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Sjogren's syndrome (SS) is an autoimmune disease characterized by lymphocytic infiltration of the exocrine glands and other organs. Women with SS often experience gynecological symptoms due to the disease and need extra care regarding their sexual activity, reproductive health and during pregnancy, conditions that are not properly conducted in the clinical practice. To cover this gap, a panel of experts from the Brazilian Society of Rheumatology conducted a systematic review and meta-analysis on the identification of symptoms, diagnosis, monitoring, prognosis, and treatment of these manifestations. A Focus Group meeting was held and included experts in the field and methodologists, based on a previously developed script, with themes related to the objective of the study. The most important topics were summarized and 11 recommendations were provided.

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.

Worldwide occurrence of envenomation by snakebite in pregnancy is rare according to the medical literature. The objective of the present study was to evaluate the frequency of snakebite envenomation cases as well as their obstetrical consequences. Thus, we conducted this study based on the medical records of 157 patients, aged from 13 to 54 years old who had been attended at the "João de Barros Barreto" University Hospital from January 1989 to May 1994. These patients presented epidemiological and clinical diagnosis of snakebite envenomation. Of the 157 patients, 8 (5%) were pregnant, 6 of them (75%) were in the first trimester of pregnancy and 2 (25%) in the 2nd trimester. Most of the victims were usually over 18 years old (7 cases, 87.5%). Concerning the severity of the envenomation cases, 5 of them were considered mild accidents. Obstetrical consequences occurred in 3 (37.5%) patients, 2 patients (25%) with moderate manifestations, while 1(12.5%) presented severe manifestations. Patients developed vaginal bleeding in 3 cases (100%), and uterine contraction, threatened abortion, decreased fetal movements, absence of fetal heartbeat and fetal death in one case (33.35%). Snakebite is not common among pregnant women, since in most cases, these women are homebound. However, the obstetrical consequences are severe and related to severity of the envenomation

Sjogren's syndrome (SS) is an autoimmune disease characterized by lymphocytic infiltration of the exocrine glands and other organs, associated with sicca syndrome but also with systemic involvement with varying degrees of severity. Despite their importance, some systemic manifestations, mainly liver, gastrointestinal, and pancreatic are not routinely evaluated. To address these manifestations, the Sjögren's Syndrome Committee of the Brazilian Society of Rheumatology conducted a broad systematic review of the literature on studies investigating prevalence and diagnosis of these symptoms in Sjogren´s patients and made recommendations based on the findings. Agreement between the experts was achieved using the Delphi method. This is the second part of this guideline, providing 6 recommendations for liver, gastrointestinal, and pancreatic care of SS patients.

BackgroundInflammation has been associated with higher cardiovascular risk in rheumatic autoimmune diseases. Primary Sjögren’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.ObjectivesTo assess the cardiovascular event and to evaluate its association with traditional and disease associated risk factors in pSS.MethodsWe 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.ResultsMost 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.ConclusionTraditional 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 Sjögren syndrome: a systematic review and meta-analysis. Arthritis Care Res, 2018 (Epub ahead of print);[2] Valim V, et al. Atherosclerosis in Sjögren’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 Sjögren’s Syndrome. Rev Recent Clin Trial 13(3):164-169, 2018.Disclosure of InterestsNone declared

BACKGROUNDAbout 5-10% of patients with Sjögren's syndrome (SS) will develop non-Hodgkin's lymphoma, presenting a 16 to 44 increased risk compared to the general population. The relationship between SS and other types of cancers has not been much described in the literature. METHODSA total of 1,010 consecutive patients were included in the Brazilian Sjögren's syndrome registry (RE-BRASS), fulfilling the 2002/2016 classification criteria for primary SS. The patients who developed neoplasia diagnosed according to the National Cancer Institute classification were retrospectively analyzed. RESULTSA total of 1,010 patients were included, 975 women, average age 57.21 years, 110 out of 909 patients (10%) presented cancer that was more prevalent in 55-year-old patients (p < 0,001). The diagnosis of cancer occurred after the diagnosis of SS in 10 out of 15 (66.7%) of the cases of lymphoma, 37 out of 68 (54.4%) other cancers and in 12 out of 16 (75.0%) of skin cancers. Regarding the subtypes of cancer, 21 (2.5%) patients had skin cancer, 15 (1.5%) had lymphoma, and 68 (6.7%) had other neoplasms. Basal cell carcinoma 13 (59%) was the most prevalent skin cancer, followed by melanoma 5 (0.6%). Lymphoma was the most prevalent cancer after skin cancer in our population, and non-Hodgkin MALT lymphoma 11 (73%) was the most prevalent with respect to lymphoma subtypes. Regarding other cancers, thyroid cancer 9 (0.9%) was the most prevalent, followed by ovarian cancer, 4 (0.4%) cases. Regarding the prognosis 18 out of 21 (90%) with skin cancer, 13 out of 15 (86.7%) with lymphoma and 51 out of 68 (81%) with other cancers progressed to cure, 4 (6.3%) patients had metastases, 4 (6.3%) relapsed and 4 (6.3%) died. The presence of cancer was associated with age (OR = 1.03), disease duration (OR = 1.01) and the presence of anti-Ro/SSa antibodies p < 0.008. CONCLUSIONSkin cancer is the most prevalent cancer as it is in Brazilian general population. Lymphoma was the most prevalent cancer after skin cancer in our population. Among other cancers, thyroid and ovarian cancer were the most prevalent, which differs from the Brazilian female population in which breast cancer is the most prevalent and thyroid cancer is the fifth in prevalence. Patients with SS have different kinds of cancer and its prevalence is superior and differs from the general population without SS.

BACKGROUNDThe Brazilian Registry on Primary Sjögren's Syndrome (RE-BRASS) is a prospective national cohort and research biobank. To date, 16 centers from all Brazilian regions are collaborating, with the potential to include more centers over time. The RE-BRASS started in 2021 and has the support of the Brazilian Society of Rheumatology. The main objective of RE-BRASS is to generate a set of Brazilian epidemiological data on patients with pSS fulfilling the 2002 AECG and/or 2016 ACR-EULAR classification criteria and to promote high-quality clinical research. METHODSResearch Electronic Data Capture (REDCap) will be used to insert the clinical data. In addition to demographic and laboratory data, clinical data that are being collected from enrolled patients such as disease activity (EULAR Sjögren's syndrome disease activity index -ESSDAI), disease damage (Sjögren's syndrome disease damage index -SSDDI), comorbidities, cardiovascular risk (Framingham), labial biopsy and salivary gland ultrasound of minor salivary gland, pharmacological and nonpharmacological treatment. Patients are also been interviewed by patient-reported outcomes such as EULAR Sjögren's syndrome patient-reported index (ESSPRI), profile of fatigue and discomfort (PROFAD), hospital anxiety and depression scale (HADS), Epworth sleepiness scale (ESE), international physical activity questionnaire-short form (IPAQ-SF) and EuroQol-5 Domain (EQ-5D). RESULTSCurrently 1,010 patients are included. Mean age is 55.6 ± 13.6 years and 96.5% are women. Most of the patients are white (52%), employed (38.1%) and married (56.2%). Ninety-three percent of patients present xerostomia, 93.7% xerophthalmia, 78.9% anti-Ro and 41.8% anti-La. From the 70.5% of the patients who performed labial biopsy gland, 86.3% were positive. Ninety-four percent and 90% have met the 2002 AECG and 2016 ACR-EULAR classification criteria, respectively.