Lessons From Diseases Mimicking Sjögren’s Syndrome (original) (raw)
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Arthritis & Rheumatism, 1980
We prospectively evaluated 24 patients with systemic lupus erythematosus for clinical, histologic, and serologic evidence of sicca syndrome. Abnormalities suggestive of sicca syndrome in patients with systemic lupus were identified by parotid scan (58%), questionnaire (-%), labial biopsy (50%), Schirmer's test (21%), and parotid flow rate (13%). An increased frequency of autoantibodies to gamma globulin and cellular antigen SS-A was detected in lupus patients with histologic changes on labial biopsy. The serologic findings are similar to those of patients with sicca syndrome alone with the exception of the reduced incidence of anti-SS-B in patients with systemic lupus.
Sjögren’s Syndrome: Concerted Triggering of Sicca Conditions
Journal of Immunology Research
Aim. The aim of this study was to evaluate the expression of persistence of mumps virus and some cells that interact with viral infection in the focus of the autoimmune epithelitis and peripheral blood of Sjögren’s syndrome patients in comparison to patients with rheumatoid arthritis (RA) and nonautoimmune sicca syndrome (nSS). Materials and Methods. 126 patients (119 women and 7 men) were grouped into four groups: (1) patients with primary Sjögren’s syndrome (pSS), (2) patients with secondary Sjögren’s syndrome due to rheumatoid arthritis (sSS), (3) patients with rheumatoid arthritis (RA), and (4) patients with nonautoimmune sicca syndrome (nSS). Immunohistochemical analysis of immune response to the suggested silent persistence of mumps virus in the minor labial salivary gland biopsies and flow cytometric analysis of blood cells was done. Results. Immunohistochemical signs of mumps virus persistence were found in the minor salivary glands of all study groups. Also, a significantly...
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, ...
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.
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
The frequency of sicca symptoms and Sjögren's syndrome in patients with systemic sclerosis
International Journal of Rheumatic Diseases, 2012
ObjectiveThe objectives are to detect the frequency of sicca symptoms and Sjögren's syndrome (SS) in patients with systemic sclerosis (SSc) based on the diagnostic criteria of the American–European Consensus Group (AECG) and to evaluate demographic, clinical and serologic characteristics.Patients and methodOne hundred and eighteen SSc patients referred to our hospital were included in this study. All SSc patients were questioned with respect to sicca symptoms. Levels of rheumatoid factor (RF), anti‐nuclear antibodies (ANA), anti‐Ro and anti‐La antibodies were measured; non‐stimulated saliva amounts were recorded and Schirmer test and break‐up time were applied to all patients. Minor salivary gland biopsy samples were obtained from those patients giving ≥ 3 positive answers to sicca symptom questions, patients with positive xerostomia/xerophthalmia test results, and patients with at least one antibody being positive. Patients presenting with grade 3 and/or grade 4 sialoadenitis b...
Arthritis & Rheumatism, 2006
Objective. To determine the prevalence of sicca symptoms and Sjögren's syndrome (SS) in a 2-center prospective series of patients with systemic sclerosis (SSc), using the American-European Consensus Group criteria for SS. Methods. Consecutive SSc patients hospitalized for followup care were evaluated for sicca symptoms. When the initial clinical evaluation yielded positive findings, a labial salivary gland biopsy was performed; histologic analysis evaluated focal lymphocytic sialadenitis and/or glandular fibrosis. Computed tomography and respiratory function tests were used to assess pulmonary fibrosis. Results. We included 133 SSc patients (mean ؎ SD age 55 ؎ 13 years; mean ؎ SD disease duration 6.5 ؎ 6 years). Eighty-one patients had limited cutaneous SSc (lcSSc). Ninety-one patients (68%) had sicca syndrome. Histologic analysis revealed fibrotic involvement in 50 of these 91 patients, but labial salivary gland fibrosis was not associated with any organ involvement we evaluated. Nineteen of the 133 patients (14%) had SS. In this subgroup, lcSSc was present at a significantly higher frequency (18 of 19 patients) than in the remaining patients with sicca syndrome (39 of 72 patients) and the patients without sicca syndrome (24 of 42
TURKISH JOURNAL OF MEDICAL SCIENCES
Background/aim: This study aimed to investigate the prevalence of sicca symptoms and secondary Sjögren's syndrome (SjS) in patients with systemic sclerosis (SSc). Also this study aimed to evaluate the expression of α-smooth muscle actin (α-SMA) in minor salivary gland (MSG) specimens, a possible marker of fibrosis responsible for myofibroblastic transformation. Materials and methods: Patients with SSc who were followed in Rheumatology outpatient clinic at a university hospital evaluated. The questionnaire of sicca symptoms and classification of SjS were evaluated according to the American-European Consensus Group (AECG) criteria. Histopathologic evaluations were done in MSG specimens investigating the presence of focal lymphocytic sialadenitis and glandular fibrosis, also assessing the expression of α-SMA. Results: This cross-sectional study included 102 patients with SSc [91 females (89%), mean age 52.5 ± 12 years]. In this cohort 76 (75%) patients had sicca symptoms and 36 (35.3%) patients fulfilled the AECG criteria for SjS; all with limited form. Having SjS found to be associated with older age and the presence of positive anti-SS-A antibodies. On histopathologic examinations, glandular fibrosis was observed in 67 (80%) and lymphocytic sialadenitis was detected in 38 (45%) patients; but only 7 samples were positive for α-SMA. Conclusion: This study suggested sicca symptoms were found to be very common among patients with SSc. Also secondary SjS was detected in nearly one-third of patients with SSc; especially in limited subtype. Anti SS-A positivity and older age were detected as predictors for SjS. Histopathologic evaluations showed significant glandular fibrosis but rare α-SMA staining in patients with SSc.