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Yellow fever (YF) vaccination is known to induce a suboptimal response in patients with autoimmune diseases (AIDs). To date, few studies have focused on the performance of 17DD-YF vaccination in patients with spondyloarthritis (SpA). In general, patients with SpA are young and have less comorbidities than other patients with AIDs, and frequently receive biological disease-modifying antirheumatic drugs (DMARDs) that may impact their response to vaccines. Taking this background information, the present study aimed to investigate whether the use of biological DMARDs, even after planned washout, or disease activity measured by the Bath Ankylosing Spondylitis Disease Activity Index (BASDAI), would impact the overall performance of planned 17DD-YF primary vaccination in patients with SpA. For this purpose, 74 subjects were enrolled in a prospective study, including adult patients with SpA (SpA; n = 51) and a healthy control (HC; n = 23) group. Analysis of YF specific neutralizing antibodies test (PRNT), along with YF viremia and the levels of serum chemokines, cytokines, and growth factors were performed at distinct time points (D0, D3, D4, D5, D6, D7, D14, and D28). The BASDAI scores were evaluated at D0 and D180. Data demonstrated that overall, the SpA group presented lower PRNT titers and seropositivity rates as compared to the HC group (GeoMean = 112 vs. 440; 73% vs. 96%, respectively). In SpA subgroup analyses, previous biological DMARDs (BIO-IT) led to a lower PRNT titers (BIO-IT 79, 95% CI [39-150] vs. without biological DMARDs [non-BIO-IT] 159, 95% CI [94-267], p 0.001). The non-BIO-IT group achieved a response similar to the HC group (81% vs. 96%, p = 0.112), whereas the BIO-IT group had a lower seroconversion rate (64% vs. 96% HC, p = 0.007). The BASDAI was not associated with PRNT levels and did not change after 6 months of follow-up. No differences in YF viremia were observed amongst subgroups. Higher baseline levels of serum biomarkers were observed in the BIO-IT group vs. the non-BIO-IT group, as well as in those with a BASDAI ≥ 4 vs. those with a BASDAI 4. Increasing levels of several biomarkers were observed in SpA, especially in the BIO-IT and BASDAI ≥ 4 subgroups throughout the timeline kinetics, with impairment/disturbance in the IFN-γ/IL-10 axis around the peak of viremia (D5). Altogether, these findings suggested that the use of biological DMARDs impacts the response to the 17DD-YF vaccine, even after planned washout. Therefore, previous biological DMARD therapy, the inflammatory status prior vaccination, and impairment of the IFN-γ/IL-10 axis at the peak of viremia may determine the immunogenicity of 17DD-YF vaccination in patients with SpA.

The present study aimed to investigate whether the serum biomarkers of immune response orchestrate the seroconversion status in patients with autoimmune diseases (AID) upon planned primary 17DD-YF vaccination. For this purpose a total of 161 individuals were enrolled in a prospective study, including patients with Rheumatoid Arthritis (RA = 38), Spondyloarthritis (SpA = 51), Systemic Lupus Erythematosus (SLE = 21) and Sjögren’s Syndrome (SS = 30) along with a group of healthy controls (HC = 21). Analysis of plaque reduction neutralization test (PRNT) titers and seropositivity rates along with the 17DD-YF viremia and serum biomarkers were carried out at distinct time points (D0/D3–4/D5–6/D7/D14–28). The results demonstrated an overall lower PRNT titer and seropositivity rate (170 vs. 448; 77 vs. 95%) in AID as compared to HC, especially in SpA and SLE subgroups. No significant differences were observed in the viremia levels amongst groups. In general, a more prominent serum biomarker response was observed in AID as compared to HC, throughout the timeline kinetics. Remarkably, AID/PRNT(−) exhibited higher levels of several biomarkers at baseline as compared to AID/PRNT+. Moreover, while AID/PRNT(+) exhibited earlier increase in serum biomarkers at D3–4/D5–6, the AID/PRNT(−) displayed higher response at later time points (D7/D14–D28). Of note, a synchronic increase of IFN-γ at the peak of viremia (D5–6) was observed in HC and AID/PRNT(+) groups, whereas a later asynchronous IFN-γ response was reported for AID/PRNT(−) at D7. The biomarker profile tends to deflate at post-vaccination timeline, highlighting a putative immunomodulatory effect of live attenuated 17DD-YF vaccine in AID/PRNT(+), but not in AID/PRNT(−). Altogether these data suggested that inflammatory status prior vaccination, low IFN-γ at viremia peak and the occurrence of asynchronous biomarker storm after 17DD-YF vaccination may orchestrate the lack of neutralizing antibody response γ.

