Abstract Background The Primary Sjögren’s Syndrome Quality of Life questionnaire (PSS-QoL) is the first specific instrument to assess health-related quality of life (HRQoL) in Sjögren’s disease (SjD). The aim is to translate and cross-culturally adapt the PSS-QoL into Brazilian Portuguese and to evaluate its psychometric properties. Methods The original English version was translated into Brazilian Portuguese by two native Brazilians who were proficient in the English language. The retranslation was conducted by two native Americans proficient in Brazilian Portuguese. A committee undertook an analysis of the translated and retranslated versions, resulting in the generation of the first Brazilian version, which was submitted to the cross-cultural adaptation phase. In this phase, 50 participants with SjD responded to the instrument in Stages I and II, resulting in the generation of the second and final Brazilian version. To assess the psychometric properties, demographic and clinical data were collected from 75 patients. The HRQoL questionnaires (final Brazilian version of the PSS-QoL, Short Form-36 Health Survey (SF-36) and EuroQoL-5 dimension (EQ-5D)) were completed. Construct validity was analyzed using the Pearson or Spearman correlation coefficient. Reliability was analyzed using Cronbach’s alpha and the intraclass correlation coefficient (ICC). Results Eight questions and one response item were revised due to an incomprehension rate of greater than 15% among the participants in the cross-cultural adaptation phase. The final Brazilian version of the PSS-QoL was validated, revealing a high correlation between the total score and functional capacity (r= −0.713, p < 0.001), and vitality (r= −0.770, p < 0. 001) and mental health (r= −0.742, p < 0.001) domains of the SF-36 and a moderate correlation with the other domains of the SF-36 and a moderate correlation with the EQ-5D-tto (r= −0.573, p < 0.001), and EQ-5D-VAS (r= −0.559, p < 0.001). The intraobserver (ICC = 0.939; Cronbach’s alpha = 0.964) and interobserver (ICC = 0.965; Cronbach’s alpha = 0.964) reliability of the total score showed very high consistency. Conclusion The Brazilian version of the PSS-QoL has been demonstrated to be a valid and reproducible instrument for the assessment of HRQoL in patients with SjD. Sjögrens Sjögren s PSSQoL PSS QoL (PSS-QoL healthrelated related (HRQoL . (SjD) crossculturally cross culturally properties language versions crosscultural cultural phase 5 II 7 PSSQoL, QoL, Form36 Form 36 Form-3 SF36 SF (SF-36 EuroQoL5 EuroQoL EuroQoL- EQ5D EQD EQ 5D D (EQ-5D) completed Cronbachs Cronbach ICC. ICC (ICC) 15 validated r= r (r 0713 0 713 −0.713 0.001, 0001 0.001 , 001 0770 770 −0.770 0742 742 −0.742 SF-3 EQ5Dtto EQDtto tto 0573 573 −0.573 EQ5DVAS EQDVAS VAS 0559 559 −0.559 0.001. 0.939 0939 939 0.964 0964 964 0.965 0965 965 consistency (SjD Form3 3 Form- SF3 (SF-3 (EQ-5D 1 071 71 −0.71 000 0.00 00 077 77 −0.77 074 74 −0.74 SF- Dtto 057 57 −0.57 DVAS 055 55 −0.55 0.93 093 93 0.96 096 96 (SF- 07 −0.7 0.0 05 −0.5 0.9 09 9 (SF −0. −0 −

Abstract Background Enteropathic spondyloarthritis is underdiagnosed and inflammatory biomarkers and ultrasonography (US) could be useful for screening inflammatory bowel disease (IBD) patients. The objective of this study was to evaluate the prevalence of spondyloarthritis (SpA) in IBD patients, according to the Assessment of SpondyloArthritis International Society (ASAS) criteria and the correlation of results of US of entheses and joints with plasma calprotectin levels. Methods This was an observational cross-sectional study. Patients from the IBD outpatient clinic of a reference center were evaluated according to ASAS criteria classification, results of US of entheses and joints, and inflammatory biomarker measurements (erythrocyte sedimentation rates, C-reactive protein levels, fecal and plasma calprotectin levels). A p value lower than 0.05 was considered significant. Results A total of 30.5% of the studied sample (n = 118) of patients with IBD presented at least one inflammatory musculoskeletal manifestation. The overall prevalence of enteropathic SpA was 13.55%, with 10.16% axial SpA and 4.23% peripheral SpA according to the ASAS criteria. A total of 42.1% of patients had an MASEI score greater than 18, 35.2% had synovitis, and 14.7% had tenosynovitis on US, increasing the frequency of diagnosis of entero- pathic SpA to 22.8%. Plasma calprotectin levels were similar to those in healthy controls, and correlated only with the fecal calprotectin level (p 0.041). Conclusions A total of 13.5% of patients met the criteria in accordance with the ASAS criteria for enteropathic SpA, which increased to 22.8% with the addition of US. The prevalence of enthesitis, synovitis and tenosynovitis by US of symptomatic joints and entheses were 42%, 35% and 14.7% respectively. Plasma calprotectin was correlated with fecal calprotectin but not with inflammatory biomarkers or US or ASAS criteria. (US (IBD (SpA (ASAS crosssectional cross sectional classification erythrocyte rates Creactive C reactive . levels) 005 0 05 0.0 significant 305 30 5 30.5 n 118 manifestation 1355 13 55 13.55% 1016 10 16 10.16 423 4 23 4.23 421 42 1 42.1 18 352 35 2 35.2 147 14 7 14.7 entero 228 22 8 controls 0.041. 0041 0.041 041 0.041) 135 13.5 22.8 enthesitis 42% respectively 00 0. 3 30. 11 13.55 101 10.1 4.2 42. 35. 14. 004 0.04 04 13. 22. 10. 4.

