scholarly journals Comparison of Effect on Sicca Symptoms of Anticentromere Antibody–positive Sjögren Syndrome and Primary Sjögren Syndrome Alone

2019 ◽  
Vol 47 (6) ◽  
pp. 876-880
Author(s):  
Yifan Li ◽  
Arthur A.M. Bookman

Objective.To determine whether positive anticentromere antibody (ACA) serology affects the severity of sicca symptoms in patients with primary Sjögren syndrome (pSS).Methods.Evaluation to detect subjective and objective sicca symptoms included questionnaires, physical examination, and pathology. Cases of pSS were classified according to the 2002 American-European Consensus Group (AECG) criteria. All patients were evaluated for presence of anti-Ro, anti-La, and ACA serology. Patients with pSS were categorized into ACA+ SS and ACA–SS. The groups were compared for measures of severity of oral and ocular sicca.Results.The pSS group had 446 patients, of whom 26 were ACA+ SS. Subjective ocular sicca measured 7.0 ± 2.4 (out of 10) in ACA+ SS and 6.4 ± 2.6 in ACA–SS (p = 0.197). Objective ocular sicca measured 3.2 mm ± 1.8 mm/5 min in ACA+ SS and 4.2 mm ± 4.4 mm/5 min in ACA–SS (p = 0.038). Subjective oral sicca measured 8.5 ± 1.4 in ACA+ SS and 6.7 ± 2.4 in ACA–SS (p < 0.001). Objective oral sicca measured 0.1 ml ± 0.2 ml/15 min in ACA+ SS and 0.4 ml ± 1.0 ml/15 min in ACA–SS (p < 0.001). Only 35% of ACA+ patients with SS were anti-Ro–positive or anti-La–positive compared with 77% of the ACA–patients with SS (p < 0.001). There was no significant difference in minor salivary gland fibrosis or focus scores between ACA+ SS and ACA–patients with SS.Conclusion.ACA+ SS is associated with more severe objective ocular sicca and more severe subjective and objective oral sicca compared to ACA–SS. The majority of ACA+ patients with SS meet AECG criteria for pSS despite negative serology for anti-Ro/La antibodies.

CHEST Journal ◽  
2009 ◽  
Vol 136 (4) ◽  
pp. 1072-1078 ◽  
Author(s):  
Aryeh Fischer ◽  
Jeffrey J. Swigris ◽  
Roland M. du Bois ◽  
Steve D. Groshong ◽  
Carlyne D. Cool ◽  
...  

2014 ◽  
Vol 41 (6) ◽  
pp. 1178-1182 ◽  
Author(s):  
Naoto Yokogawa ◽  
Scott M. Lieberman ◽  
Faizan Alawi ◽  
Sharon Bout-Tabaku ◽  
Marta Guttenberg ◽  
...  

Objective.To determine an appropriate focus score cutoff for childhood Sjögren syndrome (SS).Methods.Labial salivary gland tissue from specimens from children with SS and age-matched controls was retrospectively identified and reviewed by a blinded oral pathologist.Results.The presence of any focal sialadenitis (focus score > 0 foci/4 mm2) was common among childhood SS samples but present in only 1 of 8 control samples.Conclusion.The presence of any focal lymphocytic sialadenitis in minor labial salivary gland tissue is suggestive of childhood SS and should be included in future childhood SS-specific diagnostic or classification criteria.


2013 ◽  
Vol 40 (9) ◽  
pp. 1566-1571 ◽  
Author(s):  
Efstathia K. Kapsogeorgou ◽  
Maria I. Christodoulou ◽  
Demosthenes B. Panagiotakos ◽  
Spyros Paikos ◽  
Anna Tassidou ◽  
...  

Objective.The lymphocytic infiltrates of minor salivary gland (MSG) lesions of Sjögren syndrome (SS) vary in grade and composition and are generally thought to develop in stepwise manner. Their progression over time is not well defined.Methods.We studied repetitive MSG biopsy specimens from 28 patients with primary SS.Results.The infiltration grade and prevalence of the major infiltrating cell types (T and B cells, macrophages, dendritic cells, natural killer cells) remained largely unchanged during a median 55 month biopsy time interval followup (quartiles 42–81).Conclusion.We found significant disease progression involving the development of mucosa-associated lymphoid tissue lymphoma in patients expressing adverse serologic prognostic factors, such as low serum C4 complement levels and cryoglobulinemia.


2015 ◽  
Vol 42 (7) ◽  
pp. 1119-1122 ◽  
Author(s):  
Sunil Das ◽  
DoQuyen Huynh ◽  
Hong Yang ◽  
Arnoldas Ceponis ◽  
Arthur Kavanaugh

Objective.To assess salivary gland ultrasonography (US) as a diagnostic tool for secondary Sjögren syndrome (sSS) in patients with rheumatoid arthritis (RA).Methods.Salivary gland US images from 30 patients with RA were graded using a validated semiquantitative scoring system. Sicca symptoms, oral health, and RA disease activity were assessed.Results.US changes consistent with SS were found in 40% of patients. Patients with higher US scores had more sicca symptoms as well as higher RA activity and poorer oral health.Conclusion.Salivary gland US may aid the diagnosis of sSS in patients with RA.


