Objective We studied the risk of dementia in patients with primary Sjögren's syndrome ( pSS ) using a nationwide, population‐based cohort in Taiwan. Methods Our study analyzed the medical data of the Taiwanese population from 2000 to 2014. We identified 17,072 patients with pSS and 68,270 controls. Dementia risk was analyzed using a Cox proportional hazards regression model stratified by sex, age, and comorbidities. Results A higher incidence of dementia development in the pSS group during the observation period ( P = 0.0001). In multivariate analysis adjusted by age groups, gender, and the comorbidities, the adjusted hazard ratio ( aHR ) of developing dementia was 1.246 (95% CI 1.123–1.384) times greater in the pSS group than in the non‐ pSS group. When stratified by sex, age, and comorbidities, the patients with pSS less than 60 years ( aHR 1.67, 95% CI 1.16–2.41), and without any comorbidity ( aHR 2.27, 95% CI 1.76–2.93) were particularly associated with a higher risk of dementia. Furthermore, the patients with pSS combined with any other comorbidity had an additionally higher risk of dementia ( aHR : 3.978, 95% CI 3.309–4.782), also suggesting that pSS was an independent risk factor for the development of dementia. Interpretation Primary Sjogren's syndrome is associated with increased dementia risk and further study is needed to understand why and what the specific dementia phenotypes are.

Objective To compare Pneumocystis jirovecii pneumonia (PJP) risk between patients with autoimmune rheumatic diseases (ARD) and the general population Methods We identified patients with ARD recorded in the National Health Insurance Research Database of Taiwan from 2002 to 2015 and randomly selected a comparison cohort from the general population matched for age and sex. We analyzed PJP risk stratified by sex, age, comorbidities, and medications using Cox proportional hazard model. Results We enrolled 103,117 patients with ARD. PJP risk significantly increased in patients with any ARD and with each individual ARD like rheumatoid arthritis (RA), systemic lupus erythematosus (SLE), Sjogren’s syndrome (SjS), polymyositis and dermatomyositis (PM/DM), systemic sclerosis (SSc), and systemic vasculitis. Patients with PM/DM showed prominent risk with incidence rate of 12.47/100,000 patient year (95% confidence interval (CI), 32.16–86.70). In a time-dependent Cox proportional hazard model with comorbidities and medications as covariates, PM/DM, SSc, SLE, and SjS significantly increased adjusted hazard ratios (aHR) of 5.40, 5.12, 4.09, and 3.64, respectively (95% CI of 2.82–10.35, 2.16–12.13, 2.41–6.95, and 2.06–6.42, respectively). AHR after adjusting for male sex, cancer, human immunodeficiency virus infection (HIV), and interstitial lung disease also significantly increased. Use of daily oral steroid dose of >10 mg conferred the highest risk followed by mycophenolate. Use of injected steroids, cyclophosphamide, biological agents, methotrexate, and cyclosporine conferred a significantly higher risk. Conclusion Underlying ARD significantly predisposes patients to PJP, with PM/DM posing the highest threat. In addition to underlying disease, comorbidities and concomitant immunosuppressants are major risks. The strongest risk is recent daily steroid dose of >10 mg. Mycophenolate seems to be a more prominent risk factor than cyclophosphamide. Key Points • Autoimmune rheumatic diseases (ARD) significantly increased the overall risk of PJP, and so did each individual ARD. • Use of steroids, mycophenolate, cyclophosphamide, biological agents, methotrexate, and cyclosporine all significantly increased risk of PJP. • Male, elderly, malignancy, HIV, and interstitial lung disease are also related to increased risk of PJP. • Underlying ARD, comorbidities, and use of immunosuppressant should all be considered in determining the overall risk of PJP.

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Objectives: The data concerning the association between Tx and ADs remain unclear and are scarce. This study was undertaken to investigate whether people with Tx are more likely to develop ADs, compared to those without Tx. Methods: Individuals who received Tx between 2002 and 2015 were identified and matched on age and sex with individuals without Tx. We performed multivariate and stratified analysis using the Kaplan-Meier method and Cox proportional hazards models in order to estimate the association between Tx and the risk of developing ADs. Results: A total of 2550 thymectomized (Txd) patients and 24,664.941 non-Txd comparison subjects were selected from NHIRD. Tx-MG (myasthenia gravis) as compared with general population (nonTx-nonMG), adjusted hazard ratio (aHR) were higher for incident Addison disease (aHR = 10.40, 95% CI 1.01-107), autoimmune hemolytic anemia (aHR = 21.54, 95% CI 2.06-14.8), Hashmoto thyroiditis (aHR = 5.52, 95% CI 1.34-34.7), ankylosing spondylitis (aHR = 2.73, 95% CI 1.09-6.84), rheumatoid arthritis (aHR = 5.25, 95% CI 1.79-15.47), primary Sjogren syndrome (pSS) (aHR = 3.77, 95% CI 1.30-11.0), and systemic lupus erythemtoasus (aHR = 10.40). Tx-nonMG as compared with general population, aHR were higher for incident autoimmune hemolytic anemia (aHR = 25.50), Hashmoto thyroiditis (aHR = 6.75) and systemic lupus erythematosus (SLE) (aHR = 13.38). NonTx-MG as compared with general population, aHR were higher for incident Hashmoto thyroiditis (aHR = 6.57), pSS (aHR = 4.50), SLE (aHR = 17.29), and systemic vasculitis (aHR = 25.86). Interpretation: In conclusion, based on a retrospective cohort study throughout Taiwan, patients with Tx have a higher risk of new onset ADs than patients without Tx.