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Objective To characterize the systemic phenotype of primary Sjögren’s syndrome at diagnosis by analysing the EULAR-SS disease activity index (ESSDAI) scores. Methods The Sjögren Big Data Consortium is an international, multicentre registry based on worldwide data-sharing cooperative merging of pre-existing databases from leading centres in clinical research in Sjögren’s syndrome from the five continents. Results The cohort included 10 007 patients (9352 female, mean 53 years) with recorded ESSDAI scores available. At diagnosis, the mean total ESSDAI score was 6.1; 81.8% of patients had systemic activity (ESSDAI score ≥1). Males had a higher mean ESSDAI (8.1 vs 6.0, P 65 years, P Conclusion The systemic phenotype of primary Sjögren’s syndrome is strongly influenced by personal determinants such as age, gender, ethnicity and place of residence, which are key geoepidemiological players in driving the expression of systemic disease at diagnosis.

Background There is a lack of information on the role of chronic use of hydroxychloroquine during the SARS-CoV-2 outbreak. Our aim was to compare the occurrence of COVID-19 between rheumatic disease patients on hydroxychloroquine with individuals from the same household not taking the drug during the first 8 weeks of community viral transmission in Brazil. Methods This baseline cross-sectional analysis is part of a 24-week observational multi-center study involving 22 Brazilian academic outpatient centers. All information regarding COVID-19 symptoms, epidemiological, clinical, and demographic data were recorded on a specific web-based platform using telephone calls from physicians and medical students. COVID-19 was defined according to the Brazilian Ministry of Health (BMH) criteria. Mann–Whitney, Chi-square and Exact Fisher tests were used for statistical analysis and two binary Final Logistic Regression Model by Wald test were developed using a backward-stepwise method for the presence of COVID-19. Results From March 29th to May 17st, 2020, a total of 10,443 participants were enrolled, including 5166 (53.9%) rheumatic disease patients, of whom 82.5% had systemic erythematosus lupus, 7.8% rheumatoid arthritis, 3.7% Sjögren’s syndrome and 0.8% systemic sclerosis. In total, 1822 (19.1%) participants reported flu symptoms within the 30 days prior to enrollment, of which 3.1% fulfilled the BMH criteria, but with no significant difference between rheumatic disease patients (4.03%) and controls (3.25%). After adjustments for multiple confounders, the main risk factor significantly associated with a COVID-19 diagnosis was lung disease (OR 1.63; 95% CI 1.03–2.58); and for rheumatic disease patients were diagnosis of systemic sclerosis (OR 2.8; 95% CI 1.19–6.63) and glucocorticoids above 10 mg/ day (OR 2.05; 95% CI 1.31–3.19). In addition, a recent influenza vaccination had a protective effect (OR 0.674; 95% CI 0.46–0.98). Conclusion Patients with rheumatic disease on hydroxychloroquine presented a similar occurrence of COVID-19 to household cohabitants, suggesting a lack of any protective role against SARS-CoV-2 infection. Trial registration Brazilian Registry of Clinical Trials (ReBEC; RBR – 9KTWX6).

International audience; Objective. Juvenile Sjögren's syndrome (SS) is a rare, poorly defined, and possibly underdiagnosed condition affecting children and adolescents. The aim of this study was to characterize symptoms and clinical findings of juvenile SS and to explore the clinical application of major salivary gland ultrasonography (SGUS) in patients with juvenile SS. Methods. A cross-sectional multicenter study recruited patients with disease onset until age 18 years (n = 67). Disease characteristics were recorded, and unstimulated whole sialometry and SGUS examination of the parotid and submandibular salivary glands were performed. Results. The female:male ratio was 58:9. The mean age at first symptom was 10.2 years and 12.1 years at diagnosis. Ocular and oral symptoms were noted in 42 of 67 patients (63%) and 53 of 66 patients (80%), respectively. The American-European Consensus Group or American College of Rheumatology/European League Against Rheumatism classification criteria for primary SS were fulfilled by 42 of 67 patients (63%). Pathologic SGUS findings were observed in 41 of 67 patients (61%); 26 of 41 SGUS+ patients (63%) fulfilled primary SS criteria. Salivary gland enlargements/parotitis were noted in 37 of 58 patients and were nonsignificantly associated with SGUS+ status (P = 0.066). The mean levels of saliva were 5.6 ml/15 minutes in SGUS-patients compared to 3.3 ml/15 minutes in the SGUS+ patients (P = 0.049). A total of 36 of 41 SGUS+ patients (88%) were anti-Ro/La+ compared to 14 of 26 SGUS-patients (54%) (P = 0.001). In addition, 24 of 39 SGUS+ patients (62%) were positive for rheumatoid factor (RF), whereas only 5 of 25 SGUS-patients (20%) were RF+ (P = 0.001). Conclusion. Juvenile SS is characterized by a large spectrum of clinical symptoms and findings. Several glandular and extraglandular parameters such as hyposalivation, swollen salivary glands, and autoantibodies are associated with pathologic SGUS findings.