Abstract Background Diagnosis of SS is a complex task, as no symptom or test is unique to this syndrome. The American-European Consensus Group (AECG 2002) and the American-European classification criteria of 2016 (ACR/EULAR 2016) emerged through a search for consensus. This study aims to assess the prevalence of Sjögren’s Syndrome (SS) in patients with Systemic Lupus Erythematosus (SLE), according to AECG 2002 and ACR-EULAR 2016 classifications, as well as clinical and histopathological features in this overlap. To date, there is no study that has evaluated SS in SLE, using the two current criteria. Methods This cross-sectional study evaluated 237 SLE patients at the outpatient rheumatology clinic between 2016 and 2018. Patients were submitted to a dryness questionnaire, whole unstimulated salivary flow (WUSF), “Ocular Staining Score” (OSS), Schirmer’s test I (ST-I), and labial salivary gland biopsy (LSGB). Results After verifying inclusion and exclusion criteria, a total of 117 patients were evaluated, with predominance of females (94%) and mixed ethnicity (49.6%). The prevalence of SS was 23% according to AECG 2002 and 35% to ACR- EULAR 2016. Kappa agreement between AECG 2002 and ACR-EULAR 2016 were 0.7 (p < 0.0001). After logistic regression, predictors for SS were: anti/Ro (OR = 17.86, p < 0.05), focal lymphocytic sialadenitis (OR = 3.69, p < 0.05), OSS ≥ 5 (OR = 7.50, p < 0.05), ST I positive (OR = 2.67, p < 0.05), and WUSF ≤ 0.1 mL/min (OR = 4.13, p < 0.05). Conclusion The prevalence of SS in SLE was 23% (AECG 2002) and 35% (ACR-EULAR 2016). The presence of glandular dysfunction, focal lymphocytic sialadenitis, and anti/Ro were predictors of SS in SLE. The greatest advantage of the new ACR-EULAR 2016 criteria is to enable an early diagnosis and identify the overlapping of these two diseases. ACR- EULAR 2016 criteria is not yet validated for secondary SS and this study is a pioneer in investigating prevalence based on the new criteria.

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 articles classified according to Oxford & Grade were included. These recommendations were developed in order to guide the 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.

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.

Objetivo: Fazer 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. Método: Foi 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 (ESS-PRI), Functional Assessment of Chronic Illness Therapy Fatigue Subscale (Facit-F) e EuroQOL(EQ-5D). Resultados: A 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.

Objective: To 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. Method: Conceptual, 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). Results: The 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 was 0.86; and total score was 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 domains of EQ-5D. The total score of PROFAD-SSI-SF had a nonstatistically significant correlation only with Mobility domain and with 1–100 range of EQ-5D.

INTRODUÇÃO: O EULAR Sjögren's Syndrome Disease Activity Index (ESSDAI) é um índice de atividade sistêmica da síndrome de Sjögren primária (SSP). OBJETIVO: Realizar a adaptação transcultural do ESSDAI para a língua portuguesa. MÉTODO: Estudo transversal com 62 pacientes com SSP de acordo com consenso europeu-americano de 2002. Foram realizadas seis etapas: equivalência conceitual, de item, semântica, operacional, funcional e de mensuração (reprodutibilidade interobservador e a validade de constructo). Para a validade, o ESSDAI foi comparado com a avaliação global do médico (PhGA), o Sjögren's Syndrome Disease Activity Index (SSDAI) e o Sjögren's Systemic Clinical Activity Index (SCAI). Os pacientes foram classificados por um médico especialista conforme a atividade da doença em dois grupos, "ativo" e "inativo", e conforme a intenção de tratar nos grupos "aumento de terapia" e "sem aumento de terapia". O ESSDAI foi testado nesses grupos. Utilizou-se os testes estatísticos: coeficiente de correlação intraclasse (CCI) e método de Bland Altman para a reprodutibilidade; e coeficiente de Spearman (r s) e teste de Mann-Whitney para a validade (P < 0,05 e IC 95%). RESULTADOS: A média do ESSDAI foi de 4,95 ± 6,73. A reprodutibilidade obteve um forte CCI de 0,89 e boa concordância. Na comparação com outros índices, apresentou forte coeficiente de Spearman com o PhGA (r s = 0,83; P < 0,000), moderado com o SSDAI (r s = 0,658 ; P < 0,000) e fraco com o SCAI (r s = 0,411; P = 0,001). O grupo "ativo" e o grupo "com aumento de terapia" obtiveram maiores valores de ESSDAI (P = 0,000). CONCLUSÃO: a versão em português do ESSDAI mostrou ser adaptável, reprodutível e válida para a língua portuguesa.

