-
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 -
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 -
Bratislavske Lekarske Listy 2012Sjögren´s syndrome is a systemic autoimmune disease characterized by a reduced function of exocrine glands, mainly salivary and lacrimal glands based on chronic... (Review)
Review
Sjögren´s syndrome is a systemic autoimmune disease characterized by a reduced function of exocrine glands, mainly salivary and lacrimal glands based on chronic plasmocellular infiltration. Syndrome includes affection of glands in gastrointestinal system, respiratory apparatus, skin, and vaginal mucosa. I tis described separately as a primary Sjögren´s syndrome or together with other inflammatory rheumatic diseases as a secondary Sjögren´s syndrome. Advanced diagnostic is based mainly on serologic examinations. Treatment of patients reflects the form of Sjögren´s syndrome. Despite recent knowledge the treatment is rather symptomatic than causal (Ref. 12).
Topics: Humans; Sjogren's Syndrome
PubMed: 22897382
DOI: 10.4149/bll_2012_116 -
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 -
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 -
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 -
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 -
European Respiratory Review : An... Sep 2020Primary Sjogren syndrome (pSS) is a systemic autoimmune disease characterised by lymphocytic infiltration of exocrine glands and by a number of systemic manifestations,... (Review)
Review
Primary Sjogren syndrome (pSS) is a systemic autoimmune disease characterised by lymphocytic infiltration of exocrine glands and by a number of systemic manifestations, including those regarding the lung. Pulmonary involvement in pSS includes interstitial lung disease (ILD) and airway disease, together with lymphoproliferative disorders.Patients with pSS-ILD report impaired health-related quality of life and a higher risk of death, suggesting the importance of early diagnosis and treatment of this type of pulmonary involvement. In contrast, airway disease usually has little effect on respiratory function and is rarely the cause of death in these patients.More rare disorders can be also identified, such as pleural effusion, cysts or bullae.Up to date, available data do not allow us to establish an evidence-based treatment strategy in pSS-ILD. No data are available regarding which patients should be treated, the timing to start therapy and better therapeutic options. The lack of knowledge about the natural history and prognosis of pSS-ILD is the main limitation to the development of clinical trials or shared recommendations on this topic. However, a recent trial showed the efficacy of the antifibrotic drug nintedanib in slowing progression of various ILDs, including those in pSS patients.
Topics: Humans; Lung Diseases; Sjogren's Syndrome
PubMed: 32817113
DOI: 10.1183/16000617.0021-2020 -
Lancet (London, England)Sjögren's syndrome is a chronic autoimmune disorder of the exocrine glands with associated lymphocytic infiltrates of the affected glands. Dryness of the mouth and eyes... (Review)
Review
Sjögren's syndrome is a chronic autoimmune disorder of the exocrine glands with associated lymphocytic infiltrates of the affected glands. Dryness of the mouth and eyes results from involvement of the salivary and lacrimal glands. The accessibility of these glands to biopsy enables study of the molecular biology of a tissue-specific autoimmune process. The exocrinopathy can be encountered alone (primary Sjögren's syndrome) or in the presence of another autoimmune disorder such as rheumatoid arthritis, systemic lupus erythematosus, or progressive systemic sclerosis. A new international consensus for diagnosis requires objective signs and symptoms of dryness including a characteristic appearance of a biopsy sample from a minor salivary gland or autoantibody such as anti-SS-A. Exclusions to the diagnosis include infections with HIV, human T-lymphotropic virus type I, or hepatitis C virus. Therapy includes topical agents to improve moisture and decrease inflammation. Systemic therapy includes steroidal and non-steroidal anti-inflammatory agents, disease-modifying agents, and cytotoxic agents to address the extraglandular manifestations involving skin, lung, heart, kidneys, and nervous system (peripheral and central) and haematological and lymphoproliferative disorders. The most difficult challenge in diagnosis and therapy is patients with symptoms of fibromyalgia (arthralgia, myalgia, fatigue) and oral and ocular dryness in the presence of circulating antinuclear antibodies.
Topics: Humans; Sjogren's Syndrome
PubMed: 16039337
DOI: 10.1016/S0140-6736(05)66990-5 -
Nature Reviews. Rheumatology May 2023In primary Sjögren syndrome (pSS), chronic inflammation of exocrine glands results in tissue destruction and sicca symptoms, primarily of the mouth and eyes. Fatigue,... (Review)
Review
In primary Sjögren syndrome (pSS), chronic inflammation of exocrine glands results in tissue destruction and sicca symptoms, primarily of the mouth and eyes. Fatigue, arthralgia and myalgia are also common symptoms, whereas extraglandular manifestations that involve the respiratory, nervous and vascular systems occur in a subset of patients. The disease predominantly affects women, with an estimated female to male ratio of 14 to 1. The aetiology of pSS, however, remains incompletely understood, and effective treatment is lacking. Large-scale genetic and epigenetic investigations have revealed associations between pSS and genes in both innate and adaptive immune pathways. The genetic variants mediate context-dependent effects, and both sex and environmental factors can influence the outcome. As such, genetic and epigenetic studies can provide insight into the dysregulated molecular mechanisms, which in turn might reveal new therapeutic possibilities. This Review discusses the genetic and epigenetic features that have been robustly connected with pSS, putting them into the context of cellular function, carrier sex and environmental challenges. In all, the observations point to several novel opportunities for early detection, treatment development and the pathway towards personalized medicine.
Topics: Humans; Female; Male; Sjogren's Syndrome; Inflammation; Treatment Outcome; Epigenesis, Genetic
PubMed: 36914790
DOI: 10.1038/s41584-023-00932-6