The 2023 ACR/EULAR antiphospholipid syndrome classification criteria identify patients at high risk of complications.

OBJECTIVES: This study aims to evaluate the utility of the 2023 ACR/EULAR antiphospholipid syndrome (APS) classification criteria in identifying primary APS patients at high risk of complications. METHODS: In this single-center study, primary APS patients were classified according to both the revised Sapporo criteria and the 2023 ACR/EULAR criteria. The risk of complications was assessed using the adjusted Global Antiphospholipid Syndrome Score (aGAPSS). RESULTS: Forty-five patients (73% females, median age 49 years) were included. Thirty-six patients met the 2023 ACR/EULAR criteria, all of whom also fulfilled the revised Sapporo criteria. Additionally, four out of nine patients not meeting the 2023 ACR/EULAR criteria satisfied the revised Sapporo criteria. Agreement rate between the two classification criteria was 91%, with a Cohen's kappa index of 0.66. Patients meeting the 2023 ACR/EULAR criteria had significantly higher aGAPSS scores compared to those who did not (13, 8-13 vs. 3, 0-5; p = 0.005). Furthermore, 55% of patients meeting the 2023 ACR/EULAR criteria were categorized as high risk based on aGAPSS scores, while those not meeting the criteria were predominantly categorized as low risk (77%). Interestingly, patients not meeting the 2023 ACR/EULAR criteria but fulfilling the revised Sapporo criteria had significantly higher aGAPSS scores compared to those not meeting either set of criteria (7, 5-13 vs. 0, 0-1.5; p = 0.015). CONCLUSION: The 2023 ACR/EULAR criteria effectively identify primary APS patients at increased risk of complications, as indicated by the aGAPSS score. Key Points • Identifying primary APS patients at high risk of complications remains a significant challenge. • The 2023 ACR/EULAR criteria show a correlation with the aGAPSS score, exhibiting the highest correlation with laboratory domains and minimal correlation with clinical domains. • The 2023 ACR/EULAR classification criteria are effective in identifying primary APS patients at high risk of complications.

Updates in diagnosis and treatment of pediatric antiphospholipid syndrome.

PURPOSE OF REVIEW: This review offers an overview of the most important recent articles on pediatric APS. RECENT FINDINGS: Non-thrombotic extra criteria manifestations were prevalent in pediatric APS. Pregnancy morbidity has been described as the first manifestation of APS at youth age, impairing gestational outcomes. The 2023 APS criteria were developed for adult APS patients, and there is still a lack of pediatric-specific APS criteria. Catastrophic APS was more commonly reported as the initial manifestation of pediatric APS than in adults. Regarding treatment, direct oral anticoagulants have been recently approval for pediatric patients with venous thrombosis. New approaches have been proposed for severe cases, for arterial thrombosis, and rituximab for refractory cases. Recurrences typically occurred early and were associated with older age at diagnosis. Current studies highlighted the multifaceted nature of pediatric APS. Further large prospective multicenter studies evaluating new medications capable of reducing recurrence risk and improving prognosis in this population will be required.

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.

Is vitamin D status relevant to psoriasis and psoriatic arthritis? A retrospective cross-sectional study

Abstract BACKGROUND: Psoriasis is a systemic, immune-mediated disease characterized by inflammatory manifestations in the skin and joints. Vitamin D deficiency is currently considered a pandemic and is associated with comorbidities including psoriasis and psoriatic arthritis (PsA). OBJECTIVES: To determine the prevalence of hypovitaminosis D [25(OH)D] in patients with plaque psoriasis, with and without PsA, and of independent predictors of serum 25(OH)D levels. DESIGN AND SETTING: Retrospective cross-sectional study conducted among 300 patients at an outpatient clinic in a university center in Juiz de Fora, Minas Gerais, Brazil. METHODS: Demographic and clinical data (psoriasis area and severity index [PASI], family history, age at onset, disease duration, and the presence of PsA according to Classification Criteria for Psoriatic Arthritis), skin phototype, and season of the year were reviewed. RESULTS: Hypovitaminosis D (< 30 ng/mL) was highly prevalent in patients with psoriasis with and without PsA (82.2% and 74.9%, respectively). An inverse correlation between PASI and vitamin D was found (without PsA r = -0.59 and, PsA r = -0.52, P < 0.001), and multivariate regression revealed that hypovitaminosis D was associated with disease severity, season, and phototype. It was confirmed by binary logistic regression between PASI and vitamin D deficiency (< 30 ng/mL), (odds ratio, OR 1.78 CI: -0.20-0.53, P < 0.001). CONCLUSION: Hypovitaminosis D (< 30 ng/mL) was highly prevalent in psoriatic patients with and without PsA. Season and skin phototype were associated with 25(OH)D levels. An inverse association between PASI and serum 25(OH)D levels was established.

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.

Antinuclear antibodies (ANA) as a criterion for classification and diagnosis of systemic autoimmune diseases.

