LATEST SJOGREN'S NEWS

Complete Hypokalemic Quadriparesis as a First Presentation of Sjögren Syndrome, was published in the Canadian Journal of Kidney Health and Disease.​​​​​​​


Researchers discuss the case of a 73-year-old woman who came to the hospital with extreme muscle weakness in all four limbs.

Her blood tests suggested this was caused by critically low levels of potassium. But potassium supplements failed to raise her potassium levels to normal range.

Her only other symptoms were fatigue and ocular dryness.

“Our report emphasizes that although Sjögren syndrome is most often associated with chronic sicca [dryness] symptoms, it may present for the first time with extraglandular manifestations which may be life threatening,” the researchers concluded.
 

Researchers investigated peripheral TFH cells in primary Sjogren's Syndrome (pSS.) 
B cells have a prominent role in SS, and the T follicular helper (TFH) cells provide B cells with survival.  
B-cells are a type of white blood cell that are key to the development of pSS. TFH cells are crucial for the survival, development, and function of B-cells.

Some findings were that disease activity in pSS patients caused higher TFH cells and lower B-cells, and increased activation of B-cells.​​​​​​​

This means that people with pSS show altered levels of specific types of immune T-cells and B-cells, which may play an important role in disease activity.
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Patients with chronic periodontitis present increased risk for primary Sjögren syndrome: a nationwide population-based cohort study.  The findings revealed a significant association between CP exposure and pSS risk. CP is Chronic periodontitis - the most common periodontal disease which is related to the chronic accumulation of bacterial biofilm leading to host-mediated slowly progressive destruction of periodontium. CP is the primary risk factor for tooth loss in adults 

Meibomian gland dropout in Sjögren’s syndrome and non-Sjögren’s dry eye patients. The purpose of this study is to explore the differences in ocular symptoms and signs between Sjögren’s syndrome (SS) and non-SS aqueous-deficient dry eye (ADDE) patients.​​​​​​​

Conclusion

SS patients exhibit more severe meibomian gland destruction of the upper eyelid than non-SS patients. Meibomian gland dysfunction is another key cause of SS-associated dry eye.


​​​​​​​Cerebellar degeneration in primary Sjogren syndrome,  the investigators concluded that in rare cases, patients with primary Sjogren’s syndrome can have manifestations of cerebellar atrophy, a neurological manifestation of SS. 
Meibomian gland dropout in Sjögren’s syndrome and non-Sjögren’s dry eye patients. The purpose of this study is to explore the differences in ocular symptoms and signs between Sjögren’s syndrome (SS) and non-SS aqueous-deficient dry eye (ADDE) patients.​​​​​​​

Conclusion

SS patients exhibit more severe meibomian gland destruction of the upper eyelid than non-SS patients. Meibomian gland dysfunction is another key cause of SS-associated dry eye.


​​​​​​Cerebellar degeneration in primary Sjogren syndrome,  the investigators concluded that in rare cases, patients with primary Sjogren’s syndrome can have manifestations of cerebellar atrophy, a neurological manifestation of SS. 

Mixed‐Methods Study Identifying Key Intervention Targets to Improve Participation in Daily Living Activities in Primary Sjögren's Syndrome Patients

This data highlighted the fact that in addition to managing primary SS symptoms, interventions aiming to improve patient empowerment, general wellbeing, access to health care, patient education, and social support are important to facilitate improved participation in daily living activities. SOURCE
Genetic Determinants of Fatigue in Primary Sjögren`s Syndrome – a Genome Wide Association Study

Background/Purpose:

Fatigue is common in primary Sjögren`s syndrome (pSS), but what leads to that fatigue is not fully understood. They hypothesized that there is a genetic basis for fatigue, and that specific gene-variants influence the severity of fatigue.

To investigate this further they performed a genome wide association study of 367 Scandinavian pSS patients. 

They identified genetic variants in RTP4 exceeding the GWS level for association with fatigue. This gene encodes a protein involved in pain processing. Pain is known to influence fatigue, and this finding could point to a possible molecular explanation. ​​​​​​​

The present study is the largest GWAS of fatigue in autoimmune disease, and adds further evidence to a genetic regulation of fatigue.

Read Full Article here.

Significant improvement in Salivary Gland Ultrasound Scores after treatment with rituximab 

In patients with primary Sjogren's syndrome, improvements in salivary gland ultrasound were demonstrated after treatment with rituximab, according to the results of the TRACTISS randomised double-blind multicentre substudy published in the Annals of the Rheumatic Diseases in 2018.

A total of 52 patients (26 treated with rituximab and 26 treated with placebo) from 9 centers completed the baseline visit and 1 or more follow-up visits.

The research showed statistically significant improvement in total ultrasound score (TUS) after rituximab compared with the placebo. 

Primary Sjögren's syndrome (pSS) impact on smell, taste, sexuality and quality of life in female patients: A systematic review and meta-analysis.
378 female participants were included in the study which was reported in Modern Rheumatology in July 2017.
pSS had a negative impact on smell, taste, sexual function and quality of life in the women. The most impact was considered to be on the physical and mental component of the quality of life.  

 rituximab with a peptide epitope