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The aim of this study was to evaluate the safety and effectiveness of a supervised walking program in women with primary Sjogren’s syndrome (pSS). Forty-five sedentary women fulfilling the American European Consensus Criteria for pSS were randomized to a training group (TG, n = 23) or control group (CG, n = 22). Patients in the TG were submitted to supervise walking three times a week for 16 weeks. The patients of the CG were instructed to not perform any kind of regular physical exercise. Physical fitness [maximum oxygen uptake (VO2max) and distance], EULAR Sjogren’s Syndrome Disease Activity Index (ESSDAI), hematological tests, and Medical Outcomes Study 36 (SF-36) were assessed at baseline and week 16. In addition, EULAR Sjogren’s Syndrome Patient Reported Index (ESSPRI), Functional Assessment of Chronic Illness Therapy Fatigue Subscale (FACIT-fatigue), and Beck Depression Inventory (BDI) were measured prior to intervention, after 8 and 16 weeks. Patient global assessment of response to therapy was completed at the final assessment. An intent-to-treat analysis was performed. After 16 weeks, the mean change of VO2max (ml/kg/min), distance, and FACIT-fatigue were higher in the TG than in the CG (p = 0.016, p = 0.043 and p = 0.030, respectively). Improved cardiorespiratory fitness was associated with improvements in fatigue scores and physical components of quality of life (SF-36). Furthermore, improved fatigue scores were associated with reduced depression and improvements in the physical and mental components of SF-36. Overall, 95.4% of patients in the TG rated themselves as clinically improved versus 62% of the patients in the CG (p = 0.049). There was no flare in disease activity and no serious adverse events with exercise. This supervised walking program was demonstrated to be feasible and safe with improvements in cardiorespiratory fitness, exercise tolerance, fatigue, and patient perception of improvement in pSS patients. Clinical Trials.gov ID, number NCT02370225.

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: Alive vaccines should be used with caution in autoimmune diseases (AID).1 Objectives: To evaluate prospectively adverse events and efficacy of the Yellow Fever vaccine in patients with AID. Methods: Prospective study, including 190 individuals, 47 with Rheumatoid Arthritis (RA), 36 primary Sjogren’s Syndrome (SS), 48 Ankylosing Spondylitis (AS), 8 Systemic Sclerosis (SSc), 24 Systemic Lupus Erythematosous (SLE), and 29 Healthy Controls (HC). All individuals received 17DD (Biomanguinhos-FIOCRUZ) YF vaccine, for the first time during the 2017 Brazilian Campaign, aged > 18 years, had no or low disease activity, low immunossupression. Exclusion Criteria: previous vaccination for yellow fever or PRNT > 1:50 at baseline, primary or secondary immunodeficiency, under treatment with cyclophosphamide, chlorambucil, mycophenolate mofetil, calcineurin inhibitors, azathioprine> 2mg/kg/day, prednisone ≥20mg/day, methotrexate >20mg/week or any immunobiological drug. Viremia (CRP) and plaque reduction neutralization test (PRNT; GeoMean title) were measured before (D0) and D3, D4, D5, D6, D7, D14, D28 after vaccine. Adverse events (AE) were registered through patient report diary and medical interview at D7, D14 and D28. Results: Mean age was 52 years old for AID and 56 for HC. No serious adverse event was reported. However, mild local AE was more reported in AID as compared to HC (34.2% vs 3.4%, p=0.000). The frequency of AE was higher in AID compared to HC (34.1 vs. 3.4%). The Risk (Odds Ratio; 95%CI) for AE was 14.53 (1.9-109.7, p=0.000) and AID subgroups: RA=14.5 (1.8-116.2, p=0.0016), AS= 9.3 (1.1-76.2, p=0.0248), SS 25.1 (3.1-204.5, p

