Clinical and laboratory findings of primary Sjögren's syndrome patients without sicca symptoms (original) (raw)
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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
Scientific Reports, 2020
Understanding the impact of the disease on quality of life is crucial in patient management. in this cross-sectional study, general and oral health-related quality of life questionnaires, and thorough examinations of oral and ocular dryness were performed in age-and sex-matched patients with primary Sjögren's syndrome (pSS group), non-Sjögren's syndrome sicca (non-SS group) and healthy controls. General and oral health-related quality of life were investigated with the 36-Item Short Form Health Survey and the 14-Item Oral Health Impact Profile questionnaires, respectively. Subjective symptoms of xerostomia and ocular dryness were recorded using the Summated Xerostomia inventory and ocular Surface Disease index, respectively. clinical examinations included evaluation of clinical oral dryness scores, candida counts, unstimulated and stimulated saliva secretory rates, tear osmolarity, tear film break-up time, Schirmer I test and ocular surface staining. Both patient groups had pronounced signs and symptoms of xerostomia and ocular dryness. even though the non-SS patients had less severe clinical signs than the pSS patients, they demonstrated much poorer general and oral health-related quality of life. in conclusion, non-SS patients require more attention in order to improve their quality of life. Sjögren's syndrome (SS) is a systemic, autoimmune connective tissue disease presenting a wide range of sicca symptoms, mainly dry mouth and dry eyes that result from permanently impaired salivary and lacrimal gland function 1. Sjögren's syndrome is also associated with extraglandular renal, pulmonary, and neurological manifestations in about 30% of patients 2. Around 5% of patients may develop lymphoma which is the most severe complication 2,3. Sjögren's syndrome is defined as primary SS (pSS) when occurring in the absence of an underlying rheumatic disorder, and as secondary SS (sSS) when associated with another connective tissue disorder 4,5. The large and diverse group of patients that have sicca symptoms consists of patients with various disorders who receive medical treatments that cause hypofunction of salivary and/or lacrimal glands. However, some patients have sicca symptoms in the absence of known diseases or medication, but without the distinctive features of pSS, namely Ro/SSA and/or La/SSB autoantibodies and lymphocyte infiltration in their minor salivary glands 6. In the present paper, this diverse group of patients was named the non-SS group, in order to highlight the fact that although they present with SS-like symptomatology they do not fulfil the diagnostic criteria for SS 7-20. The oral manifestations of SS can include subjective symptoms of dry mouth (xerostomia), burning sensation on the tongue, reduced sense of taste, and difficulties in swallowing food 21. Clinical findings of dry mouth include dry and fissured tongue, lack of saliva pool and bubbly saliva, tooth decay, and candidiasis 22. The subjective symptoms of dry eyes in SS can manifest as reduced vision, ocular discomfort, scratchiness, and pain in the eyes.
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
Total body water and sicca symptoms in primary Sjögren's syndrome
Clinical and Experimental Rheumatology, 2021
Objective To evaluated the total body water (TBW) among patients with primary Sjögren's syndrome (pSS) and assess its correlation with the severity of oral and ocular sicca symptoms, and some objective sicca variables. Methods We included 85 patients and 85 controls matched by sex, age, and body mass index (BMI). We assessed the Schirmer-I test and the non-stimulated whole salivary flow (NSWSF). We evaluated ocular and oral symptoms during the past 15 days using a 0-10 visual analogue scale (VAS) (highest score=worst symptoms). We obtained the TBW by bioelectric impedance analysis. Results 80% were women (mean age 54.8 years and mean disease duration 11.5 years). TBW was similar in pSS and controls (46.8±4.6 vs. 46.9±4.5, p=0.88). TBW correlated with age (ρ=-0.25, p=0.02), disease duration (ρ=-0.30, p=0.005), BMI (ρ=-0.78, p=0.001) and ocular VAS scale (ρ=-0.28, p=0.01); but not with NSWSF, Schirmer test or oral VAS scale. When comparing patients in the lowest TBW percentile (≤25%) with the remaining patients, the former group was older, had longer disease duration, higher BMI, lower frequency of anti-Ro/SSA and antinuclear antibodies, and higher ocular VAS scores. In the multivariate analysis, the ocular VAS score (OR 1.88, 95% CI 1.08-3.2, p=0.02) and the BMI 1.92 (OR 1.4, 95% CI 1.4-2.66, p=0.0001) remained associated with a lower TBW percentage. Conclusion Patients with pSS had similar TBW percentages to control subjects. However, lower TBW percentages in the pSS were associated with higher BMI and also with the most severe ocular symptoms.
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, ...
