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.

Comparison of Labial Minor Salivary Gland Biopsies from Childhood Sjögren Syndrome and Age-matched Controls

2014 ◽  
Vol 41 (6) ◽  
pp. 1178-1182 ◽  
Author(s):  
Naoto Yokogawa ◽  
Scott M. Lieberman ◽  
Faizan Alawi ◽  
Sharon Bout-Tabaku ◽  
Marta Guttenberg ◽  
...  
Keyword(s):  
Salivary Gland ◽  
Minor Salivary Gland ◽  
Sjögren Syndrome ◽  
Classification Criteria ◽  
Sjogren Syndrome ◽  
Labial Salivary Gland ◽  
Focus Score ◽  
Salivary Gland Tissue ◽  
Gland Tissue ◽  
Control Samples

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.

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 Oral manifestations and histopathology of minor salivary gland from patients with Sjögren's Syndrome and its diagnosis in a public health system

2016 ◽  
Vol 73 (4) ◽  
pp. 297
Author(s):  
Elizângela Cristina Barbosa ◽  
Jéssica Bruna Corrêa Lindoso ◽  
Nikeila Chacon de Oliveira Conde ◽  
Luiz Fernando Souza Passos ◽  
Sandra Lúcia Euzébio Ribeiro ◽  
...  
Keyword(s):  
Public Health ◽  
Health System ◽  
Public Health System ◽  
Minor Salivary Gland ◽  
Signs And Symptoms ◽  
Salivary Flow ◽  
Sjögren Syndrome ◽  
Sjogren Syndrome ◽  
Oral Manifestations ◽  
A Minor

Objective: to analyze the oral manifestations, sialometry and the histopathology of the minor salivary glands of patients with Sjögren Syndrome (SS) treated in a public health system and diagnosed according to European American Consensus Group (EACG) criteria. Material and Methods: the 32 patients were submitted to Shirmer test, oral cavity exam, unstimulated and stimulated salivary flow measurement and, in some cases, to the serological testing. For certain patients a minor salivary gland biopsy was carried out. Results: 10 patients were diagnosed with Sjögren Syndrome (SS), among whom: 40% were diagnosed with primary (pSS) and 60% with secondary Sjögren Syndrome (sSS). All patients diagnosed with this condition complained of xerostomia and xeropthalmia. Besides xerostomia, the most frequent oral manifestations were difficulty in swallowing, dry lips, hyperemic gums and atrophic change in tongue papillae. The average scores of the Schirmer and salivary flow tests were lower in patients with sSS. Conclusion: the oral signs and symptoms are extremely important in the multisystem involvement of the SS, which emphasizes the dental surgeon responsibility in managing these patients. The establishment of multidisciplinary diagnostic centers is of utmost importance, as well as the ability to offer more objective exams in the public health system aiming at increasing the accuracy of Sjögren Syndrome diagnosis.

Cardiac Sarcoidosis: Cytokine Patterns in the Course of the Disease

2003 ◽  
Vol 127 (9) ◽  
pp. 1207-1210 ◽  
Author(s):  
Michael Schoppet ◽  
Sabine Pankuweit ◽  
Bernhard Maisch
Keyword(s):  
Time Course ◽  
Mononuclear Cells ◽  
Cardiac Sarcoidosis ◽  
Systemic Disease ◽  
Healing Process ◽  
Unknown Etiology ◽  
Enzyme Linked Immunosorbent Assay ◽  
Rapid Decline ◽  
Peripheral Blood Mononuclear ◽  
Multiple Organs

Abstract Sarcoidosis is a chronic systemic disease of unknown etiology, which is characterized by noncaseating epitheloid granulomas usually in multiple organs. Here we describe changes in cytokine mRNA expression by peripheral blood mononuclear cells (PBMCs) and changes of cytokine protein levels in plasma over a time course of 12 months in a patient with sarcoidosis confined to the heart as diagnosed by endomyocardial biopsy. Mitogen-stimulated PBMCs exhibited a more TH1 cytokine profile at onset of symptoms before immunosuppressive therapy was initiated, with a change to a TH0 response in the course of the disease as evidenced by multiplex-polymerase chain reaction. In plasma, high levels of interleukin-6 could be detected by an enzyme-linked immunosorbent assay system, with rapid decline correlating with immunosuppression and improving clinical course. These changes may point to a role of TH1 and TH2 cytokines in the pathogenesis and the healing process of cardiac sarcoidosis.

scholarly journals Minor Salivary Gland Inflammatory Lesions in Sjögren Syndrome: Do They Evolve?

2013 ◽  
Vol 40 (9) ◽  
pp. 1566-1571 ◽  
Author(s):  
Efstathia K. Kapsogeorgou ◽  
Maria I. Christodoulou ◽  
Demosthenes B. Panagiotakos ◽  
Spyros Paikos ◽  
Anna Tassidou ◽  
...  
Keyword(s):  
Salivary Gland ◽  
Minor Salivary Gland ◽  
Cell Types ◽  
Sjögren Syndrome ◽  
Time Interval ◽  
Sjogren Syndrome ◽  
T And B Cells ◽  
Biopsy Specimens ◽  
Lymphocytic Infiltrates ◽  
Significant Disease

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.

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