Author(s):  
Reyhan Köse Çobanoglu ◽  
Taşkın Şentürk

Objectives: This study aims to compare initial albumin-to-globulin ratio (AGR) in patients with rheumatoid arthritis (RA) and primary Sjögren syndrome (pSS) presenting with undifferentiated arthritis (UA) and to investigate whether there was a difference in terms of AGR between the two patient groups and healthy controls. Patients and methods: Between January 2019 and December 2019, a total of 177 patients including 96 RA (10 males, 86 females; mean age: 53.6±10.8 years; range, 21 to 74 years) and 81 pSS (5 males, 76 females; mean age: 53.2±14.1 years; range, 23 to 79 years) and 82 healthy controls (20 males, 62 females; mean age: 50.5±13.6 years; range, 20 to 79 years) were included in this case-control study. Demographic characteristics, albumin, and globulin levels of all participants were recorded. The AGR, erythrocyte sedimentation rate (ESR), C-reactive protein (CRP), rheumatoid factor (RF), anti-nuclear antibody (ANA), and anti-citrullinated protein antibodies (ACPA) were assessed. Results: The mean AGR was 1.50±0.16 in the control group, 1.48±0.24 in the RA group, and 1.30±0.23 in the pSS group, indicating a significant difference between the pSS and the other two groups (p<0.001). The receiver operating characteristic analysis revealed that the cut-off value for AGR was 1.39 (area under the curve=0.736) with a sensitivity of 0.642 and a specificity of 0.646 (p<0.001). The ESR and CRP values were higher (p<0.001), and ANA (p<0.001) and RF (p=0.003) positivity were lower in the RA group, compared to the pSS group. Conclusion: This study findings indicate that AGR is a helpful tool in the differential diagnosis of RA and pSS presenting with UA at the time of admission, and Sjögren syndrome should be considered in case of AGR ≤1.39.


2020 ◽  
Author(s):  
Manuel Ramos-Casals ◽  
Nihan Acar-Denizli ◽  
Arjan Vissink ◽  
Pilar Brito-Zerón ◽  
Xiaomei Li ◽  
...  

OBJECTIVES. To characterize the phenotypic presentation at diagnosis of childhood-onset primary Sjogren syndrome (SjS). METHODS. The Big Data Sjogren Project Consortium is an international, multicentre registry using worldwide data-sharing cooperative merging of pre-existing clinical SjS databases from the five continents. For this study, we selected those patients in whom the disease was diagnosed below the age of 19 according to the fulfilment of the 2002/2016 classification criteria. RESULTS. Among the 12,083 patients included in the Sjogren Big Data Registry, 158 (1.3%) patients had a childhood-onset diagnosis (136 girls, mean age of 14.2 years): 126 (80%) reported dry mouth, 111 (70%) dry eyes, 52 (33%) parotid enlargement, 118/122 (97%) positive minor salivary gland biopsy and 60/64 (94%) abnormal salivary US study, 140/155 (90%) positive ANA, 138/156 (89%) anti-Ro/La antibodies and 86/142 (68%) positive RF. The systemic ESSDAI domains containing the highest frequencies of active patients included the glandular (47%), articular (26%) and lymphadenopathy (25%) domains. Patients with childhood-onset primary SjS showed the highest mean ESSDAI score and the highest frequencies of systemic disease in 5 (constitutional, lymphadenopathy, glandular, cutaneous and haematological) of the 12 ESSDAI domains, and the lowest frequencies in 4 (articular, pulmonary, peripheral nerve and central nervous system) in comparison with patients with adult-onset disease. CONCLUSIONS. Childhood-onset primary SjS involves around 1% of patients with primary SjS, with a clinical phenotype dominated by sicca features, parotid enlargement and systemic disease. Age at diagnosis plays a key role on modulating the phenotypic expression of the disease.


2016 ◽  
Vol 43 (10) ◽  
pp. 1858-1863 ◽  
Author(s):  
Nemanja Damjanov ◽  
Vera Milic ◽  
Juan Carlos Nieto-González ◽  
Iustina Janta ◽  
Lina Martínez-Estupiñan ◽  
...  

Objective.To evaluate the multiobserver reliability of salivary gland ultrasonography (SGUS) for scoring greyscale (GS) parenchymal inhomogeneity and parenchymal color Doppler (CD) signal in patients with established primary Sjögren syndrome (pSS).Methods.The study comprised 2 multiobserver reliability assessments in patients with pSS in 2 European centers. The first reliability exercise was performed on 24 patients with pSS and 8 controls who were independently evaluated with GS and CD US by 5 observers at the Institute of Rheumatology, Belgrade, Serbia. The second reliability exercise was carried out on 10 patients with pSS who were independently assessed with GS and CD US by 8 observers at the Hospital G.U. Gregorio Marañón, Madrid, Spain. SGUS parenchymal inhomogeneity and parenchymal CD signal were semiquantitatively scored using a 4-grade scoring system. The multiobserver agreement was calculated by the overall agreement and Light’s κ statistics.Results.A total of 640 SGUS examinations were performed in the first reliability exercise and a total of 320 examinations in the second reliability exercise. Multiobserver reliability was good (κ = 0.71–0.79) to excellent (κ = 0.81–0.82) for GS parenchymal inhomogeneity in both exercises. There was a moderate (κ = 0.53–0.58) to good (κ = 0.70) multiobserver reliability for parenchymal CD signal in the first exercise. However, there was no agreement or only a fair agreement (κ = 0.03–0.29) for parenchymal CD signal in the second exercise.Conclusion.US may be a reliable technique in the multiobserver scoring of GS parenchymal inhomogeneity of major SG in patients with established pSS. CD scoring of SG needs further standardization to be used in multicenter studies.


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