scholarly journals The European Community Study Group on diagnostic criteria for Sjogren's syndrome. Sensitivity and specificity of tests for ocular and oral involvement in Sjogren's syndrome.

1994 ◽  
Vol 53 (10) ◽  
pp. 637-647 ◽  
Author(s):  
C Vitali ◽  
H M Moutsopoulos ◽  
S Bombardieri
2020 ◽  
Vol 9 (10) ◽  
pp. 3299
Author(s):  
Katarzyna Błochowiak ◽  
Piotr Celichowski ◽  
Bartosz Kempisty ◽  
Katarzyna Iwanik ◽  
Michał Nowicki

Sjögren’s syndrome (SS) is characterized by xerostomia. We aimed to investigate and compare gene expressions in the labial salivary glands of SS patients with xerostomia SS (sicca) and without xerostomia SS (non-sicca) and of healthy subjects (HS) by means of microarray analysis, and to find genes involved in xerostomia. The study group comprised 11 SS patients (3 SS (sicca) and 8 SS (non-sicca)) and 9 HS. The relative gene expression changes were validated with RT-qPCR in the larger study group. Among the differently expressed genes belonging to the “secretion” ontology group with a fold change >2 and with a p value < 0.05, the Transmembrane P24 Trafficking Protein 10 (TMED10), Protein Disulfide Isomerase Family A Member 4 (PDIA4), Calnexin (CANX), Amyloid Beta Precursor Protein (APP), and Transmembrane BAX Inhibitor Motif Containing 6 (TMBIM6) gene expressions in both SS (sicca) and SS (non-sicca) groups were lower than in HS. Significant correlations were observed between TMED10, PDIA4, and CANX gene expression in SS (sicca) patients compared to the controls. There were no differences between the SS (sicca) and SS (non-sicca) study groups in the expression of the aforementioned genes. Results indicate their role in the endoplasmic reticulum system, their overlapping function and the loss of the APP neuroprotective function in xerostomia. It has a multifactorial origin and can be triggered by disturbances to the various signaling pathways in saliva secretion.


Rheumatology ◽  
2020 ◽  
Vol 59 (Supplement_2) ◽  
Author(s):  
Rosie Close ◽  
Aishwarya Lant ◽  
Eve Smith ◽  
Catriona M Anderson ◽  
Samundeeswari Deepak ◽  
...  

Abstract Background Sjögren's syndrome is an inflammatory disorder involving the exocrine glands, characterised by dry eyes and mucosal surfaces, fatigue and arthralgia. Sjögren's may occur in isolation (primary) or secondary to another autoimmune condition and is considered rare in the paediatric population (incidence and prevalence unknown). Anecdotal reports of differences in clinical presentation and absence of validated paediatric classification criteria render reliable diagnosis challenging and optimal treatment pathways difficult to ascertain. The American-European Consensus Group (AECG) criteria are considered gold standard for diagnosis of primary Sjogren’s syndrome in adults. A 2017 BSR Sjögren's guideline recommends treating children according to the adult pathway. We report a multicentre service evaluation exploring the presentation and management of children with Sjögren's presenting to UK paediatric rheumatology services, with reference to existing adult criteria. Methods Retrospective study conducted using a standardised data collection proforma under local service evaluation permissions. Inclusion criteria: pre-existing diagnosis of either primary or secondary Sjögren's syndrome under 16 years of age. Each case was assessed against the current adult criteria and guidance (AECG and BSR). Results 20 patients (16 female) in eight UK centres with a mean age at diagnosis of 11 (range 2-16 years). 15 patients had primary Sjögren's, 3 secondary Sjögren's and 2 were not categorised. No primary Sjögren's patients fulfilled AECG diagnostic criteria. Dry eye symptoms were present in 13%, dry mouth symptoms in 53% and 14/15 patients were tested for anti Ro/La (100% anti-Ro positive, 43% anti-La positive). Objective ocular dryness (Schirmers or van Bijsterveld positive), objective oral dryness and labial gland focus score were not formally assessed in any patients. No primary Sjögren's patients fulfilled ACR EULAR diagnostic criteria and one patient fitted exclusion criteria. The most common primary Sjögren's symptoms included parotid pain or swelling (73%), dry mouth (53%), arthralgia (47%) and fatigue (40%). Rash and headaches were also described whereas fever and lymphadenopathy were not reported. Sicca symptoms were reported in 100% secondary Sjogren’s patients (66% had parotid pain or swelling.) Management of all patients was assessed against 2017 BSR guidelines. There was documented evidence in 75% of receiving education, diet and lifestyle advice. 50% were prescribed moisturising eye drops. Hydroxychloroquine treatment was considered in 75%; of these, 4% were not referred for annual eye screening. Conclusion Sjögren's syndrome is rare in children and specific paediatric guidance does not exist. Despite extensive investigations no primary Sjögren's patients fulfilled the AECG or ACR EULAR diagnostic criteria due to difficulty in obtaining objective evidence of oral or ocular dryness. These data suggest that primary paediatric presentations may differ from adult cohorts with frequent parotid pain or swelling and less self-reporting of sicca symptoms. Further work is needed to support development of paediatric specific guidelines for Sjögren's syndrome. Disclosures R. Close None. A. Lant None. E. Smith None. C.M. Anderson None. S. Deepak None. R. Foster None. K. Haslam None. L. Bray None. P. Dawson None. C. Bhat None. F. McErlane None.


1993 ◽  
Vol 36 (3) ◽  
pp. 340-347 ◽  
Author(s):  
Claudio Vitali ◽  
Stefano Bombardieri ◽  
Haralampos M. Moutsopoulos ◽  
Genesio Balestrieri ◽  
Walter Bencivelli ◽  
...  

2012 ◽  
Vol 65 (1) ◽  
pp. 216-225 ◽  
Author(s):  
Divi Cornec ◽  
Sandrine Jousse-Joulin ◽  
Jacques-Olivier Pers ◽  
Thierry Marhadour ◽  
Béatrice Cochener ◽  
...  

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