The classification and diagnosis of systemic autoimmune diseases are frequently based on a collection of criteria composed of clinical, laboratory, imaging, and pathology elements that are strongly associated with the respective disease. Autoantibodies are a distinctive hallmark and have a prominent position in the classification criteria of many autoimmune diseases. The indirect immunofluorescence assay on HEp-2 cells (HEp-2 IFA), historically known as the antinuclear antibody test, is a method capable of detecting a wide spectrum of autoantibodies. A positive HEp-2 IFA test is part of the classification criteria for systemic lupus erythematosus (SLE) and juvenile idiopathic arthritis (JIA), as well as the diagnostic criteria for autoimmune hepatitis (AIH) and primary biliary cholangitis (PBC). A positive HEp-2 IFA test can appear as different morphological patterns that are indicative of the most probable autoantibody specificities in the sample. Only some of the HEp-2 IFA patterns are associated with the specific autoantibodies relevant to SLE, JIA, AIH, and PBC, whereas some other patterns occur mainly in non-related conditions and even in apparently healthy individuals. This paper provides a critical review on the subject and proposes that the classification and diagnostic criteria for SLE, JIA, AIH, and PBC could be improved by a modification on the HEp-2 IFA (ANA) criterion in that the staining patterns accepted for each of these diseases should be restricted according to the respective relevant autoantibody specificities.

The heterogeneity of systemic lupus erythematosus: Looking for a molecular answer / La heterogeneidad del lupus eritematoso sistémico: buscando una respuesta molecular

ABSTRACT The heterogeneity of SLE is a major limitation when designing clinical trials and understand ing the mechanisms of the disease. The analyses conducted before the new technologies for the identification of the single cell transcriptome focused on the detection of molecular patterns such as interferon signature in total blood or through the analysis of major sepa rate cell populations, such as CD4+ T cells. The analyses of molecular patterns have mainly focused on the transcriptome and DNA methylation changes. The first studies on single cell transcriptomics have now been published for mononuclear blood cells and tissues or the knowledge derived from them, total kidney, tubules and skin keratinocytes. The latter have defined patterns of nonresponse to treatment. However, much work still needs to be done to be able to use these methods in clinical practice.

RESUMEN La heterogeneidad del lupus es una limitante al momento de diseñar estudios clínicos, así como también para nuestra facultad de comprender los mecanismos de la enfermedad. Los análisis previos a las nuevas tecnologías para la detección del transcriptoma de célula única trabajaron en la identificación de patrones moleculares, como la firma del interferón en sangre total, o a través del análisis de poblaciones celulares principales separadas, como son las células T CD4+. Los análisis de patrones moleculares se han enfocado primordialmente en el transcriptoma y en los cambios de metilación del ADN. Ya se han publicado los primeros estudios de transcriptoma de célula única para células sanguíneas mononucleares y para tejidos, riñón total, túbulos y queratinocitos de piel. Estos últimos han definido patrones de no-respuesta al tratamiento. Aún falta mucho para que los métodos o los conocimientos derivados de los mismos sean de utilidad en la práctica clínica.

Assessing rheumatologists' attitudes and utilization of classification criteria for ankylosing spondylitis and axial spondyloarthritis: a global effort.

OBJECTIVES: This study aims to assess rheumatologists' perceptions, utilization patterns, and attitudes towards the modified New York (mNY) criteria for ankylosing spondylitis (AS) and Assessment of SpondyloArthritis International Society (ASAS) criteria for axial spondyloarthritis (axSpA). METHODS: Members of the national rheumatology societies in five countries (United States of America (USA), Canada, India, Turkey, and Brazil) were invited to participate in a survey containing questions regarding rheumatologists' familiarity, and use of AS and axSpA classification criteria in daily practice, perceived specificity of spondyloarthritis features in making the diagnosis, patterns of imaging tests performed in daily practice, and their opinion about the need for modification of current classification criteria. The responses were analyzed by gender, age, years in practice, as well as by country of practice. Descriptive statistics, t test, and chi-square test were used for comparison of groups. RESULTS: Approximately 6% rheumatologists (478 out of 8021 professional association members) from five countries completed the survey. The country-specific response rates were Brazil 4%, USA 4.3%, India 11%, Canada 14%, and Turkey 29%, though the overall contributions from individual countries were USA 47%, India 14.9%, Brazil 13.8%, Turkey 12.8%, and Canada 8.8%. The mean age of respondents was 50 years (± 11.8), 31% were females and 90% spent majority (> 75%) of their time in clinical practice. The mNY and ASAS criteria were regularly used in clinical practice by 44 and 66% of responders, respectively. Those reporting "always" using ASAS criteria were more likely to be women (p = 0.006), and within 5 years of completing rheumatology training. Vast majority (74%) regarded Inflammatory Back Pain (IBP) as a specific feature for axSpA. Majority (50 and 60%, respectively) regarded uveitis and dactylitis as "very specific" features helping them make the diagnosis of axSpA, whereas heel enthesitis, peripheral inflammatory arthritis, and response to NSAIDs were considered "somewhat specific" by 50% of the responders. Less than half (47%) of the responders used the mNY grading for X-ray of SI joints. In the case of normal X-ray of SI joint, the use of MRI was more frequent than CT scan (83.6 vs. 10.9%) in assessing for sacroiliitis. If sacroiliitis was not seen on X-rays, the likelihood of ordering MRI was significantly higher among rheumatologists completing training within < 15 years versus > 25 years prior (90 vs. 75%, p = 0.007). Overall, 70% thought that ASAS criteria were adequately specific for clinical trials. However, 42% respondents still felt a need to modify ASAS classification criteria for axSpA. Also, 46% respondents felt that mNY criteria should be modified. CONCLUSIONS: In the absence of diagnostic criteria, majority of rheumatologists are using the classification criteria for diagnosis of axSpA. Though axSpA classification criteria are perceived to be specific for clinical trials, 40% rheumatologists feel the need to modify these criteria. Key Points • This study informs how rheumatologists in five countries spread over four different continents diagnose axSpA in clinical practice. • Since majority rheumatologists among survey respondents across the countries use ASAS criteria for diagnosis of axSpA, more specific criteria may be required to avoid overdiagnosis. • MRI is commonly used to rule out sacroiliitis in case of normal X-ray of sacroiliac joints.

