Current issues in Sjogren's syndrome
Roland Jonsson2002, Oral Diseases
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Abstract
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This paper discusses current issues in Sjogren's syndrome, touching on its historical context, epidemiology, and the relationship between serology and systemic complications. It highlights the challenge of determining the etiology and pathogenesis of the syndrome, suggesting potential autoimmune mechanisms and genetic factors involved. The work underscores the need for better diagnostic tools and understanding of genetic markers to enhance prognosis and treatment strategies.
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Sjögren’s syndrome, the old and the new
Best Practice & Research Clinical Rheumatology, 2012
Clinical and laboratory findings of primary Sjögren's syndrome patients without sicca symptoms
Clinical and Experimental Rheumatology, 2022
Objective Primary Sjögren's syndrome (pSS) is a systemic autoimmune disease characterised by oral and eye dryness. A minority of patients can present without dryness but studies on their clinico-laboratory manifestations are scarce. Our purpose was to describe the clinical phenotype of pSS patients lacking sicca symptoms. Methods From a total of 1738 consecutive pSS patients fulfilling the 2016 ACR-EULAR criteria, those who presented without sicca symptoms were identified (non-dryness group). Their medical data was collected and compared with 2 control groups: a) the remaining unmatched sicca pSS patients with both oral and eye dryness (unmatched dryness group) and b) matched sicca pSS patients according to age, sex, and disease duration, in 1:2 ratio (matched dryness group). Results Thirty-eight (2.19%) patients lacked sicca manifestations presenting mainly with arthralgias (47%), parotid enlargement (24%), Raynaud's phenomenon (11%) and persistent lymphadenopathy (11%) that led them to be evaluated for pSS. Non-dryness pSS patients were younger than the unmatched sicca controls, displaying a higher frequency of anti-Ro/SSA antibodies (100% vs. 79.7%, p<0.001), ANA positivity (100% vs. 90.4%, p<0.001), neutropenia (20.8% vs. 7.5%, p=0.04) and thrombocytopenia (13.8% vs. 4.2%, p=0.04). They also had lower frequency of positive ocular tests compared to both unmatched and matched dryness patients. No differences were found between non-dryness pSS patients and both control groups regarding focus score or any other extraglandular manifestation. Conclusion pSS patients without sicca complaints constitute a distinct phenotype involving younger patients, sharing common immunopathologic mechanisms with typical sicca patients.
Natural history and Predictors of Progression to Sjögren's Syndrome Among Participants of the SICCA registry
Arthritis care & research, 2017
To explore changes in the phenotypic features of Sjoögren's syndrome (SS), and in SS status among participants in the Sjoögren's International Collaborative Clinical Alliance (SICCA) registry over a 2 to 3-year interval. All participants in the SICCA registry who were found to have any objective measures of salivary hypofunction, dry eye, focal lymphocytic sialadenitis in minor salivary gland biopsy, or anti-SSA/B antibodies, were recalled over a window of 2 to 3 years after their baseline examinations to repeat all clinical examinations and specimen collections to determine whether there was any change in phenotypic features and in SS status. As of September 15, 2013, 3,514 participants had enrolled in SICCA, and among 3,310 eligible, 771 presented for a follow-up visit. Among participants found to have SS using the 2012 ACR classification criteria, 93% again met the criteria after 2 to 3 years, and this proportion was 89% when using the 2016 ACR-EULAR criteria. Among those...
