Increased risk of aortic aneurysm and dissection in patients with Sjögren’s syndrome: a nationwide population-based cohort study in Taiwan

ObjectivesSjögren’s syndrome (SS) is a systemic autoimmune disorder. Several molecular pathways and the activation of matrix metalloproteinases associated with the pathogenesis of SS participate in the initiation and progression of aortic aneurysm (AA) and aortic dissection (AD). In this study, we aimed to evaluate whether patients with SS exhibit an increased risk of AA or AD.MethodsWe conducted a retrospective cohort study using a database extracted from Taiwan’s National Health Insurance Research Database. All medical conditions for each case and control were categorised using the International Classification of Diseases, Ninth Revision. HRs and 95% CIs for associations between SS and AA/AD were estimated using Cox regression and adjusted for comorbidities.ResultsOur analyses included 10 941 SS cases and 43 764 propensity score-matched controls. Compared with the controls, the patients with SS exhibited a significantly increased risk of developing an AA or AD (adjusted HR=3.642, p<0.001). Subgroup analysis revealed that compared with patients without SS, patients with primary and secondary SS both exhibited a significantly increased risk of developing AA or AD (adjusted HR=1.753, p=0.042; adjusted HR=3.693, p<0.001).ConclusionPatients with SS exhibit increased risks of developing AA or AD, and healthcare professionals should be aware of this risk when treating patients with SS. Increased aortic surveillance may be required for patients with SS.

Download Full-text

Association between Periodontal Disease and Subsequent Sjögren’s Syndrome: A Nationwide Population-Based Cohort Study

International Journal of Environmental Research and Public Health ◽

10.3390/ijerph16050771 ◽

2019 ◽

Vol 16 (5) ◽

pp. 771 ◽

Cited By ~ 1

Author(s):

Chien-Yu Lin ◽

Chien-Fu Tseng ◽

Jui-Ming Liu ◽

Heng-Chang Chuang ◽

Wei-Te Lei ◽

...

Keyword(s):

Periodontal Disease ◽

Sjögren’S Syndrome ◽

Sjögren's Syndrome ◽

Sjogren’S Syndrome ◽

Sjogren's Syndrome ◽

Cox Proportional Hazards ◽

Research Database ◽

Increased Risk ◽

Cardinal Symptom ◽

Sjögren‘S Syndrome

Xerostomia (dry mouth) is the cardinal symptom of Sjögren’s syndrome (SS), which is an autoimmune disease involving the exocrine glands and other organs. Xerostomia may predispose patients to periodontal disease (PD) and an association between SS and PD has been reported. This association may be bidirectional; therefore, we conducted this study to investigate the risk of SS in patients with PD using data from the National Health Insurance Research Database of Taiwan. A total of 135,190 patients were enrolled in our analysis. In all, 27,041 patients with PD were matched by gender, age, insured region, urbanization and income, with cases and controls in a 1:4 ratio. Both groups were followed and the risks of SS were calculated by Cox proportional hazards regression. Finally, 3292 (2.4%) patients had newly diagnosed SS. Patients with PD had a significantly higher risk of subsequent SS (903 (3.3%) vs. 2389 (2.2%), adjusted hazard 1.47, 95% confidence interval: 1.36–1.59). In conclusion, patients with PD had an approximately 50% increased risk of subsequent SS. Physicians should be aware of the symptoms and signs of SS in patients with PD.

Download Full-text

Cardiovascular Protection of Hydroxychloroquine in Patients with Sjögren’s Syndrome

Journal of Clinical Medicine ◽

10.3390/jcm9113469 ◽

2020 ◽

Vol 9 (11) ◽

pp. 3469

Author(s):

Deng-Ho Yang ◽

Yu-Hsun Wang ◽

Lung-Fa Pan ◽

James Cheng-Chung Wei

Keyword(s):

