scholarly journals Childhood-onset of primary Sjogren syndrome Phenotypic characterization at diagnosis of 158 children

2020 ◽  
Author(s):  
Manuel Ramos-Casals ◽  
Nihan Acar-Denizli ◽  
Arjan Vissink ◽  
Pilar Brito-Zerón ◽  
Xiaomei Li ◽  
...  
Keyword(s):  
Big Data ◽  
Systemic Disease ◽  
Minor Salivary Gland ◽  
Phenotypic Expression ◽  
Sjögren Syndrome ◽  
Phenotypic Characterization ◽  
Sjogren Syndrome ◽  
Childhood Onset ◽  
Minor Salivary Gland Biopsy ◽  
Primary Sjögren Syndrome

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.

scholarly journals Minor Salivary Gland Biopsy To Detect Primary Sjögren Syndrome in Patients With Interstitial Lung Disease

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 ◽  
...  
Keyword(s):  
Salivary Gland ◽  
Interstitial Lung Disease ◽  
Lung Disease ◽  
Minor Salivary Gland ◽  
Sjögren Syndrome ◽  
Sjogren Syndrome ◽  
Minor Salivary Gland Biopsy ◽  
Primary Sjögren Syndrome ◽  
Salivary Gland Biopsy

scholarly journals Sjögren Syndrome without Focal Lymphocytic Infiltration of the Salivary Glands

2019 ◽  
Vol 47 (3) ◽  
pp. 394-399 ◽  
Author(s):  
Rohan Sharma ◽  
Kaustubh S. Chaudhari ◽  
Biji T. Kurien ◽  
Kiely Grundahl ◽  
Lida Radfar ◽  
...  
Keyword(s):  
Mononuclear Cells ◽  
Systemic Disease ◽  
Minor Salivary Gland ◽  
Sjögren Syndrome ◽  
Small Subset ◽  
Sjogren Syndrome ◽  
Peripheral Blood Mononuclear ◽  
Focus Score ◽  
Extraglandular Manifestations ◽  
Elevated Expression

ObjectivePrimary Sjögren syndrome (SS) is characterized by a focal lymphocytic infiltrate in exocrine glands. We describe patients who lacked this key feature.Methods.We evaluated patients with sicca in a comprehensive clinic at which medical, dental, and ophthalmological examinations were performed. All subjects underwent a minor salivary gland biopsy with focus score calculation. Extraglandular manifestations were also determined. We categorized subjects as high, intermediate, or low in terms of expression of interferon (IFN)-regulated genes.Results.About 20% (51 of 229, 22%) of those classified as having primary SS had a focus score of zero. Compared to those with anti-Ro positivity and a focus score > 1.0, the patients with focus score of zero (who by classification criteria must be anti-Ro–positive) were statistically less likely to have anti-La (or SSB) and elevated immunoglobulin, as well as less severe corneal staining. The focus score zero patients were less likely to have elevated expression of IFN-regulated genes in peripheral blood mononuclear cells than anti-Ro–positive SS patients with a focal salivary infiltrate.Conclusion.There are only a few clinical differences between patients with primary SS with focus score zero and those with both anti-Ro and a focus score > 1.0. The small subset of focus score zero patients tested did not have elevated expression of IFN-regulated genes, but did have systemic disease. Thus, extraglandular manifestations are perhaps more related to the presence of anti-Ro than increased IFN. This may have relevance to pathogenesis of SS.

scholarly journals Accuracy of minor salivary gland biopsy in the diagnosis of Sjögren syndrome

2021 ◽  
Vol 122 (07) ◽  
pp. 454-460
Author(s):  
R. Edelstein ◽  
G. E. Kilipiris ◽  
K. Machalekova ◽  
E. Mouzalini ◽  
A. Slobodianuk ◽  
...  
Keyword(s):  
Salivary Gland ◽  
Minor Salivary Gland ◽  
Sjögren Syndrome ◽  
Sjogren Syndrome ◽  
Minor Salivary Gland Biopsy ◽  
Salivary Gland Biopsy

Cryoglobulinemia in Sjögren Syndrome: A Disease Subset that Links Higher Systemic Disease Activity, Autoimmunity, and Local B Cell Proliferation in Mucosa-associated Lymphoid Tissue

2017 ◽  
Vol 44 (8) ◽  
pp. 1179-1183 ◽  
Author(s):  
Luca Quartuccio ◽  
Chiara Baldini ◽  
Roberta Priori ◽  
Elena Bartoloni ◽  
Francesco Carubbi ◽  
...  
Keyword(s):  
Disease Activity ◽  
Activity Index ◽  
Systemic Disease ◽  
Disease Activity Index ◽  
Sjögren Syndrome ◽  
Sjogren Syndrome ◽  
Systemic Activity ◽  
Primary Sjögren Syndrome ◽  
System P ◽  
European League Against Rheumatism

Objective.To compare systemic disease activity by validated tools, i.e., the European League Against Rheumatism Sjögren Syndrome Disease Activity Index (ESSDAI) and the Clinical ESSDAI (ClinESSDAI) scores, between primary Sjögren syndrome (pSS) with positive serum cryoglobulins and pSS without serum cryoglobulins.Methods.There were 825 consecutive patients with pSS who were retrospectively evaluated.Results.The ESSDAI and the ClinESSDAI scores were significantly higher in cryoglobulin-positive patients (p < 0.0001, for both scores). Cryoglobulinemia was significantly associated with these domains: constitutional (p = 0.003), lymphadenopathy (p = 0.007), glandular (p = 0.0002), cutaneous (p < 0.0001), peripheral nervous system (p < 0.0001), hematological (p = 0.004), and biological (p < 0.0001).Conclusion.Cryoglobulin-positive patients show the highest systemic activity in pSS.

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