Primary Sjogren's syndrome (pSS) patients identify fatigue as their most important symptom and the one most difficult to cope with, but there are still many challenges and few solutions to manage this debilitating symptom. Promising pharmacological treatments, such as rituximab, have failed in more stringent tests including randomized controlled trials (RCTs) and meta-analysis. While non-pharmacological interventions may be safer, less costly, and address other common comorbidities, to date only aerobic exercise seems to be effective at reducing fatigue in pSS. All interventions, pharmacological or not, need to be tested in high-quality RCTs. The aim of this review is to provide an overview of fatigue management in pSS and discuss potential opportunities for future research.

Objective To assess the prevalence of parotid gland swelling (PGS) and its association with features of SS and other causes of sialadenosis in a Latin-American cohort of primary SS. Methods We included 668 patients from Argentina, Brazil, Mexico and Paraguay. We retrospectively registered demographics, disease duration, oral/ocular symptoms, serology and scored the basal ESSDAI. We defined PGS as a recurrent or persistent increase of volume of any parotid glands during adulthood (self-reported and/or physical examination). We registered the presence of diabetes mellitus, dyslipidaemia, body mass index and alcohol consumption. We used logistic regression analysis reporting odds ratio (OR) and 95% CI. Results PGS was present in 242 patients (36.2%): 78 previous to SS diagnosis, 86 concomitantly, 73 during follow-up and five unknown. At the multivariate analysis, PGS was associated with RF (OR 2.47, 95% CI: 1.1, 6.5, P = 0.0001), basal articular ESSDAI domain (OR 1.63, 95% CI: 1.01, 2.6, P = 0.04) and alcohol consumption (OR 2.42, 95% CI: 1.41, 4-15). Patients with PGS during the follow-up had a higher prevalence of alcohol consumption (45.3%) compared with the remaining PGS cases (26.8%; OR 2.41 95% CI: 1.2, 4.7), or patients without parotid gland swelling (15.6%; OR 3.8 95% CI: 1.7, 8.2) in all the adjusted models. Conclusion PGS generally precedes or presents concomitantly with SS diagnosis, and is related to RF and articular activity. Alcohol consumption is an additional factor in PGS, especially during follow-up. The meaning of this last finding as well as its prognostic implications remains to be elucidated and deserves further evaluation in prospective studies.

Sjögren’s syndrome (SS) is an autoimmune disease affecting the salivary and lacrimal glands. Symptoms range from dryness to severe extra-glandular disease involving manifestations in the skin, lungs, nervous system, and kidney. Fatigue occurs in 70% of patients, characterizing primary SS (pSS) and significantly impacting the patient’s quality of life. There are some generic and specific instruments used to measure fatigue in SS. The mechanisms involved with fatigue in SS are still poorly understood, but it appears fatigue signaling pathways are more associated with cell protection and defense than with pro-inflammatory pathways. There are no established pharmacological treatment options for fatigue in pSS. So far, exercise and neuromodulation techniques have shown positive effects on fatigue in pSS. This study briefly reviews fatigue in pSS, with special attention to outcome measures, biomarkers, and possible treatment options.

