Plummer-Vinson syndrome in primary Sjögren syndrome: a case-based review

Abstract Background Abdominal symptoms in patients with primary Sjögren syndrome (pSS) are poorly documented. The objective of the study was to describe the abdominal symptoms of patients with pSS and to assess their association with characteristics of the disease. Methods One hundred and fifty patients with pSS were evaluated using a composite global symptom score for abdominal symptoms and their severity. Data concerning the clinical and biological characteristics of pSS and abdominal disorders were also collected. Results Of the patients with pSS, 95% suffered from abdominal symptoms (median global symptom score 7.5 ± 5.5 points out of 30). More than half of the patients experienced abdominal tension (68%), upper abdominal pain (54%), abdominal discomfort (58%) and/or constipation (54%). Regarding the pSS activity, in relation to European League Against Rheumatism (EULAR) Sjögren syndrome disease activity index score items, general and central nervous system involvement wereassociated with a high global symptom score. The EULAR Sjogren Syndrome Patient Reported Index (ESSPRI) symptom score was positively correlated with the global symptom score (p < 0.01). Multivariate analysis showed a significant association between a high global symptom score and SSA seronegativity, gastroparesis, and ESSPRI score (p < 0.01 for each). Conclusions The majority of patients with pSS suffered abdominal symptoms. There is currently no therapeutic recommendation because of the lack of information on the underlying pathophysiological mechanisms. Trial registration NCT03157011. Date of registration: July 17, 2017.

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Peripheral Neuropathy in Primary Sjögren Syndrome

Archives of Neurology ◽

10.1001/archneur.63.11.1612 ◽

2006 ◽

Vol 63 (11) ◽

pp. 1612 ◽

Cited By ~ 87

Author(s):

Lasse G. Gøransson ◽

Anita Herigstad ◽

Anne B. Tjensvoll ◽

Erna Harboe ◽

Svein I. Mellgren ◽

...

Keyword(s):

Peripheral Neuropathy ◽

Sjögren Syndrome ◽

Sjogren Syndrome ◽

Primary Sjögren Syndrome

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Primary Sjogren syndrome increases the risk of bisphosphonate-related osteonecrosis of the jaw

Scientific Reports ◽

10.1038/s41598-020-80622-5 ◽

2021 ◽

Vol 11 (1) ◽

Author(s):

Pei-I Kuo ◽

Tzu-Min Lin ◽

Yu-Sheng Chang ◽

Tsung-Yun Hou ◽

Hui-Ching Hsu ◽

...

Keyword(s):

Propensity Score ◽

Osteonecrosis Of The Jaw ◽

Sjögren Syndrome ◽

Cox Proportional Hazards Model ◽

Matched Pair ◽

Sjogren Syndrome ◽

Research Database ◽

Primary Sjögren Syndrome ◽

Defined Daily Doses ◽

Increased Risk

AbstractThe risk of bisphosphonate-related osteonecrosis of the jaw (BRONJ) in primary Sjogren syndrome (pSS) has rarely been explored. To explore the association between BRONJ and pSS, we conducted a population-based propensity-score-matched cohort study using Taiwan’s National Health Insurance Research Database, including pSS patients receiving antiosteoporotic therapy and patients without pSS receiving antiosteoporotic therapy. A 1:4 matched-pair cohort based on propensity score was created. The stratified Cox proportional hazards model compared the risk of BRONJ in the pSS and non-pSS groups. In the study, 23,280 pSS patients and 28,712,152 controls were enrolled. After matching, 348 patients with pSS receiving antiosteoporotic drugs and 50,145 without pSS receiving antiosteoporotic drugs were included for analysis. The risk of developing BRONJ was 1.96 times higher in pSS patients compared with non-pSS patients after adjustment for age, sex, and comorbidities. No dose–response effect was observed in the bisphosphonate-treated pSS cohorts, documented as the cumulative defined daily doses of either < 224 or ≥ 224 (hazard ratio [HR]: 2.407, 95% confidence interval [CI] 1.412–7.790; HR: 2.143, 95% CI 1.046–4.393, respectively) increased risk of developing osteonecrosis of the jaw. In conclusion, the risk of BRONJ is significantly higher in patients with pSS compared with the general population.

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