Bidirectional Relationship Between Primary Sjögren Syndrome and Non-Hodgkin Lymphoma: A Nationwide Taiwanese Population-based Study

2020 ◽  
Vol 47 (9) ◽  
pp. 1374-1378
Author(s):  
Li-Hui Wang ◽  
Wei-Ming Wang ◽  
Chun-Yu Lin ◽  
Sheng-Hsiang Lin ◽  
Chi-Chang Shieh

Objective.Bidirectional relationships between some autoimmune diseases and non-Hodgkin lymphoma (NHL) may exist. We conducted this nationwide population-based study in Taiwan to investigate whether there is a bidirectional relationship between primary Sjögren syndrome (pSS) and NHL.Methods.Using the National Health Insurance Research Database of Taiwan, we identified 15,636 patients with new-onset pSS without previous cancer and 25,074 patients with new-onset NHL without previous pSS as 2 non-overlapping cohorts from 1998 to 2012, and followed them until 2013. Standardized incidence ratios (SIR) for NHL in the patients with pSS and SIR for pSS in the patients with NHL were compared with the general population.Results.Among the 15,636 patients with pSS, 741 developed cancers, including 51 with NHL. The highest SIR of specific cancer risk in patients with pSS was that for NHL (SIR 4.6, 95% CI 3.4–6.0). Among the 25,074 patients with NHL, 49 developed pSS; the SIR was also increased (SIR 3.2, 95% CI 2.4–4.2). The risk was highest within 1 year after the diagnosis of each disease.Conclusion.This nationwide population-based study is the first to report a bidirectional relationship between pSS and NHL. Our findings suggest being alert for patients with pSS or NHL who have early signs of the other disease in clinical care. The underlying mechanisms of the bidirectional relationship merit further investigation.

2020 ◽  
pp. jrheum.201352
Author(s):  
Yun-Tzu Liang ◽  
Pui-Ying Leong ◽  
James Cheng-Chung Wei

We have read with great interest in the article by Wang, et al on the higher incidence of non-Hodgkin lymphoma (NHL) in patients with primary Sjögren syndrome (pSS) and the higher incidence of pSS in patients with NHL1. Thank you for the discovery of the bilateral relationship between pSS and NHL in this nationwide population-based study1.


2021 ◽  
pp. jrheum.201537
Author(s):  
Yu-Wen Chen ◽  
Po-Yu Tsai ◽  
James Cheng-Chung Wei

We read with interest the research article entitled, “Bidirectional Relationship Between Primary Sjögren Syndrome and Non-Hodgkin Lymphoma: A Nationwide Population-based Study” by Wang, et al published in The Journal of Rheumatology.1 The authors conducted research revealing that primary Sjögren syndrome (SS) and non-Hodgkin lymphoma (NHL) are bidirectionally associated with each other.


2016 ◽  
Vol 25 (2) ◽  
pp. 95-99
Author(s):  
Claudia Deaconu ◽  
◽  
Ruxandra Ionescu ◽  
Daniela Opris ◽  
◽  
...  

Sjögren syndrome (SS) is a chronic autoimmune condition that mainly targets exocrine glands where it produces lymphocytic infiltrates. The clinical display varies in severity and can include malignancy association such as non-Hodgkin lymphoma. Its incidence ranges from 5 to 8%, while the risk of occurrence is 16 to 44 times higher. This article aims to describe the evolution of a patient with primary SS who developed pulmonary lymphoma for which she received Rituximab as part of the oncological therapeutic scheme. During the course of the disease she developed membranous glomerulonephritis, rarely described in these patients and low complement levels cited as predictive factor for lymphoproliferation.


2019 ◽  
Vol 46 (7) ◽  
pp. 727-730 ◽  
Author(s):  
Luisa Servioli ◽  
Gabriel Maciel ◽  
Carlotta Nannini ◽  
Cynthia S. Crowson ◽  
Eric L. Matteson ◽  
...  

Objective.To explore the role of smoking and obesity in primary Sjögren syndrome (pSS).Methods.Olmsted County (Minnesota, USA) residents (n = 106) diagnosed with pSS from 2000 to 2015 were compared to 3 controls without pSS and matched for age and sex who were randomly selected from Olmsted County residents.Results.Current smokers were less likely to be pSS cases (OR 0.34, 95% CI 0.14–0.85), while there was no association between former smoking and case/control status (OR 1.27, 95% CI 0.80–2.03) compared to never smokers. Smoking status was not associated with antinuclear antibody, anti-SSA, anti-SSB, or rheumatoid factor positivity (p > 0.05). OR for obesity was 0.79 (95% CI 0.48–1.30).Conclusion.In this population-based study, current smoking was inversely associated with case/control status, while body mass index lacked any association.


Medicine ◽  
2012 ◽  
Vol 91 (1) ◽  
pp. 1-9 ◽  
Author(s):  
Michael Voulgarelis ◽  
Panayiotis D. Ziakas ◽  
Aristea Papageorgiou ◽  
Evangelia Baimpa ◽  
Athanasios G. Tzioufas ◽  
...  

2021 ◽  
Vol 16 (1) ◽  
Author(s):  
Christina Bergqvist ◽  
François Hemery ◽  
Arnaud Jannic ◽  
Salah Ferkal ◽  
Pierre Wolkenstein

AbstractNeurofibromatosis 1 (NF1) is an inherited, autosomal-dominant, tumor predisposition syndrome with a birth incidence as high as 1:2000. A patient with NF1 is four to five times more likely to develop a malignancy as compared to the general population. The number of epidemiologic studies on lymphoproliferative malignancies in patients with NF1 is limited. The aim of this study was to determine the incidence rate of lymphoproliferative malignancies (lymphoma and leukemia) in NF1 patients followed in our referral center for neurofibromatoses. We used the Informatics for Integrated Biology and the Bedside (i2b2) platform to extract information from the hospital’s electronic health records. We performed a keyword search on clinical notes generated between Jan/01/2014 and May/11/2020 for patients aged 18 years or older. A total of 1507 patients with confirmed NF1 patients aged 18 years and above were identified (mean age 39.2 years; 57% women). The total number of person-years in follow-up was 57,736 (men, 24,327 years; women, 33,409 years). Mean length of follow-up was 38.3 years (median, 36 years). A total of 13 patients had a medical history of either lymphoma or leukemia, yielding an overall incidence rate of 22.5 per 100,000 (0.000225, 95% confidence interval (CI) 0.000223–0.000227). This incidence is similar to that of the general population in France (standardized incidence ratio 1.07, 95% CI 0.60–1.79). Four patients had a medical history leukemia and 9 patients had a medical history of lymphoma of which 7 had non-Hodgkin lymphoma, and 2 had Hodgkin lymphoma. Our results show that adults with NF1 do not have an increased tendency to develop lymphoproliferative malignancies, in contrast to the general increased risk of malignancy. While our results are consistent with the recent population-based study in Finland, they are in contrast with the larger population-based study in England whereby NF1 individuals were found to be 3 times more likely to develop both non-Hodgkin lymphoma and lymphocytic leukemia. Large-scale epidemiological studies based on nationwide data sets are thus needed to confirm our findings.


2021 ◽  
Vol 11 (1) ◽  
Author(s):  
Pei-I Kuo ◽  
Tzu-Min Lin ◽  
Yu-Sheng Chang ◽  
Tsung-Yun Hou ◽  
Hui-Ching Hsu ◽  
...  

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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