Sjögren’S Syndrome ◽

Sjögren's Syndrome ◽

Subclinical Atherosclerosis ◽

Medication Possession Ratio ◽

Sjogren’S Syndrome ◽

Sjogren's Syndrome ◽

Increased Risk ◽

Artery Disease ◽

One Year ◽

Sjögren‘S Syndrome

Sjögren’s syndrome (SS) is a chronic systemic inflammation disease with clinical presentation of dry eye, dry mouth, and polyarthralgia. Active inflammation is associated with an increased risk of associated arterial stiffness or subclinical atherosclerosis-related cardiovascular events. We used the longitudinal health insurance database of Taiwan, which includes one million participants, to evaluate the relationship between the clinical medication of hydroxychloroquine (HCQ) and the development of coronary artery disease (CAD). In total, 1674 patients with SS receiving HCQ medication were included after exclusion for previous CAD. Altogether, 1142 SS patients were included for evaluation after follow-up for more than one year. After adjusting for age, gender, medications, and chronic comorbidities, a significantly decreased hazard ratio (HR) for developing CAD was found among SS patients with higher medication possession ratio (MPR) of HCQ (HR = 0.49, 95% confidence interval, CI: 0.26–0.94) when compared with low MPR of HCQ. A low HR for CAD was observed in SS patients with a high cumulative dose of at least 100,267 mg of HCQ (HR = 0.25, 95% CI: 0.09–0.66). Long-term HCQ therapy may decrease the HR of CAD in SS patients. The significant cardiovascular protective effect of HCQ therapy was observed in our study.

Download Full-text

Autoimmune/Inflammatory Arthritis Associated Lymphomas: Who Is at Risk?

BioMed Research International ◽

10.1155/2016/8631061 ◽

2016 ◽

Vol 2016 ◽

pp. 1-11 ◽

Cited By ~ 8

Author(s):

Sujani Yadlapati ◽

Petros Efthimiou

Keyword(s):

Sjögren’S Syndrome ◽

Recent Literature ◽

Sjögren's Syndrome ◽

Sjogren’S Syndrome ◽

Sjogren's Syndrome ◽

Autoimmune Arthritis ◽

Malignant Lymphomas ◽

Increased Risk ◽

Disease Specific ◽

Sjögren‘S Syndrome

Specific autoimmune and inflammatory rheumatic diseases have been associated with an increased risk of malignant lymphomas. Conditions such as rheumatoid arthritis (RA), primary Sjögren’s syndrome (pSS), systemic lupus erythematosus (SLE), dermatomyositis, and celiac disease have been consistently linked to malignant lymphomas. Isolated cases of lymphomas associated with spondyloarthropathies and autoinflammatory diseases have also been reported. Direct association between autoimmunity and lymphomagenesis has been reinforced by large epidemiological studies. It is still uncertain whether disease specific determinants or phenotypic or treatment related characteristics increase likelihood of lymphomagenesis in these patients. For example, recent literature has indicated a positive correlation between severity of inflammation and risk of lymphomas among RA and Sjögren’s syndrome patients. It is also debated whether specific lymphoma variants are more commonly seen in accordance with certain chronic autoimmune arthritis. Previous studies have revealed a higher incidence of diffuse large B-cell lymphomas in RA and SLE patients, whereas pSS has been linked with increased risk of mucosa-associated lymphoid tissue lymphoma. This review summarizes recent literature evaluating risk of lymphomas in arthritis patients and disease specific risk determinants. We also elaborate on the association of autoimmune arthritis with specific lymphoma variants along with genetic, environmental, and therapeutic risk factors.