Health-related quality of life (HRQoL) has an increasing role in medical decision-making. This review of the literature aims to provide an overview on HRQoL, costs, and work disability in SS, a disease characterized by focal lymphocytic infiltration of exocrine glands with no therapeutics of proven immunomodulatory potential. HRQoL is markedly reduced in SS in multiple studies across many countries when compared with HRQoL in healthy controls. The reduction in HRQoL is similar to that observed in other chronic diseases such as RA, SLE, FM and, interestingly, non-SS sicca syndrome. Impaired HRQoL in SS has been found to be associated with fatigue, pain/articular involvement, ocular and oral involvement, pruritus, sexual dysfunction, impaired sleep, pulmonary manifestations, psychological dysfunction and impaired physical function. Until now, no therapeutic has been shown to improve HRQoL in an adequately powered double-blind, placebo-controlled randomized controlled trial. Although primary SS does not, in general, impair life expectancy and is often inappropriately considered a benign ‘nuisanvce’ disease for those patients without systemic manifestations, the associated costs and work disability are striking. This, together with the significant reduction in HRQoL, strongly argues for the development of new therapeutic approaches to manage this neglected disease.

BACKGROUND The safety profile of biologic drugs might present substantial regional differences. Since 2009, the Brazilian Society of Rheumatology has maintained BIOBADABRASIL (Brazilian Registry for Biologic Drugs), a registry for monitoring of biologic therapies in rheumatic diseases. OBJECTIVES The aim of this study was to verify the incidence rate (IR) of serious infections in rheumatoid arthritis (RA) and spondyloarthritis (SpA) patients on biologic drugs. METHODS BIOBADABRASIL prospectively included patients with rheumatic diseases who started the first biologic drug or a synthetic disease-modifying antirheumatic drug as a parallel control group. This study focuses on serious infectious adverse events (SIAEs) in RA and SpA patients on biologic drugs compared with controls, from January 2009 to June 2015. Time of exposure was set from initiation of the drug to the date of last administration or censorship. Serious infectious adverse events IR was calculated per 1000 patient/years with 95% confidence interval (CI). RESULTS A total of 1698 patients (RA, 1121; SpA, 577) were included, 7119 patient/years. Serious infectious adverse events were more common among patients on tumor necrosis factor inhibitors (TNFi's) than controls (adjusted IR ratio, 2.96 [95% CI, 2.01-4.36]; p < 0.001). Subsequent TNFi was associated with a higher SIAEs incidence when compared with first TNFI (adjusted IR ratio, 1.55 [95% CI, 1.15-2.08]; p = 0.004). Serious infectious adverse events were associated with age and corticosteroids intake. Serious infectious adverse events were more frequent in the respiratory tract in all subgroups. CONCLUSIONS In BIOBADABRASIL, biologic drugs, especially the subsequent TNFi, were associated with a higher risk of serious infections compared with synthetic DMARDs. Corticosteroid intake and age represented risk factors for SIAEs. Constant monitoring is required to follow the safety profile of drugs in the clinical setting of rheumatic conditions in Brazil.This is an open-access article distributed under the terms of the Creative Commons Attribution-Non Commercial-No Derivatives License 4.0 (CCBY-NC-ND), where it is permissible to download and share the work provided it is properly cited. The work cannot be changed in any way or used commercially without permission from the journal.

Paracoccidioidomycosis is an endemic disease in Latin America that is rarely associated with immunosuppression and biological therapy. Herein, we report for the first time a case of pulmonary paracoccidioidomycosis reactivation after infliximab treatment. A 47-year-old man from Brazil received infliximab to treat psoriatic spondyloarthropathy and presented with cough, dyspnea, weight loss, and fever. Chest computed tomography revealed a pulmonary nodule and biopsy confirmed paracoccidioidomycosis. Treatment with sulfamethoxazole and trimethoprim was initiated for fungal infection and infliximab was reintroduced two months later. Considering his clinical improvement and favorable radiologic evolution, antifungal therapy was discontinued after 29 months.