Sjögren’s syndrome: still not fully understood disease
Rheumatology International, 2014
hyper-reactivity and polyclonal production of immunoglobulins, and as a consequence, autoantibodies against pSS affected exocrine glands, especially lacrimal glands and salivary glands, but also other external glands such as the pancreas, mucous glands of the gastrointestinal and respiratory tract or bile secretion. In some patients, abnormal H+ secretion in the distal tubules has also been observed and caused distal renal tubular acidosis (type 1 RTA). In connection with the clinical symptoms which will result from the degree of attachment of these glands and epithelial injury, the pSS often uses the term "autoimmune epithelitis" or "autoimmune exocrinopathy" for better imaging of primary initiation sites of inflammation and autoimmunization. A brief history First lacrimal and salivary glands enlargement was described in a lecture of a Polish surgeon Jan Mikulicz-Radecki in 1888. In 1925, French dermatologists Henri Gougerot described a few cases of atrophy of the salivary glands with dryness of eyes, mouth and vagina. Later in 1933, Swedish ophthalmologists Henrik Sjögren in his doctoral thesis described keratoconjunctivitis sicca, and his description became a basis for pSS picture. Epidemiology It is estimated that primary Sjögren's syndrome occurs from 0.1 to 3.0 % in general population. The disease is more common for women (female/male ratio 9:1), mainly between the ages of 40-60, with the disease most frequently occurring around 50 years of age. Pathogenesis of pSS is not clear, nowadays several factors responsible for the development of the disease, such as Abstract Primary Sjögren's syndrome is an autoimmune disorder with external exocrine glands dysfunction and multiorgan involvement. The pathogenesis of primary Sjogren's syndrome is still unclear; however, our knowledge of the involvement of different cells (e.g., B and T cells, macrophages and dendritic cells) and pathways (BAFF/APRIL and interferons) leading to the development of autoimmunity is continually expanding. For clinicians, the most frequent symptoms are dryness of eyes and mouth, but often the patients have musculoskeletal symptoms and systemic manifestations. However, the increased risk of lymphoproliferative disorders in this group of patients, most commonly B-cell marginal zone lymphoma, is particularly important. Recent separation of IgG4-related diseases and attempts to create further diagnostic criteria for pSS testify to the difficulties, and at the same time a large interest, in understanding the disease so as to allow the effective treatment. This article draws attention to the problems faced by the clinician wishing to securely identify pSS by using accurate laboratory biomarkers and useful imaging tools and predict the development of complications associated with this, still not fully understood, autoimmune disease.
Sjogren's syndrome: An overview
International Journal of Nursing Education and Research
Primary Sjögren Syndrome in Spain
Medicine, 2008
We conducted the current study to characterize the clinical presentation of primary Sjögren syndrome (SS) in a large cohort of Spanish patients and to determine whether epidemiologic, clinical, and analytical features modulate disease expression. Patients were from the GEMESS Study group, which was formed in 2005 and included 12 Spanish reference centers. By March 2007, the database included 1010 consecutive patients, recruited since 1994, both incident and prevalent cases.
Sjögren’s syndrome: a systemic autoimmune disease
Clinical and Experimental Medicine, 2021
Sjögren's syndrome is a chronic autoimmune disease characterized by ocular and oral dryness resulting from lacrimal and salivary gland dysfunction. Besides, a variety of systemic manifestations may occur, involving virtually any organ system. As a result, the disease is characterized by pleomorphic clinical manifestations whose characteristics and severity may vary greatly from one patient to another. Sjögren's syndrome can be defined as primary or secondary, depending on whether it occurs alone or in association with other systemic autoimmune diseases, respectively. The pathogenesis of Sjögren's syndrome is still elusive, nevertheless, different, not mutually exclusive, models involving genetic and environmental factors have been proposed to explain its development. Anyhow, the emergence of aberrant autoreactive B-lymphocytes, conducting to autoantibody production and immune complex formation, seems to be crucial in the development of the disease. The diagnosis of Sjögren's syndrome is based on characteristic clinical signs and symptoms, as well as on specific tests including salivary gland histopathology and autoantibodies. Recently, new classification criteria and disease activity scores have been developed primarily for research purposes and they can also be useful tools in everyday clinical practice. Treatment of Sjögren's syndrome ranges from local and symptomatic therapies aimed to control dryness to systemic medications, including disease-modifying agents and biological drugs. The objective of this review paper is to summarize the recent literature on Sjögren's syndrome, starting from its pathogenesis to current therapeutic options.