Download Full-text

Sjögren’s syndrome—clinical features

10.1093/med/9780199642489.003.0127 ◽

2013 ◽

Cited By ~ 1

Author(s):

Simon Bowman ◽

John Hamburger ◽

Elizabeth Price ◽

Saaeha Rauz

Keyword(s):

Salivary Gland ◽

B Cell ◽

Sjögren’S Syndrome ◽

Sjögren's Syndrome ◽

Negative Impact ◽

B Cell Lymphoma ◽

Sjogren’S Syndrome ◽

Sjogren's Syndrome ◽

Increased Risk ◽

Sjögren‘S Syndrome

Sjögren's syndrome is a chronic, immune-mediated, condition of unknown aetiology characterized by focal lymphocytic infiltration of exocrine glands associated with dry mouth and eyes. It occurs in its own right (primary Sjögren's syndrome, pSS), or as a late feature of other rheumatic diseases such as rheumatoid arthritis, systemic lupus erythematosus or scleroderma (secondary Sjögren's syndrome). There is a strong female bias. pSS typically affects women in their middle years with an estimated prevalence of 0.1–0.6%. 75% of patients have anti-Ro and/or anti-La antibodies, often with raised immunoglobulin levels (hypergammaglobulinaemia). In patients without these antibodies the diagnosis can be confirmed by salivary gland biopsy. Treatment is generally symptomatic using artificial tears, saliva replacements/stimulants and good dental hygiene. Three-quarters of patients with pSS report significant fatigue with a negative impact on quality of life. This can be the most disabling symptom. Approximately 20% of patients develop systemic features including persistent salivary gland swelling, cutaneous vasculitis, peripheral neuropathy, interstitial lung disease, autoimmune cytopenias or renal tubular acidosis. Hydroxychloroquine and corticosteroids are the most widely used therapies for systemic features. There is a 44fold increased risk of mucosa-associated lymphoid tissue (MALT) B-cell lymphoma in pSS, typically affecting the salivary glands. On account of abnormalities in the B-cell system in pSS there is current interest in the use of anti-B-cell directed monoclonal antibodies to treat pSS and a number of clinical trials are in progress. This approach is already successfully in use for treating MALT lymphoma in pSS.

Download Full-text

OP0212 The Risk of Myocardial Infarction and Cerebrovascular Accident in Patients with SjÖGren's Syndrome: A General Population-Based Cohort Study: Table 1.

Annals of the Rheumatic Diseases ◽

10.1136/annrheumdis-2014-eular.3108 ◽

2014 ◽

Vol 73 (Suppl 2) ◽

pp. 142.3-143 ◽

Cited By ~ 2

Author(s):

M. Yurkovich ◽

E.C. Sayre ◽

K. Shojania ◽

A. Avina-Zubieta

Keyword(s):

Myocardial Infarction ◽

Cohort Study ◽

General Population ◽

Sjögren’S Syndrome ◽

Cerebrovascular Accident ◽

Sjögren's Syndrome ◽

Sjogren’S Syndrome ◽

Population Based ◽

Sjogren's Syndrome ◽

Sjögren‘S Syndrome

Download Full-text

Increased risk of thyroid disease in patients with Sjogren's syndrome: a systematic review and meta-analysis

PeerJ ◽

10.7717/peerj.6737 ◽

2019 ◽

Vol 7 ◽

pp. e6737 ◽

Cited By ~ 1

Author(s):

Xin Sun ◽

Li Lu ◽

Yanbin Li ◽

Rong Yang ◽

Ling Shan ◽

...

Keyword(s):

Systematic Review ◽

Sjögren’S Syndrome ◽

Thyroid Disease ◽

Fixed Effects ◽

Sjögren's Syndrome ◽

Meta Analysis ◽

Sjogren’S Syndrome ◽

Sjogren's Syndrome ◽

Increased Risk ◽

Sjögren‘S Syndrome

Background Sjogren’s syndrome (SS) is a chronic autoimmune epithelitis characterized by disruption of epithelial cells, ensuing lymphoplasmocytic infiltration of exocrine glands, and subsequent dryness of the mouth and eyes. Individuals with SS are more likely to have the thyroid disease. However, this association remains controversial. This meta-analysis aimed to evaluate the risk of thyroid disease in patients with SS. Methods We performed this systematic review by searching both English and Chinese literature databases. Random- or fixed-effects models were used to summarize the association between thyroid disease and SS. The results were subjected to meta-analysis with odds ratios (ORs) and corresponding 95% confidence intervals (CIs). Results The eight articles in this meta-analysis included 988 SS cases and 2,884 controls. Overall, the risk of thyroid disease in patients with SS was significantly increased compared with controls (OR, 3.29; 95% CI [2.08–5.21]). The risk of autoimmune thyroid disease (AITD) and non-AITD were also higher in patients with SS than in controls (OR, 3.48; 95% CI [1.59–7.63]; and OR, 2.90; 95% CI [1.51–5.57], respectively). Conclusions To the best of our knowledge, this systematic review is the first to demonstrate that the risk of thyroid disease was increased in SS compared to controls, suggesting that SS patients should be screened for thyroid disease.