Objective.To evaluate the safety of the methotrexate (MTX)–leflunomide (LEF) combination in rheumatoid arthritis (RA), comparing it with other therapeutic schemes involving conventional synthetic (cs-) and biologic (b-) disease-modifying antirheumatic drugs (DMARDs) or Janus kinase inhibitors (JAKi).Methods.Patients with RA starting a treatment course with a csDMARD (without previous use of bDMARD or JAKi) or their first bDMARD/JAKi were followed up in a registry-based, multicentric cohort study in Brazil (BiobadaBrasil). The primary outcome was the incidence of serious adverse events (SAEs); secondary outcomes included serious infections. Multivariate Cox proportional hazards models and propensity score matching analysis (PSMA) were used for statistical comparisons.Results.In total, 1671 patients (5349 patient-years [PY]) were enrolled; 452 patients (1537 PY) received MTX + LEF. The overall incidence of SAEs was 5.6 per 100 PY. The hazard of SAEs for MTX + LEF was not higher than for MTX or LEF (adjusted HR [aHR] 1.00, 95% CI 0.76–1.31, P = 0.98). MTX + LEF presented a lower hazard of SAEs (aHR 0.56, 95% CI 0.36–0.88, P = 0.01) and infectious SAEs (aHR 0.48, 95% CI 0.25–0.94, P = 0.03) than bDMARDs/JAKi with MTX or LEF. MTX + LEF presented lower hazard of SAEs than MTX + sulfasalazine (SSZ; aHR 0.33, 95% CI 0.16–0.65, P = 0.002). Analysis using PSMA confirmed the results obtained with traditional multivariate Cox analysis.Conclusion.In our study, MTX + LEF presented a relatively good overall safety profile in comparison to MTX + SSZ and schemes involving advanced therapies in RA.

Background:The role of chronic use of hydroxychloroquine (HCQ) in rheumatic disease (RD) patients during the SARS-CoV-2 pandemic is still subject of discussion.Objectives:To compare the occurrence of COVID-19 and its outcomes between RD patients on HCQ use with individuals from the same household not taking the drug during community viral transmission in an observational prospective multicenter study in Brazil.Methods:Participants were enrolled and monitored through 24-week (From March 29th to Sep 30th, 2020) regularly scheduled phone calls performed by trained medical professionals. Epidemiological and demographic data, as well as RD disease activity status and current treatment data, specific information about COVID-19, hospitalization, need for intensive care, and death was recorded in both groups and stored in the Research Electronic Data Capture (REDCap) database. COVID-19 was defined according to the Brazilian Ministry of Health (BMH) criteria. The statistical analysis was performed using IBM-SPSS v.20.0 software. Group comparisons were made using the Man-Whitney, Chi-Square and Fisher Exact Test, as well as multivariate regression models adjusted to confounders. Survival curves were performed using Kaplan-Meier analysis.Results:A total of 10,427 participants mean age (SD) of 44.04 (14.98) years were enrolled, including 6004 (57.6%) rheumatic disease patients, of whom 70.8% had systemic lupus erythematosus (SLE), 6.7% rheumatoid arthritis (RA), 4% primary Sjögren’s syndrome (pSS), 1.8% mixed connective tissue disease (DMTC), 1% systemic sclerosis (SSc) and others (15.9), including overlap syndromes. In total, 1,132 (10.8%) participants fulfilled criteria for COVID-19, being 6.7% RD patients and 4.1% controls (p=0.002). A recent influenza vaccination had a protective role (p=65 years (OR=0.20; 95%CI 0.11-0.34) was negatively associated. Regarding RD, the risk factors associated with COVID-19 diagnosys were SLE (OR= 2.37; 95%CI 1.92-293), SSc (OR=2.25; 95%CI 1.05-4.83) and rituximab use (OR=1.92; 95%CI 1.13-3.26). In addition, age >=65 years (OR=5.47; 95%CI 1.7-19.4) and heart disease (OR=2.60; 95%CI 1.06-6.38) were associated with hospitalization. Seven female RD patients died, six with SLE and one with pSS, and the presence of two or more comorbidities were associated with higher mortality rate.Conclusion:Chronic HCQ use did not prevent COVID-19 in RD compared to their household cohabitants. Health care profession, presence of comorbidities LES, SSc and rituximab were identified as main risk factors for COVID-19 and aging and heart disease as higher risk for hospitalization. Our data suggest these outcomes could be considered to manage them in clinical practice.Table 1.Frequency and severity of COVID-19 in patients with rheumatic diseases on chronic use of hydroxychloroquine compared to their household controlsCOVID-19 outcomesTotal(%)GroupsPPatients(%)Controls (%)DiagnosisNo9256 (89.1)5300 (88.3)3956 (90.2)0.002Yes1132 (10.9)704 (11.7)428 (9.8)SeverityMild1059 (93.6)662 (94.0)397 (92.8)0.391Moderate52 (4.6)32 (4.5)20 (4.7)Severe21 (1.9)10 (1.4)11 (2.6)HCQ: hydroxychloroquine.Moderate and severe COVID-19 included the need for any of the following: hospitalization, intensive care, mechanical ventilation or death.Acknowledgements:To the Brazilian Society of Rheumatology for technical support and rapid nationwide mobilization.To all the 395 interviewers (medical students and physicians) who collaborated in the study and the participantsTo CNPq (Number 403442/2020-6)Disclosure of Interests:None declared