Serologic features of primary Sjögren’s syndrome: clinical and prognostic correlation
International Journal of Clinical Rheumatology, 2012
Frequency and predictive value of the clinical manifestations in Sjogren's syndrome
Journal of Oral Pathology and Medicine, 2001
The purpose of this study was to examine the frequency and predictive value of glandular and extraglandular manifestations in Sjögren's syndrome (SS). The clinical profiles of 169 SS patients were compared to those of 44 non-SS controls. The specific symptoms examined were oral, ocular, vaginal, gastric, pulmonary, skin, joint and muscle pain. Statistical analyses were performed on both individual and grouped symptoms. Chi-squared analyses showed that the frequency of all symptoms was significantly higher among patients than controls. Stepwise discriminant analysis of individual symptoms suggests that the combined symptoms of dry mouth, sore mouth, and dry eyes correctly classified 93% of SS and 97.7% of the controls. While grouped gastric, muscle, psychological, vaginal, skin, nasal, and thyroid symptoms correctly classified 64.3% of SS and 86.1% of the controls. This is the first study to examine the diagnostic value of multi-system manifestation in SS. The overall results suggest that a comprehensive questionnaire of various symptoms may assist the diagnosis of SS. The high predictive value of the combined symptoms confirms their value in the evaluation of SS.
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Sjögren ' s syndrome Author : Doctor
2004
Sjögren's syndrome (SS) is a chronic autoimmune disorder. It is characterized by dysfunction and destruction of the exocrine glands associated with lymphocytic infiltrates and immunological hyperreactivity. Salivary and lacrimal glands are the most affected, thus leading to mouth and eye dryness. The disorder can occur alone (it is then known as ``primary-SS'') or in association with another autoimmune disease (it is then known as ``secondary-SS''). Prevalence of primary-SS in the general population has been estimated to be around 1 to 3%. Although patients of all ages and of both sexes may be affected, this disorder mostly affects women (9:1 female to male ratio) in their fourth or fifth decade of life. In the majority of patients, SS has an indolent or slowly progressive course with disease confined in exocrine glands. Mild rheumatic complaints have also been reported. At presentation or during the course of the disease, almost one third of the primary-SS patients experiences a more generalized disease, which does not usually evolve to the failure of the affected organ. However, stringent follow-up should be instituted in patients with adverse prognosis predictors such as purpura, low C4-complement levels or mixed monoclonal cryoglobulins. Risk of developing lymphoma and mortality are higher in these patients. To date, the management of patients has primarily aimed to provide symptomatic relief to lessen mucosal dryness-induced damage and to early recognize and treat disease complications.
Application of classification criteria of Sjogren syndrome in patients with sicca symptoms: Real-world experience at a medical center
Journal of the Formosan Medical Association, 2019
Background: Patients who have symptoms of sicca, such as dry eyes and mouth, may have Sjögren's syndrome (SS). However, the conservative culture makes patients hesitate to undergo an invasive biopsy, which contributes to the difficulty of confirming a diagnosis. We aimed to identify the characteristics of patients with sicca symptoms to develop a better predictive value for each item included in the three different diagnostic criteria for SS and clarify the best diagnostic tools for the local population. Methods: This is a single-center retrospective case-control study from January 2016 to December 2017. Patients who underwent sialoscintigraphy because of clinical symptoms of xerostomia and xerophthalmia at one medical center were reviewed via the patients' electronic medical records. Results: Of 515 patients enrolled, the severity of results for sialoscintigraphy and Schirmer's test was correlated with a diagnosis of SS and generated receiver operator characteristic
Lessons From Diseases Mimicking Sjögren’s Syndrome
Clinical Reviews in Allergy & Immunology, 2007