Evaluation of Patients With Dry Eye for Presence of Underlying Sjögren Syndrome
Cornea, 2010
Purpose: To evaluate the rate of Sjögren syndrome (SS) in a cohort of patients with dry eye syndrome. Methods: Medical records of patients with a primary diagnosis of dry eye syndrome (International Classification of Diseases [ICD] code 375.15 or 370.33) were reviewed retrospectively. Patients who had 2 or more visits to a single dry eye center during a 2-year period (January 2004 to January 2006) were considered. Results: Two hundred twenty patients with dry eye syndrome were identified. A total of 57 patients (25.9%) had an underlying rheumatic condition: 25 patients (11.4%) had rheumatoid arthritis and 24 (10.9%) had primary Sjögren syndrome (PSS). Majority of the patients with rheumatoid arthritis (96%) carried the diagnosis at the time of presentation. Of all patients with PSS, only 33.3% (8/24) carried the diagnosis at the time of presentation. Fifty percent (12/24) were diagnosed as a result of the initial evaluation. Among those, only 66.6% (8/12) tested SSA (anti-Ro antibodies) or SSB (anti-La antibodies) positive. One third of patients (4/12) tested only antinuclear antibody positive at a titer of ,1/320 and required minor salivary gland biopsy for definitive diagnosis. Additional 16.7% (4/24), who were initially serologically negative, eventually underwent minor salivary gland biopsy and became diagnosed with SS. Conclusions: PSS seems to be underdiagnosed in patients with dry eye syndrome and should be the focus of diagnostic evaluations. A minor salivary gland biopsy might be required for a definitive diagnosis in a significant proportion of the patients with SS.
Scientific Reports, 2017
w and swallowing 11. As a result of reduced salivary secretion, these patients have a higher risk of developing caries, candidiasis, and mucositis and they may also suffer from bad breath (halitosis) and difficulties wearing dentures 12. Consequently, dry mouth often results in reduced quality of life 13. Dry eye disease (DED), defined by the 2007 International Dry Eye Workshop (DEWS), is "a multifactorial disease of the tears and ocular surface that results in symptoms of discomfort, visual disturbance, and tear film instability with potential damage to the ocular surface" 14. Symptoms of DED include ocular burning and foreign body sensation, soreness, stinging, irritation, reduced visual acuity, photophobia, double vision, and ocular pain. Burden of DED can vary from mild discomfort in daily activities to incapacitation in physical functioning 14. Oral and ocular symptoms in pSS can be relieved with saliva and tear substitutes and stimulants, while involvement of the extra-glandular organs such as kidneys, lungs, skin, joints and muscles may require systemic treatment 15. The range of symptoms associated with multiple organ involvement make pSS a complex disease to handle, for patients, dentists, rheumatologists and other health workers 16. Patients with SS have a 6-fold increased risk of developing non-Hodgkin's lymphoma (NHL), and the lifetime risk of developing lymphoma is 5-10% 5. Interestingly, findings from salivary gland biopsies such as the presence of germinal centres may be a predictor for lymphoma development 17. Interdisciplinary, comprehensive evaluation of pSS including rheumatological examinations, and detailed examinations of dry mouth and dry eyes together with histopathological investigations may therefore have an important role in subgrouping the patients, and in turn benefit the choice of treatment strategies. Furthermore, detailed knowledge about the interplay between symptoms and findings of oral and ocular dryness is lacking. The aim of this study was to comprehensively investigate oral and ocular symptoms and findings in pSS patients and to perform comparisons with age-and gender-matched healthy and sicca control groups. A further aim was to explore possible correlations between oral and ocular findings in the pSS group.
Journal of Autoimmunity, 1989
The prevalence of dry eyes or dry mouth, and of primary Sj6gren's syndrome (primary SS) according to the Copenhagen criteria were established in 705 randomly selected subjects, aged 52-72 years who answered a simple questionnaire, and of whom 247 (35%) reported symptoms. A subgroup with symptoms (n = 77) and a matched asymptomatic control group (n = 32) were examined with the Schimer-1 test (Slt), tear film break-up time (BUT), van Bijsterveld score (vB), unstlmulated whole sialometry (Slalo) and, in about 40% of them labial salivary gland (LSG) biopsy and salivary gland scintigraphy. Apart from four cases of keratoconjunctivitis sicca (KCS) among controls, cases of KCS (15), xerostomia (12), autoimmune sialoadenitis (6) and primary SS (6) were exclusively confined to the symtomatic group. The calculated frequencies (with 95% confidence intervals) for the whole population were 14.9 (7.3-22.6)% for KCS, 5.5(3.0-7.9)% for xerostomia and 2.7 (l~l-4.5)~~ for autoimmune sialoadenltis and primary SS. The serum levels of ANA and RF were similar in the two groups, though those of anti-SS-B/La antibodies were higher in the group with symptoms (P<O.Ol).
British Journal of Ophthalmology, 2012
To investigate the prevalence and predictive factors of Sjögren's syndrome (SS) in a cohort of patients with burning mouth symptoms. Methods: A total of 125 patients with burning mouth symptoms were enrolled in a prospective study and assessed for the presence of SS. The severity of oral symptoms was evaluated by using questionnaires. Salivary flow rates and salivary scintigraphy were used to evaluate salivary function. Patient laboratory work-ups were reviewed, and SS was diagnosed by a rheumatologist based on the American-European Consensus Group criteria. The differences between the SS patient group and the non-SS patient group were analyzed with chi-square test or t test. Results: A total of 12 of the 125 enrolled patients (9.5%) had a positive autoimmune antibody test, and 6 (4.8% of the entire cohort) had SS (4 [3.2%] primary and 2 [1.6%] secondary). Patients with SS exhibited significantly decreased hemoglobin levels, an increased erythrocyte sedimentation rate, and an increased prevalence of autoantibody positive results compared to non-SS patients. Salivary scintigraphy showed that the uptake ratio of the submandibular gland in SS patients was decreased significantly. Conclusion: The prevalence of SS in patients with burning mouth symptoms was 4.8%. Therefore, clinicians who treat patients with burning mouth symptoms should evaluate laboratory findings and salivary functions to identify patients with SS.