We aim to discuss the association of isolated atrophy of the abductor digiti quinti muscle in patients with rheumatoid arthritis as well as review the anatomy and imaging findings of this condition on MRI. A consecutive series of 55 patients diagnosed with rheumatoid arthritis according to the 2010 ACR/EULAR classification criteria were recruited. MRI of the clinically dominant feet was performed using a 1.5-T scanner. The study population was predominantly female (94.5%), and the age range was 31–79 years (mean 57.5 ± 11). A total of 55 ankles were examined by MRI, and 20 patients (36.3%), all females, showed abductor digiti quinti denervation signs. Seven patients demonstrated severe fatty atrophy of the abductor digiti quinti, corresponding to Goutallier grade 4, 2 patients showed moderate fatty atrophy (Goutallier grade 3), and the remaining 11 patients showed less than 50% fatty atrophy, corresponding to a Goutallier grade 2. Substantial agreement was found for both intra- and interobserver agreement regarding the Goutallier grading system. Prevalence of signs of abductor digiti quinti denervation on MRI was high in the studied population, suggesting that rheumatoid arthritis may be associated with inferior calcaneal nerve compression.

Background Primary Sjögren’s syndrome (pSS) is a systemic immune-mediated disease whose main characteristic is exocrine gland inflammation and, subsequent reduction in tear and saliva production. A delayed diagnosis is common due to the nonspecific clinical manifestations of disease. The aim of the present study was to develop recommendations for the diagnosis of glandular manifestations of pSS based on evidence and expert opinion. Main body of the abstract We conducted a systematic literature review to retrieve the best evidence available on the accuracy of diagnostic tests for pSS. We also held two in-person meetings with experts (rheumatologists, pathologists, ophthalmologists and dentists) to establish their level of agreement using the Delphi method. Ultimately, we generated 18 recommendations that aim to facilitate the diagnosis of the glandular manifestations of pSS. Conclusion The diagnosis of glandular manifestations of pSS is complex and multidisciplinary. It requires specific knowledge in the field of ophthalmology, immunology, pathology and imaging, making it compulsory for the rheumatologist to work with professionals from these different areas in order to improve accuracy and early diagnosis. Glandular dysfunction tests, ANA, RF, Anti-Ro, protein electrophoresis, urinalysis, blood count, C-Reactive protein, complement, testing for syphilis and viruses (HCV, HIV) and SGUS should be investigated when dryness or systemic manifestation are present. Minor salivary gland biopsy is recommended for all anti-Ro negative or incomplete criteria cases.

Sarcoidosis is a multisystem disease with unknown etiology, marked by T lymphocytes and macrophages agglomeration, which leads to the formation of noncaseating granulomas in the affected tissues. We describe a case of a 40-year-old black patient referred to our service for evaluation of nephrolithiasis and persistent elevation of plasma creatinine. He reported important weight loss, fever episodes, and abdominal and low back intermittent pain in the past 6 months. The investigation revealed elevated serum calcium level, hepatosplenomegaly, retroperitoneal lymphadenopathy, anemia, thrombocytopenia, and nephrolithiasis. The initial diagnostic hypothesis was lymphoproliferative disease, but the laparoscopic propaedeutic showed multiple white lesions on the liver surface, which biopsy identified as noncaseating granulomas with asteroid corpuscles, suggestive of sarcoidosis. He was treated with corticosteroids with significant improvement in symptoms and in calcium and creatinine levels. Besides, the patient presented a long-term large joints arthropathy, especially on the knees (with bilateral prosthesis), wrists, and ankles, of unknown etiology. We discuss the systemic manifestations of sarcoidosis related to the reported case, as well as the possible overlapping of idiopathic juvenile arthritis with sarcoidosis.