Download Full-text

Increased risk of multiple myeloma in primary Sjögren’s syndrome is limited to individuals with Ro/SSA and La/SSB autoantibodies

Annals of the Rheumatic Diseases ◽

10.1136/annrheumdis-2019-216287 ◽

2019 ◽

Vol 79 (2) ◽

pp. 307-308 ◽

Cited By ~ 1

Author(s):

Johannes Mofors ◽

Albin Björk ◽

Karin E Smedby ◽

Marika Kvarnström ◽

Helena Forsblad-d'Elia ◽

...

Keyword(s):

Multiple Myeloma ◽

Sjögren’S Syndrome ◽

Sjögren's Syndrome ◽

Sjogren’S Syndrome ◽

Sjogren's Syndrome ◽

Primary Sjögren’S Syndrome ◽

Primary Sjogren’S Syndrome ◽

Primary Sjögren's Syndrome ◽

Increased Risk ◽

Sjögren‘S Syndrome

Download Full-text

Sjögren's syndrom – the review of the latest diagnostic guidelines essential for otolaryngologists

Polski Przegląd Otorynolaryngologiczny ◽

10.5604/01.3001.0013.1985 ◽

2019 ◽

Vol 8 (2) ◽

pp. 1-6

Author(s):

Karolina Kruk ◽

Anna Rzepakowska ◽

Ewa Osuch-Wójcikiewicz ◽

Kazimierz Niemczyk

Keyword(s):

Sjögren’S Syndrome ◽

Sjögren's Syndrome ◽

Sjogren’S Syndrome ◽

Sjogren's Syndrome ◽

Sicca Syndrome ◽

Diagnostic Tools ◽

Molecular Heterogeneity ◽

Increased Risk ◽

Whole Saliva ◽

Sjögren‘S Syndrome

Sjogren's syndrome (SS) is a complex connective tissue disease with autoimmune background and high clinical, radiological and molecular heterogeneity. SS is typically manifested by sicca syndrome, characterized by dry eyes and dry mouth due to autoimmune-induced inflammation of the lacrimal and salivary glands. Complications of sicca syndrome are dental caries, oral candidiasis, dysosmia, dysgeusia, difficulties in swallowing and chewing. SS may coexist with other diseases of rheumatoid and autoimmune etiology. SS is linked to a 16-fold increased risk of non-Hodgkin lymphoma. Early diagnosis results in inappropriate treatment and may slow down the course of the disease and limit extraglandular involvement. Due to diverse clinical phenotypes and symptomatology, establishing the diagnosis is often difficult. In 2016 the American-European Consensus Group (AECG) and European League Against Rheumatism (EULAR) proposed a classification system that defines SS as a systemic disease. Diagnostic tools in establishing SS diagnosis are serological tests, ultrasonography, Schirmer’s test, unstimulated whole saliva flow rate, and Ocular Staining Score. The complete curing of SS is still not possible. As a complex multisystem disease, SS requires multidisciplinary cooperation and individual diagnostic and therapeutic approaches in patients. Therapy is focused on the treatment of symptoms and prophylaxis of complications. The laryngological treatment of oral cavity symptoms in SS includes supervision of proper oral hygiene habits and adequate fluids supplementation. The EULAR Sjögren's syndrome disease activity index (ESSDAI) and Clinical Oral Dryness Score(CODS) is used to monitor disease progression and treatment effectiveness.

Download Full-text