diseases Clinics of North... Aug 2018Dry eye and dry mouth symptoms are each reported by up to 30% of persons more than 65 years of age, particularly in women. Medication side effects are the most common... (Review)
Review
Dry eye and dry mouth symptoms are each reported by up to 30% of persons more than 65 years of age, particularly in women. Medication side effects are the most common contributing factors. The evaluation of these symptoms requires measures of ocular and oral dryness. Sjögren syndrome is the prototypical disease associated with dryness of the eyes and mouth and predominantly affects women in their perimenopausal and postmenopausal years. In addition to topical treatment of the mucosal dryness, patients with Sjögren syndrome may require treatment with systemic immunomodulatory and immunosuppressive agents to manage a variety of extraglandular manifestations.
Topics: Aged; Diagnosis, Differential; Disease Management; Dry Eye Syndromes; Humans; Sjogren's Syndrome; Xerostomia
PubMed: 30001784
DOI: 10.1016/j.rdc.2018.03.002 -
Frontiers in Immunology 2023Systemic lupus erythematosus (SLE), antiphospholipid syndrome (APS), and Sjögren's syndrome (SS) are heterogeneous autoimmune diseases. Severe manifestations and... (Review)
Review
Systemic lupus erythematosus (SLE), antiphospholipid syndrome (APS), and Sjögren's syndrome (SS) are heterogeneous autoimmune diseases. Severe manifestations and refractory/intolerance to conventional immunosuppressants demand other options, namely biological drugs, and small molecules. We aimed to define evidence and practice-based guidance for the off-label use of biologics in SLE, APS, and SS. Recommendations were made by an independent expert panel, following a comprehensive literature review and two consensus rounds. The panel included 17 internal medicine experts with recognized practice in autoimmune disease management. The literature review was systematic from 2014 until 2019 and later updated by cross-reference checking and experts' input until 2021. Preliminary recommendations were drafted by working groups for each disease. A revision meeting with all experts anticipated the consensus meeting held in June 2021. All experts voted (agree, disagree, neither agree nor disagree) during two rounds, and recommendations with at least 75% agreement were approved. A total of 32 final recommendations (20 for SLE treatment, 5 for APS, and 7 for SS) were approved by the experts. These recommendations consider organ involvement, manifestations, severity, and response to previous treatments. In these three autoimmune diseases, most recommendations refer to rituximab, which aligns with the higher number of studies and clinical experience with this biological agent. Belimumab sequential treatment after rituximab may also be used in severe cases of SLE and SS. Second-line therapy with baricitinib, bortezomib, eculizumab, secukinumab, or tocilizumab can be considered in SLE-specific manifestations. These evidence and practice-based recommendations may support treatment decision and, ultimately, improve the outcome of patients living with SLE, APS, or SS.
Topics: Humans; Antiphospholipid Syndrome; Sjogren's Syndrome; Rituximab; Lupus Erythematosus, Systemic; Biological Products; Biological Therapy
PubMed: 37138867
DOI: 10.3389/fimmu.2023.1117699 -
Scandinavian Journal of Immunology Mar 2022Why should we explore and study disease mechanisms? This is particularly important when we are dealing with complex pathogenesis without a direct causal agent, for... (Review)
Review
Why should we explore and study disease mechanisms? This is particularly important when we are dealing with complex pathogenesis without a direct causal agent, for example, syndromes with multiple organ involvements. Sjögren's syndrome is definitely such an entity. Also, there are a number of reasons for such studies such as disclosing the aetiology, to identify biomarkers for diagnosis and assessment of the disease process and monitor response to treatment, to determine targets for treatment, to define critical items in classification criteria, amongst others. Samples available for the study of disease mechanisms in Sjögren's syndrome have included serum (autoantibodies, cytokines), DNA (gene profiling, GWAS), cells (phenotypes/flow cytometry, proportion of cells/CyTOF), tissue (focal inflammation, germinal centres, mass cytometry), and saliva (proteomics, biochemistry, mucosal immunity). An original explanatory concept for the pathogenesis of Sjögren's syndrome proposed a specific and self-perpetuating immune-mediated loss of exocrine tissue as the principal cause of glandular hypofunction. This hypothesis however falls short of accommodating several Sjögren's syndrome-related phenomena and experimental findings. Today, the emergence of advanced bio-analytical platforms has further enabled the identification of central pathogenic processes and potential biomarkers. The purpose of this minor review is to highlight a selection of previous but also recent and novel aspects on the disease mechanisms in Sjögren's syndrome.
Topics: Animals; Biomarkers; Humans; Saliva; Serum; Sjogren's Syndrome
PubMed: 35073430
DOI: 10.1111/sji.13145