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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 -
Medicina Clinica Feb 2023In 1933, the Swedish ophthalmologist Hendrik Sjögren was the first to suggest that behind the dryness that several of his patients presented, there could be a systemic... (Review)
Review
In 1933, the Swedish ophthalmologist Hendrik Sjögren was the first to suggest that behind the dryness that several of his patients presented, there could be a systemic disease related to an abnormal immune response. Since then, the term Sjögren's syndrome (SjS) has been used and it has been considered a minor and infrequent disorder compared to other systemic autoimmune diseases (SAD) and, consequently, with little progress both in clinical and therapeutic research. The emergence of new technologies at the end of the 20th century rapidly promoted the development of international projects of great impact and diffusion, which have completely changed this scenario, and in the last 20 years significant progress has been made in understanding the main epidemiological determinants and pathogenic mechanisms to increase the diagnostic accuracy and to design specific and individualized therapeutic strategies. Currently, SjS should be considered one of the most frequent SADs with an undoubtedly systemic phenotype beyond dryness, in which the identification of prognostic factors can allow personalized follow-up and, therefore, early therapeutic interventions that avoid severe, irreversible outcomes.
Topics: Humans; Sjogren's Syndrome; Phenotype
PubMed: 36528400
DOI: 10.1016/j.medcli.2022.10.007 -
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 -
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 -
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 -
Current Vascular Pharmacology 2020Sjögren's syndrome is a rheumatic autoimmune disease that primarily affects middle-aged women and runs a slowly progressing course with sicca symptoms being the... (Review)
Review
Sjögren's syndrome is a rheumatic autoimmune disease that primarily affects middle-aged women and runs a slowly progressing course with sicca symptoms being the prevalent manifestation. Premature atherosclerosis and increased cardiovascular (CV) morbidity and mortality are frequently encountered in rheumatic diseases characterized by significant systemic inflammation, such as the inflammatory arthritides, systemic vasculitides and systemic lupus erythematosus. In the same context, chronic inflammation and immune aberrations underlying Sjögren's syndrome are also reported to be associated with augmented risk of atherosclerosis. Increased CV disease (CVD) frequency has been found in recent meta-analyses. The involvement of the CV system is not a common feature of Sjögren's syndrome; however, specific manifestations, such as autoantibody-mediated heart block, pericarditis, pulmonary arterial hypertension and dysautonomia, have been described. This review focuses on studies addressing CV morbidity in Sjögren's syndrome and presents current data regarding distinct CV features of the disease.
Topics: Animals; Antirheumatic Agents; Autoimmune Diseases; Cardiovascular Diseases; Comorbidity; Heart Disease Risk Factors; Humans; Inflammation; Prognosis; Risk Assessment; Sjogren's Syndrome
PubMed: 31995009
DOI: 10.2174/1570161118666200129125320 -
British Journal of Hospital Medicine... Aug 2017This review discusses important aspects of the diagnosis and management of Sjögren's syndrome, covering clinical features, diagnosis and management, and summarizes... (Review)
Review
This review discusses important aspects of the diagnosis and management of Sjögren's syndrome, covering clinical features, diagnosis and management, and summarizes recent developments in diagnosis, prognostication and treatment.
Topics: Disease Management; Humans; Prognosis; Sjogren's Syndrome
PubMed: 28783408
DOI: 10.12968/hmed.2017.78.8.438 -
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 -
Rheumatic Diseases Clinics of North... Nov 2017The neurologic manifestations of primary Sjögren syndrome are varied and can be divided anatomically into 2 categories: peripheral neuropathies and central nervous... (Review)
Review
The neurologic manifestations of primary Sjögren syndrome are varied and can be divided anatomically into 2 categories: peripheral neuropathies and central nervous system (CNS) conditions. Distal sensory and sensorimotor neuropathies are the most common manifestations of peripheral nerve disease in primary Sjögren syndrome. CNS manifestations associated with primary Sjögren syndrome include focal central lesions, conditions that mimic multiple sclerosis, encephalitis, aseptic meningitis, cerebellar syndromes, movement disorders, and problems with memory, cognition, and depression. The heterogeneity of neurologic manifestations in primary Sjögren syndrome complicates the approach to treatment, which should be directed toward the underlying neuropathologic mechanism.
Topics: Central Nervous System Diseases; Humans; Peripheral Nervous System Diseases; Sjogren's Syndrome
PubMed: 29061239
DOI: 10.1016/j.rdc.2017.06.002