Background Primary Sjogren’s syndrome (pSS) is an orphan systemic autoimmune disease with no treatment based on evidence1. There is an international effort for multicentric registries for getting information about phenotypes, complication, response of treatment, and biobank consortium. Objectives To describe the creation of the Brazilian Sjogren’s Syndrome Registry (BRASS) and present the preliminary data. Methods BRASS is supported by Brazilian Society of Rheumatology (SBR) and is including patients from all regions of the country. Recruitment started in 2018 and is due to be completed in 2024. We are including patients with pSS according to AECG 2002 or ACR-EULAR 2016 classification criteria. All patients are being assessed for disease activity (ESSDAI), disease damage (SSDDI), symptoms assessment (ESSPRI), fatigue (FACIT-Fatigue), anxiety and depression (HADS), sleepiness (Epworth Sleepiness Scale), physical activity (IPAQ-SF) and quality of life (EQ-5D). In addition, demographics, immunological tests, unstimulated whole salivary flow (UWSF), salivary gland biopsy (SGB), comorbidities, treatment and complications such as cancer and cardiovascular risk assessment are being collected. Results There are currently 10 centers across the Brazil and 248 patients were evaluated until now. Most patients were female, white (45%) or mixed (38.3%), with the mean of the disease duration of 8 years. ESSDAI at baseline was 6.62±6.37 and currently is 4.21±5.16 (p Conclusion ESSDAI has decreased over time and more than half of patients are on hydroxychloroquine, immunosuppressant or biological therapy. Further analysis is needed to understand whether the reduction in ESSDAI reflects the natural course of the disease or greater access to treatment. BRASS Registry will be important to enhance research and to develop public health planning. References [1] Valim V et al. Recommendations for the treatment of Sjogren’s syndrome. Rev Bras Reumatol 55:446–57, 2015. Disclosure of Interests None declared

Background Inflammation has been associated with higher cardiovascular risk in rheumatic autoimmune diseases. Primary Sjogren’s syndrome (pSS) is associated with increased cardiovascular morbidity1. However, few and controversy data on the prevalence of cardiovascular events in patients with pSS have been reported2,3 with no data regarding on Latin-American pSS patients. Objectives To assess the cardiovascular event and to evaluate its association with traditional and disease associated risk factors in pSS. Methods We included 455 patients with pSS according to AECG 2002 or ACR-EULAR 2016 classification criteria, attending tertiary referral centers from Brazil (n=183), Mexico (n=236) and Argentina (n=36). We retrospectively registered demographics, disease duration, cardiovascular event (CE) (coronary heart disease, cerebrovascular accident, peripheral arterial disease), smoking, and use of prednisone (PDN), immunosupressors and antimalarials. We evaluated traditional risk factors and scored the ESSDAI, ESSPRI and SSDDI at last follow-up. Results Most patients were female, miscigenous ethnicity, with median disease duration of 9.5 years. RF was positive in 48.5%, ANA in 85.6%, anti-Ro-SSA in 78.9% and anti-La-SSA in 43.1%. ESSDAI mean was 3.2±4.4, SSDDI 2.54±1.78 and ESSPRI 5.03±2.38. Fifty five percent used glucocorticoid in the past and 20% are curruntly using it, 33.4% are using immunosupressors, 44% antimalarials and 10.3% biological therapy. It was found 26 (5.7%, CI95% 3.73-8.37) CE. The prevalence of hypertension was 35.6%, diabetes 11.2%, dyslipidemia 27.7% and smoking 17.5%. In univariate regression analysis, aging, hypertension, diabetes, dyslipidemia and smoking were associated with CE. Beyond traditional risk factors, prednisone > 20mg/day, ESSDAI constitutional domain and ESSPRI fatigue were associated with CE. In multivariate analysis, aging OR= 1.037 (1.001-1.075, p= 0.043), hypertension OR=2.509 (1.028-6.128, p=0.043) and diabetes OR= 2.736 (1.059-7.070, 0.038) were independent risk for CE. Conclusion Traditional risk factors are independent risk for CE in pSS. Disease-associated factors, such as glucocorticoid, fatigue and disease activity seem to be associated and should be explored in larger samples. References [1] Beltai A, et al. Cardiovascular morbidity and mortality in primary Sjogren syndrome: a systematic review and meta-analysis. Arthritis Care Res, 2018 (Epub ahead of print); [2] Valim V, et al. Atherosclerosis in Sjogren’s syndrome: evidence, possible mechanisms and knowledge gaps. Gerdts Clin Exp Rheumatol.34(1):133-42, 2016. [3] Berardicurti O, et al. Cardiovascular Disease in Primary Sjogren’s Syndrome. Rev Recent Clin Trial 13(3):164-169, 2018. Disclosure of Interests None declared