INTRODUCTION: The EULAR Sjögren's Syndrome Disease Activity Index (ESSDAI) is an index of primary Sjögren's syndrome (PSS) systemic activity. OBJECTIVE: To perform the ESSDAI transcultural adaptation into Brazilian Portuguese. METHOD: This was a cross-sectional study with 62 patients with PSS according to the criteria of the 2002 American-European Consensus. Six stages were conducted: conceptual, item, semantic, operational, functional, and measurement equivalences (interobserver reproducibility and construct validity). For the validity assessment, the ESSDAI was compared with the Physician's Global Assessment (PhGA), the Sjögren's Syndrome Disease Activity Index (SSDAI), and the Sjögren's Systemic Clinical Activity Index (SCAI). Patients were classified by a specialist physician into two groups according to disease activity (active and inactive), and according to the intention-to-treat (increase in therapy and no increase in therapy). The ESSDAI was tested in these groups. The following statistical tests were used: intraclass correlation coefficient (ICC), Bland-Altman plot for reproducibility, and Spearman's correlation coefficient (r s) and Mann-Whitney's test for validity (P < 0.05 and 95% CI). RESULTS: The mean ESSDAI score was 4.95 ± 6.73. The reproducibility obtained a strong ICC of 0.89 and good agreement. When compared with other indices, it showed a strong r s with PhGA (0.83; P < 0.000), a moderate r s with SSDAI (0.658; P < 0.000) and a weak r s with the SCAI (0.411; P = 0.001). The group "active" and the group " increase in therapy" had higher ESSDAI values (P = 0.000). CONCLUSION: The Brazilian Portuguese version of ESSDAI was shown to be adaptable, reproducible, and valid for this language.

Não há estudo anterior sobre a prevalência de síndrome de Sjögren (SS) no Brasil. O objetivo deste estudo foi avaliar a prevalência de SS na população geral de Vitória, estado do Espírito Santo, Brasil. Trata-se de estudo epidemiológico, observacional e transversal com 1.205 indivíduos randomizados, cujas idades variaram de 18 a 65 anos, residentes em Vitória. Os indivíduos foram triados para xerostomia e xeroftalmia por meio de entrevista domiciliar. Aqueles com sintomas "sicca" foram convidados a comparecer ao hospital para realizar avaliação médica adicional, avaliação do fluxo salivar não estimulado, teste de Schirmer I, exame de sangue e biopsia de glândula salivar labial menor. Os sintomas "sicca" foram identificados em 18% (217 indivíduos) da amostra. Dos 217 indivíduos com sintomas "sicca", 127 (58%) estavam disponíveis para exame. Nessa amostra, 61,7% eram mulheres e 46,8% usavam medicação. A síndrome "sicca" foi confirmada em 12% deles por pelo menos um exame (fluxo salivar ou teste de Schirmer I). Dois pacientes (0,17%) preencheram quatro critérios classificatórios americano-europeus (95% IC = 0,020-0,5983).

There has been no previous prevalence study about of Sjögren's syndrome (SS) in Brazil. The aim was to evaluate the SS prevalence in a general population in Vitória, ES, Brazil. This was an epidemiological, observational, and cross-sectional study conducted on 1,205 randomized people, aged 18-65 years, who lived in Vitória. The subjects were screened for xerostomia and xerofphthalmia through home interviews. Those with sicca symptoms were asked to report to a hospital for further medical evaluation, unstimulated salivary flow, Schirmer I test, blood analysis and minor labial salivary biopsy. Sicca symptoms were found in 18% (217 subjects) of the sample. Of the 217 subjects with sicca symptoms, 127 (58%) were available for examination. In this sample, 61.7% were female and 46.8% were under medication. Sicca syndrome was confirmed in 12% by at least one examination (salivary flow or Schirmer I). Two patients (0.17%) matched four criteria according to American-European Criteria (95% CI = 0.020-0.5983).