Diagnostic and Prognostic Features of Sjögren’s Syndrome
Insights and Perspectives in Rheumatology, 2012
Sicca Symptoms and Its Relationship with Primary Sjögren’s Syndrome in Geriatric Patients
European Journal of Geriatrics and Gerontology
Primary Sjögren's Syndrome (pSS) is a systemic autoimmune disease, where the exocrine glands are mainly affected. Permanent dry mouth and dry eye occur due to functional and structural impairment of salivary and tear glands. According to the European League Against Rheumatism (EULAR)-SS task force, sicca symptoms are the most common manifestation of SS, with up to 98% of cases (4). The frequency of dry mouth and dry eye increases with age (5). pSS can occur in patients of all ages, it mainly manifests in the fourth and fifth decade of life. The prevalence of pSS ranges between 0.01-5% (6). The difference in the prevalence ratios is generally due to the age of the studied population, the differences in sample sizes, and the use of different classification criteria for pSS. Precise data could
Clinical and laboratory evaluation of sicca complaints: distinctive aspects of primary, secondary and non-Sjogren syndrome
Advances in Rheumatology
Background Sjögren Syndrome (SS) is a systemic autoimmune disease with a wide spectrum of manifestations that can lead to misdiagnosis. This study describes and compares demographic, clinical, serological, and histopathological data from subjects with SS and non-Sjögren Syndrome (NSS). It also details specific features within the primary SS (pSS) and secondary SS (sSS) groups identifying sub-groups. Methods The sample included individuals referred to an academic medical center in Brazil for investigation of SS from 2012 to 2020. Patients were retrospectively classified as primary SS (pSS), secondary SS (sSS), or NSS, based on the American-European Consensus Group criteria (AECG-2002), after multi-professional clinical and laboratory evaluation. Results A total of 676 individuals were screened and 510 (75.4%) completed the assessments; 198 patients were classified as pSS, 149 as sSS, and 163 as NSS. Symptoms and glandular dysfunction tests were similar in the groups. Concerning pSS, ...
Sjögren Syndrome or Sjögren Disease? The Histological and Immunological Bias Caused by the 2002 Criteria
Clinical Reviews in Allergy & Immunology, 2009
The current 2002 classification criteria do not cover the broad clinical and immunological heterogeneity of primary Sjögren syndrome (SS), since five of the six criteria focus exclusively on glandular involvement and the remaining criterion is the mandatory presence of anti-Ro/La antibodies. The aim of this study was to analyze the clinical features of patients with a well-established diagnosis of primary SS who do not fulfill the 2002 classification criteria. Five hundred seven patients diagnosed with primary SS (1993 criteria) were consecutively included and followed up. Two hundred twenty-one (44%) patients did not fulfill the 2002 criteria. These patients were older at diagnosis (p<0.001) and had a lower frequency of parotid enlargement (p=0.002), fever (p=0.041), arthritis (p=0.041), vasculitis (p=0.050), peripheral neuropathy (p=0.002), cranial nerve involvement (p=0.015), raised erythrocyte sedimentation rate (ESR) levels (p<0.001), anemia (p<0.001), leukopenia (p=0.037), hypergammaglobulinemia (p<0.001), positive rheumatoid factor (RF; p=0.002), and cryoglobulinemia (p=0.049) in comparison with those fulfilling 2002 criteria. However, there were no significant differences in the prevalence of sicca features, diagnostic tests, overall systemic involvement, antinuclear antibodies, complement levels, development of B-cell lymphoma, or survival. Patients with anti-Ro antibodies had the highest frequencies of systemic features, hematological abnormalities, and altered immunological markers. In conclusion, patients fulfilling the 2002 criteria, who have either a specific histological diagnosis (lymphocytic infiltration) or highly specific autoantibodies (Ro/La), might well be considered to have Sjögren "disease." In contrast, etiopathogenic mechanisms other than lymphocytic-mediated epithelial damage could be involved in patients with negative Ro and negative biopsy, in whom the term Sjögren "syndrome" seems more adequate.