Background: As Brazil is accountable for 33% of the tuberculosis (TB) burden in Americas, with an annual incidence of 33.5/100,000 in 2017[1], the occurrence of tuberculosis infection in patients with rheumatic diseases in use of biological therapy is a recurrent concern. Objectives: To assess incident TB among patients with rheumatic disease in use of biological therapy in a country with high incidence of TB. Methods: BiobadaBrasil is a multicentric prospective cohort study involving patients with rheumatic diseases who started the first biologic or a synthetic disease modifying anti-rheumatic drug (DMARD)[6]. This analysis includes patients with rheumatoid arthritis (RA), psoriatic arthritis (PsA), and ankylosing spondylitis (AS) recruited from Jan 2009 to Aug 2018 and followed-up for one or multiple courses of treatment until censoring or incident TB. The primary outcome was the incidence of tuberculosis (in any organ site). The history of exposure to tuberculosis, chest Rx, screening and treatment for latent tuberculosis infection (LBTI), and use of prophylactic isoniazid were evaluated before each course of treatment. Multivariate Cox proportional hazards models (with DMARDs included as time-varying covariates) were used to estimate hazard ratios (HR) and 95% confidence intervals (CI); analyses were performed with the Survival package of R. Results: Sample: 2858 patients (female =70,2%; RA = 72%, AS=20%, and PsA = 8%). A total of 31 (1.1%) patients developed tuberculosis during treatment. One patient on abatacept and one on tocilizumab developed TB while all the others were on TNF-inhibitors(29). The median (interquartile range) exposure to the current DAMRD course was 11.1 (5.9-20) months. TB patients did not significantly differ from others regarding disease duration, sex or age. Almost half (n=14,45%) of TB patients did not present evidence of previous exposure or any screening positive test for TB; 9 (29%) TB patients had received adequate LTBI treatment (isoniazid) recently or in the past. Furthermore, 8(26%) had incomplete/inconsistent screening. The overall incidence of TB was 1.9/1000 patients/year, and was not significantly different among the diseases (1.63 for RA, 2.06, for AS, and 3.79/1000/year for PsA). In univariate analysis, exposure to anti-TNF monoclonal antibodies (HR 3.1, 95%CI 1.18-8.15, P=0.02) and presence of any marker of previous contact with TB (positive history of TB, known contact with TB, positive TST, or abnormal chest X-ray: HR 4.2 (2.1-8.5, p Conclusion: Monoclonal anti-TNF antibodies and previous exposure/diagnosis of TB are independent risk factors for developing TB in Brazil. TB cases occurred both early and lately during treatment courses, suggesting LTBI screening failures, treatment non-adherence or re-exposure. Current application and content of the protocol for screening and treatment of LTBI needs to be reviewed. References [1] WHO TB report2018.https://www.who.int/tb/publications/global_report/en/ [2] Rev Bras Reumatol Engl Ed. 2017;57Suppl 2:477-483. doi: 10.1016/j.rbre.2017.05.005. Acknowledgement: monitor P Cabral, MDs N Sacilotto, H Pereira, F Sztajnbok. Disclosure of Interests: Ana Medeiros: None declared, M Bredemeier: None declared, V Valim: None declared, M Pinheiro Consultant for: Janssen, Pfizer, Speakers bureau: Abbot,Janssen,Novartis, C Macieira: None declared, A Duarte: None declared, B Stadler: None declared, R Ranza: None declared, M Bertolo: None declared, J Miranda Speakers bureau: Pfizer, C Brenol Speakers bureau: Pfizer, Roche, Janssen, Bristol., G Castro: None declared, V Fernandes Speakers bureau: Janssen, D Titton: None declared, F Sauma: None declared, I Pereira: None declared, R Botelho: None declared, H Carvalho: None declared, A Hayata: None declared, P Louzada: None declared, A Ranzolin: None declared, S Studart: None declared, G Castelar: None declared, A Kakehasi Grant/research support from: Abbvie, UCB, JANSSEN, ROCHE, NOVARTIS, PFIZER, Consultant for: Abbvie, UCB, JANSSEN, ROCHE, NOVARTIS, PFIZER, Paid instructor for: JANSSEN, Speakers bureau: Abbvie, UCB, JANSSEN, ROCHE, NOVARTIS, PFIZER, W Bianchi: None declared, R Acayaba: None declared, I Silveira: None declared, H Resende: None declared, J Amaral: None declared, L Rocha: None declared, M Gazzeta: None declared, L Carvalho: None declared, S Schowalski: None declared, D Feldman: None declared, I Laurindo Consultant for: Abbvie, UCB, GSK, JANSSEN, LILLY, NOVARTIS, PFIZER, Paid instructor for: Abbvie, JANSSEN, Speakers bureau: Abbvie, UCB, GSK, JANSSEN, LILLY, NOVARTIS, PFIZER, ROCHE, J Provenza: None declared

