Supervised walking improves cardiorespiratory fitness, exercise tolerance, and fatigue in women with primary Sjögren's syndrome: a randomized-controlled trial.

OBJECTIVE: The aim of this study was to evaluate the safety and effectiveness of a supervised walking program in women with primary Sjögren's syndrome (pSS). METHODS: 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 Sjögren'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 Sjögren'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. RESULTS: 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. CONCLUSIONS: 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. TRIAL REGISTRATION: Clinical Trials.gov ID, number NCT02370225.

Prevalence of spondyloarthritis in inflammatory bowel disease according ASAS and ultrassonography and its correlation with plasma calprotectin.

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 enteropathic 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.

Brazilian version of the "Primary Sjögren's Syndrome - Quality of Life questionnaire (PSS-QoL)": translation, cross-cultural adaptation and validation.

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.

Prevalence of Sjögren's syndrome according to 2016 ACR-EULAR classification criteria in patients with systemic lupus erythematosus.

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.

Previous biological therapy and impairment of the IFN-γ/IL-10 axis are associated with low immune response to 17DD-YF vaccination in patients with spondyloarthritis.

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.

Serum biomarker profile orchestrating the seroconversion status of patients with autoimmune diseases upon planned primary 17DD Yellow fever vaccination.

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.

Planned Yellow Fever Primary Vaccination Is Safe and Immunogenic in Patients With Autoimmune Diseases: A Prospective Non-interventional Study.

Yellow Fever (YF) vaccination is suggested to induce a large number of adverse events (AE) and suboptimal responses in patients with autoimmune diseases (AID); however, there have been no studies on 17DD-YF primary vaccination performance in patients with AID. This prospective non-interventional study conducted between March and July, 2017 assessed the safety and immunogenicity of planned 17DD-YF primary vaccination in patients with AID. Adult patients with AID (both sexes) were enrolled, along with healthy controls, at a single hospital (Vitória, Brazil). Included patients were referred for planned vaccination by a rheumatologist; in remission, or with low disease activity; and had low level immunosuppression or the attending physician advised interruption of immunosuppression for safety reasons. The occurrence of AE, neutralizing antibody kinetics, seropositivity rates, and 17DD-YF viremia were evaluated at various time points (day 0 (D0), D3, D4, D5, D6, D14, and D28). Individuals evaluated (n = 278), including patients with rheumatoid arthritis (RA; 79), spondyloarthritis (SpA; 59), systemic sclerosis (8), systemic lupus erythematosus (SLE; 27), primary Sjögren's syndrome (SS; 54), and healthy controls (HC; 51). Only mild AE were reported. The frequency of local and systemic AE in patients with AID and HC did not differ significantly (8 vs. 10% and 21 vs. 32%; p = 1.00 and 0.18, respectively). Patients with AID presented late seroconversion profiles according to kinetic timelines of the plaque reduction neutralization test (PRNT). PRNT-determined virus titers (copies/mL) [181 (95% confidence interval (CI), 144-228) vs. 440 (95% CI, 291-665), p = 0.004] and seropositivity rate (78 vs. 96%, p = 0.01) were lower in patients with AID after 28 days, particularly those with SpA (73%) and SLE (73%), relative to HC. The YF viremia peak (RNAnemia) was 5-6 days after vaccination in all groups. In conclusion, consistent seroconversion rates were observed in patients with AID and our findings support that planned 17DD-YF primary vaccination is safe and immunogenic in patients with AID.

Prevalence of spondyloarthritis in inflammatory bowel disease according ASAS and ultrassonography and its correlation with plasma calprotectin

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.

Chronic Inflammatory Arthropathy Preceding Acute Systemic Manifestations of Sarcoidosis: A Possible Overlap of Idiopathic Juvenile Arthritis and Sarcoidosis.

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.

Prevalence of Sjögren's syndrome according to 2016 ACR-EULAR classification criteria in patients with systemic lupus erythematosus

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.

Assessment of fatigue and dryness in primary Sjögren's syndrome: Brazilian version of "Profile of Fatigue and Discomfort - Sicca Symptoms Inventory (short form) (PROFAD-SSI-SF)".

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 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. CONCLUSION: The Portuguese version of PROFAD-SSI-SF proved to be an adaptable, reproducible and valid tool for the Brazilian Portuguese language.

Validation and psychometric properties of the Eular Sjögren's Syndrome Patient Reported Index (ESSPRI) into Brazilian Portuguese.

OBJECTIVE: 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-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. 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.

Recommendations for the treatment of Sjögren's syndrome.

The 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.

Transcultural adaptation of the "EULAR Sjögren's Syndrome Disease Activity Index (ESSDAI)" into Brazilian Portuguese.

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.

Primary Sjögren's syndrome prevalence in a major metropolitan area in Brazil.

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).

Acute fibrinous and organizing pneumonia and undifferentiated connective tissue disease: a case report.

Acute fibrinous and organizing pneumonia (AFOP), recently described, is a histologic pattern characterized by the presence of fibrin "balls" within alveolar spaces. The term undifferentiated connective tissue disease (UCTD) is used to identify autoimmune systemic diseases that do not fulfill the criteria to be classified as a definitive connective tissue disease. The AFOP has never been reported in association with UCTD. The present reported case is a 39-year-old Caucasian, female with dry cough and progressive dyspnea. Eight months later, she was diagnosed with "organizing pneumonia" based on clinical history and radiologic images. She manifested Raynaud's Phenomenon, sicca syndrome, boot and gloves neuropathic pain, and previous hypothyroidism. Antinuclear antibody, rheumatoid factor, and specific autoantibodies were negative. Salivary gland biopsy and electroneuromyiography were normal. The capillaroscopy showed a "scleroderma" pattern with capillary deletion and ectasia. She experienced clinical and radiologic worsening. Despite being submitted to cyclophosphamide pulse, she developed hemorrhage and then died. Thoracotomy pulmonary specimen showed histological pattern of AFOP. This paper shows a rare association of AFOP with UCTD.

Validação e propriedades psicométricas do Eular Sjögren's Syndrome Patient Reported Index (ESSPRI) para a língua portuguesa / Validation and psychometric properties of the EULAR Sjögren's Syndrome Patient Reported Index (ESSPRI) into Brazilian Portuguese

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.

Avaliação da fadiga e da secura na síndrome de Sjögren primária: versão brasileira do “Profile of Fatigue and Discomfort – Sicca Symptoms Inventory (short form) (Profad-SSI-SF)” / Assessment of fatigue and dryness in primary Sjögren’s syndrome: Brazilian version of “Profile of Fatigue and Discomfort – Sicca Symptoms Inventory (short form) (PROFAD-SSI-SF)”

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. .