ObjectiveTo carry out the cross-cultural adaptation of EULAR Sjögren's Syndrome Patient Reported Index (ESSPRI) for Portuguese language and evaluate its psychometric properties.MethodCross-sectional study of patients with primary Sjögren's syndrome (SS). The psychometric properties (intraobserver reproducibility and construct validity) were studied. In construct validity, ESSPRI was compared with the Patient's Global Assessment (PGA), Profile of Fatigue and Discomfort (Profad), Sicca Symptoms Inventory (SSI) and Functional Assessment of Chronic Illness Therapy (Facit-F). Statistical tests used were: Cronbach's alpha, intraclass correlation coefficient (ICC), Bland–Altman method and Spearman coefficient. A value of p≤0.05 was considered significant.ResultsThere was no difference between versions in both languages; thus, a Brazilian consensual version was obtained. All subjects were women aged 49.4±11.6 years, with onset of symptoms of 7.2±5.4 years, and time of diagnosis of 3.0±3.3 years. The mean ESSPRI was 6.87±1.97. The intraobserver reproducibility was high and significant (0.911) and, with Bland–Altman method, there was no systematic bias in the agreement of measures among evaluations. A moderate correlation of ESSPRI with all tested instruments was observed.ConclusionThe Brazilian Portuguese version of ESSPRI is a valid and reproducible version.

RESUMOObjetivo:Fazer a adaptação transcultural do Eular Sjögren's Syndrome Patient Reported Index (ESSPRI) para a língua portuguesa e avaliar as suas propriedades psicométricas.Método:Estudo transversal de pacientes com síndrome de Sjögren primária (SS). Foram estudadas as propriedades psicométricas (reprodutibilidade intraobservador e a validade de construto). Na validade de construto, o ESSPRI foi comparado com o Patient's Global Assessment (PaGA), Profile of Fatigue and Discomfort (Profad), Sicca Symptoms Inventory (SSI) e Functional Assessment of Chronic Illness Therapy (Facit-fatigue). Os testes estatísticos usados foram o α-Cronbach, coeficiente de correlação intraclasse (CCI), método de Bland-Altman e coeficiente de Spearman. Foi considerado significativo o p ≤ 0,05.Resultados:Não houve diferença entre as versões nas duas línguas e obteve-se, assim, a versão consensual brasileira. Todos os indivíduos foram mulheres de 49,4 ± 11,6 anos, com início dos sintomas de 7,2 ± 5,4 anos e tempo de diagnóstico de 3 ± 3,3 anos. A média do ESSPRI foi de 6,87 ± 1,97. A reprodutibilidade intraobservador foi alta e significativa (0,911) e, no método de Bland-Altman, não houve viés sistemático na concordância das medidas entre as avaliações. Houve correlação moderada do ESSPRI com todos os instrumentos testados.Conclusão:A versão do ESSPRI em português é válida e reprodutível. ABSTRACTObjective:To carry out the cross-cultural adaptation of EULAR Sjögren's Syndrome Patient Reported Index (ESSPRI) for Portuguese language and evaluate its psychometric properties.Method:Cross-secal study of patients with primary Sjögren's syndrome (SS). The psychometric properties (intraobserver reproducibility and construct validity) were studied. In construct validity, ESSPRI was compared with the Patient's Global Assessment (PGA), Profile of Fatigue and Discomfort (Profad), Sicca Symptoms Inventory (SSI) and Functional Assessment of Chronic Illness Therapy (Facit-F). Statistical tests used were: Cronbach's alpha, intraclass correlation coefficient (ICC), Bland-Altman method and Spearman coefficient. A value of p ≤ 0.05 was considered significant.Results:There was no difference between versions in both languages; thus, a Brazilian consensual version was obtained. All subjects were women aged 49.4 ± 11.6 years, with onset of symptoms of 7.2 ± 5.4 years, and time of diagnosis of 3.0 ± 3.3 years. The mean ESSPRI was 6.87 ± 1.97. The intraobserver reproducibility was high and significant (0.911) and, with Bland-Altman method, there was no systematic bias in the agreement of measures among evaluations. A moderate correlation of ESSPRI with all tested instruments was observed.Conclusion:The Brazilian Portuguese version of ESSPRI is a valid and reproducible version.

ResumoAs recomendações propostas pela Comissão de Síndrome de Sjögren da Sociedade Brasileira de Reumatologia para tratamento da síndrome de Sjögren foram baseadas em uma revisão sistemática da literatura nas bases de dados Medline (PubMed) e Cochrane até outubro de 2014 e opinião de especialistas na ausência de artigos sobre o assunto. Foram incluídos 131 artigos classificados de acordo com Oxford & Grade. Essas recomendações foram elaboradas com o objetivo de orientar o manejo adequado e facilitar o acesso aos tratamentos para aqueles pacientes com adequada indicação de recebê‐los, considerando o contexto socioeconômico brasileiro e os medicamentos disponíveis no país.AbstractThe recommendations proposed by the Sjögren's Syndrome Committee of the Brazilian Society of Rheumatology for the treatment of Sjögren's syndrome were based on a systematic review of literature in Medline (PubMed) and the Cochrane databases until October 2014 and on expert opinion in the absence of studies on the subject. 131 items classified according to Oxford & Grade were included. These recommendations were developed in order to guide the appropriate management and facilitate the access to treatment for those patients with an appropriate indication, considering the Brazilian socioeconomic context and pharmacological agents available in this country.