Objectives To analyse how the age at diagnosis modifies the phenotype of primary Sjogren syndrome (SS) Methods The Big Data Sjogren Project was formed in 2014 to take a “high-definition” picture of the main features of primary SS at diagnosis by merging international SS databases. By January 2019, the database included 11,420 patients from 24 countries of the 5 continents. Results Women (52.7 vs 54.6 yrs in men, p Patients with positive immunological markers had a younger diagnostic age, except for cryoglobulins (p Patients without systemic activity (ESSDAI score = 0) were diagnosed at an older age (55.5 vs 52.1 yrs in those with systemic activity, p Conclusion Age at diagnosis plays a key role in the glandular and systemic phenotype expressed by primary SjS patients at the time of diagnosis. Disclosure of Interests: Soledad Retamozo: None declared, Nihan Acar-Denizli: None declared, Wan Fai Ng: None declared, Ildiko Fanny Horvath: None declared, Astrid Rasmussen: None declared, Raphaele Seror Grant/research support from: Pfizer, Consultant for: Bristol-Myers Squibb, Pfizer, Amgen, Eli Lilly, Roche, Celgene, GlaxoSmithKline, MedImmune, Xiaomei Li: None declared, Chiara Baldini: None declared, Jacques-Eric Gottenberg Grant/research support from: Bristol-Myers Squibb, Grant/research support from: Bristol-Myers Squibb, Consultant for: Bristol-Myers Squibb, Lilly, Pfizer, Sanofi-Genzyme, UCB Pharma, Consultant for: Bristol-Myers Squibb, Eli Lilly, UCB, Sanofi-Genzyme, Pfizer, Pulukool Sandhya: None declared, Luca Quartuccio: None declared, Roberta Priori: None declared, Gabriela Hernandez-Molina: None declared, Berkan Armagan: None declared, Aike A. Kruize: None declared, Seung-Ki Kwok: None declared, Marika Kvarnstrom: None declared, Sonja Praprotnik: None declared, Damien Sene: None declared, Elena Bartoloni Bocci: None declared, Roser Solans-Laque: None declared, Maureen Rischmueller Consultant for: Abbvie, Bristol-Meyer-Squibb, Celgene, Glaxo Smith Kline, Hospira, Janssen Cilag, MSD, Novartis, Pfizer, Roche, Sanofi, UCB, Thomas Mandl: None declared, Yasunori Suzuki: None declared, David Isenberg: None declared, Valeria Valim: None declared, Agata Sebastian: None declared, Gunnel Nordmark: None declared, Hendrika Bootsma: None declared, Hideki Nakamura: None declared, Roberto Giacomelli Grant/research support from: Pfizer, Actelion, Speakers bureau: Actelion, Bristol-Myers Squibb, Merck Sharp & Dohme, Abbvie, Pfizer, Sobi, Roche, Valerie Devauchelle-Pensec Grant/research support from: Roche-Chugai, Speakers bureau: MSD, BMS, UCB, Roche, Benedikt Hofauer Consultant for: Consultant for Galvani Bioelectronics for the area of sleep disorders., Michele Bombardieri Grant/research support from: Celgene, Consultant for: Medimmune, Virginia Fernandes Moca Trevisani: None declared, Daniel Hammenfors: None declared, Sandra Pasoto: None declared, Tamer A Gheita: None declared, Fabiola Atzeni: None declared, Jacques Morel: None declared, Cristina Vollenveider: None declared, Sandra Consani-Fernandez: None declared, Xavier Mariette Grant/research support from: Servier, Consultant for: AstraZeneca, Bristol-Myers Squibb, GlaxoSmithKline, Janssen, Pfizer, UCB Pharma, Manuel Ramos-Casals: None declared, Pilar Brito-Zeron: None declared