Laboratory features and pharmacological management of early and late-onset primary Sjögren’s syndrome
Rheumatology international, 2024
symptoms may appear years before diagnosis [2-5]. It is classified into primary (pSS) or secondary SS based on its association with other autoimmune diseases like rheumatoid arthritis (RA) or systemic lupus erythematosus (SLE) [1, 6]. It mainly affects females compared to males at a ratio of 9:1, respectively, and can occur at any age, yet the peak prevalence is approximately 50 years old [2, 5]. Although the etiology of this disorder is not fully understood, it has Introduction Sjögren's Syndrome (SS) is a systemic chronic autoimmune disorder that primarily affects the salivary gland alongside the lacrimal glands. The immune-mediated damage of SS results in dry mouth (xerostomia) and eyes (xerophthalmia). Dryness of mucosal surfaces, including airways, digestive tract, and vagina, may also be observed, which is clinically referred to as the sicca syndrome [1]. Nevertheless, Extended author information available on the last page of the article
Primary Sjögren’s Syndrome: Current Pathophysiological, Diagnostic and Therapeutic Advances
Autoimmune Disorders - Current Concepts and Advances from Bedside to Mechanistic Insights, 2011
The diagnosis of SS is currently essentially based on the American-European criteria. These criteria, 6 in total, include 2 subjective and 4 objective criteria. The subjective criteria are www.intechopen.com Autoimmune Disorders-Current Concepts and Advances from Bedside to Mechanistic Insights 42 ocular and oral symptoms, while the objective criteria are ocular signs, histopathology, salivary gland involvement, and autoantibodies. Experts have recommended making the diagnosis of SS when 4 of the 6 criteria are present, as long as histopathology or serology is positive, or when 3 of any of the 4 objective criteria are present (Vitali et al., 2002). It necessary to bear in mind that the most frequent symptoms of SS include the triad of fatigue, polyarthralgia and sicca symptoms, which are often commonly found in the general population and aging population. Many drugs have anti-cholinergic properties, which complicate the diagnostic process of SS. Exclusion criteria for the diagnosis of SS include the presence of hepatitis C and HIV viruses, sarcoidosis, prior cervical radiation, lymphoma, graft versus host disease and the use of anti-cholinergic drugs (Vitali et al., 2002). 2.1 Ocular symptoms Ocular symptoms are taken into consideration when patients complain about troublesome dry eyes, recurrent sensation of sand or gravel in the eye, or the use of tear substituent. 2.2 Oral symptoms Oral symptoms are considered pertinent when patients experience daily feeling of dry mouth, recurrent or persistent swollen salivary glands, or frequent drinking to facilitate dry food swallowing. 2.3 Ocular signs Evidence of ocular involvement relies on positive Schirmer's test (<5mm/min) or positive ocular dye score (score > 4 according to the van Bijsterveld score; Van Bijsterveld, 1969). 2.4 Histopathology A histological sign is considered positive when minor salivary gland biopsy show a focus score >1 (more than 50 lymphocytes per 4 mm 2 of tissue). 2.5 Salivary gland involvement Objective evidence of salivary gland involvement relies on low unstimulated saliva flow (< 1.5ml/15 min), abnormal sialography, or altered salivary scintigraphy. 2.6 Autoantibodies Serological signs are considered positive when the presence of antibodies to either Ro (SSA) or La (SSB) or both are detected in the serum. 2.7 New diagnostic tools To prevent excessive prescriptions of exams for establishing diagnosis of SS, newer diagnostic tools have been validated. However, these tools have not yet been included in the American-European diagnosis criteria of SS. For example, ultrasonography of salivary glands might prove to be useful to detect anatomical changes in parotid and submandibular glands, with similar diagnostic ability to sialography. Detection of hypoechoic areas, echogenic streaks, cysts and irregular gland margins are highly suggestive of SS (Tagaki et al., 2010). Parotid MRI can also prove to be an adjunct diagnostic tool to detect heterogeneity in salivary glands and specific cystic lesions (Roberts et al., 2008).
Update of Sjogren's Syndrome
Journal of Rheumatic Diseases, 2015
We reviewed the recent findings in diagnosis, pathogenesis and management of Sjögren's syndrome. New diagnostic classification criteria for Sjögren's syndrome were published by the American College of Rheumatology in 2012, and validation was completed. Salivary gland ultrasonography has been examined as a diagnostic and prognostic marker for Sjögren's syndrome, and the results have revealed that it has high specificity and low sensitivity. Disease activity assessment tools for Sjögren's syndrome have been developed and validated for upcoming clinical trials. Several associated genes were identified by genome-wide association studies, with large cohorts in Europe and China. Several single nucleotide polymorphisms of Sjögren's syndrome are related to germinal center formation and lymphoma development. New treatment modalities, including interleukin-1 receptor antagonist, anti-CD20, anti-BAFF/Blys, and CTLA4-immunoglobulin were investigated, but the optimal therapeutic compounds have yet to be found. (
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