ResumoObjetivoFazer a adaptação transcultural e a validação do Profile of Fatigue and Discomfort – Sicca Symptoms Inventory (short form) (Profad‐SSI‐SF), questionário que avalia os aspectos subjetivos dos sintomas da síndrome de Sjögren primária (SSp), para a língua portuguesa brasileira.MétodoFoi avaliada a equivalência conceitual, de item, semântica e operacional. A versão brasileira do Profad‐SSI‐SF foi aplicada a 62 mulheres com SSp conforme consenso europeu‐americano de 2002 para avaliar a equivalência de mensuração. Foi usado o α‐Cronbach para consistência interna; coeficiente de correlação intraclasse (ICC) para reprodutibilidade intraobservador; e coeficiente de correlação de Spearman para validade em comparação com o Patient Global Assessment (PaGA), Eular Sjögren's Syndrome Patient Reported Index (ESSPRI), Functional Assessment of Chronic Illness Therapy Fatigue Subscale (Facit‐F) e EuroQOL (EQ‐5D).ResultadosA consistência interna do Profad, do SSI e da pontuação total foi de 0,80, 0,78 e 0,87, respectivamente. A reprodutibilidade intraobservador do Profad total foi de 0,89; do SSI total de 0,86 e da pontuação total de 0,89. Na validade, o Profad apresentou correlação significativa com o PaGA (r=0,50), Facit‐F (r=0,59), Esspri (r=0,58) e todos os domínios do EQ‐5D, com exceção da mobilidade. Já o SSI apresentou correlação significativa com o PaGA (r=0,43), Facit‐F (r=0,57), Esspri (r=0,55) e a maioria dos domínios do EQ‐5D. A pontuação total do Profad‐SSI‐SF só não obteve correlação estatisticamente significante com o domínio mobilidade e escala 1 a 100 do EQ‐D5.ConclusãoA versão em português do Profad‐SSI‐SF mostrou ser adaptável, reprodutível e válida para a língua portuguesa brasileira.AbstractObjectiveTo perform a cross‐cultural adaptation and validation of the Profile of Fatigue and Discomfort – Sicca Symptoms Inventory (short form) (PROFAD‐SSI‐SF) questionnaire assessing the subjective aspects of the symptoms of primary Sjögren syndrome (pSS), for the Brazilian Portuguese language.MethodConceptual, of the item, semantic and operational equivalences were evaluated. The Brazilian version of PROFAD‐SSI‐SF was administered to 62 women with pSS according to the European‐American consensus 2002 to assess measurement equivalence. α‐Cronbach was used for internal consistency; intraclass correlation coefficient (ICC) for intraobserver reproducibility; and Spearman correlation coefficient for validity by comparing with Patient Global Assessment (PaGA), EULAR Sjögren's Syndrome Patient Reported Index (ESSPRI), Functional Assessment of Chronic Illness Therapy Fatigue Subscale (FACIT‐F) and EuroQOL (EQ‐5D).ResultsThe internal consistency of PROFAD, SSI and total score was 0.80; 0.78; and 0.87, respectively. The intraobserver reproducibility of total PROFAD was 0.89; of total SSI of 0.86; and total score of 0.89. In terms of validity, PROFAD correlated significantly with PaGA (r = 0.50), FACIT‐F (r = 0.59), ESSPRI (r = 0.58) and all domains of EQ‐5D, with the exception of Mobility. On the other hand, SSI correlated significantly with PaGA (r = 0.43), FACIT‐F (r = 0.57), ESSPRI (r = 0.55) and most areas of EQ‐5D. The total score of PROFAD‐SSI‐SF had a non‐statistically significant correlation only with Mobility domain and with 1‐100 range of EQ‐5D.ConclusionThe Portuguese version of PROFAD‐SSI‐SF proved to be an adaptable, reproducible and valid tool for the Brazilian Portuguese language.