-
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 -
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 -
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 -
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 -
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