Questions as regards the recognition of elderly-onset primary Sjögren's syndrome: Where we are and where we would rather be / Preguntas sobre el reconocimiento del síndrome de Sjögren primario con inicio en la edad avanzada: ¿dónde estamos y dónde preferiríamos estar?

ABSTRACT Several epidemiological studies have suggested that the prevalence of the onset of primary Sjögren's Syndrome in the elderly (EOpSS) is significantly higher (between five to eight times) than in other age groups. However, when a literature review was performed, the number of patients with EOpSS was much lower than epidemiologically expected. An evaluation was performed on Sjögren (sicca) syndrome, including immunological markers, labial salivary glands biopsy, and some extra-glandular manifestations. These could be confounding factors in the elderly patients, much more so than in other age groups, and lead to a misdiagnosis of EOpSS. This article presents a review of the most common difficulties that may be present in the recognition of EOpSS, and highlights the lack of elderly patient-centred studies as the most important unmet need.

RESUMEN Varios estudios epidemiológicos han sugerido que la prevalencia del síndrome de Sjögren primario (SSp) en la población de edad avanzada (Elderly-Onset primary Sjögren's Syndrome [EOpSS], según la clasificación inglés) es considerablemente mayor (entre 5 y 8 veces) que en grupos de edad diferente. Sin embargo, una revisión sistemática de la literatura mostró que el número de pacientes con EOpSS era mucho menor de lo que se esperaba epidemiológicamente. La evaluación del síndrome de sicca, los marcadores inmunológicos, la biopsia de las glándulas salivales labiales y algunas manifestaciones extraglandulares podrían convertirse en factores de confusión en pacientes de edad avanzada mucho más frecuentemente que en personas de otros grupos de edad, lo que favorecería un diagnóstico erróneo del EOpSS. En este artículo se revisan las principales dificultades que pueden afectar al reconocimiento del EOpSS, destacando la falta de estudios centrados en el paciente anciano como la necesidad insatisfecha más importante.

Comparison among ACR1997, SLICC and the new EULAR/ACR classification criteria in childhood-onset systemic lupus erythematosus.

BACKGROUND: To date there are no specific classification criteria for childhood-onset systemic lupus erythematosus (cSLE). This study aims to compare the performance among the American College of Rheumatology (ACR) 1997, the Systemic Lupus International Collaborating Clinics criteria (SLICC) and the new European League Against Rheumatism (EULAR)/ACR criteria, in a cSLE cohort. METHODS: We conducted a medical chart review study of cSLE cases and controls with defined rheumatic diseases, both ANA positive, to establish each ACR1997, SLICC and EULAR/ACR criterion fulfilled, at first visit and 1-year-follow-up. RESULTS: Study population included 122 cSLE cases and 89 controls. At first visit, SLICC criteria had higher sensitivity than ACR 1997 (89.3% versus 70.5%, p <  0.001), but similar specificity (80.9% versus 83.2%, p = 0.791), however performance was not statistically different at 1-year-follow-up. SLICC better scored in specificity compared to EULAR/ACR score ≥ 10 at first visit (80.9% versus 67.4%, p = 0.008) and at 1-year (76.4% versus 58.4%, p = 0.001), although sensitivities were similar. EULAR/ACR criteria score ≥ 10 exhibited higher sensitivity than ACR 1997 (87.7% versus 70.5%, p <  0.001) at first visit, but comparable at 1-year, whereas specificity was lower at first visit (67.4% versus 83.2%, p = 0.004) and 1-year (58.4% versus 76.4%, p = 0.002). A EULAR/ACR score ≥ 13 against a score ≥ 10, resulted in higher specificity, positive predictive value, and cut-off point accuracy. Compared to SLICC, a EULAR/ACR score ≥ 13 resulted in lower sensitivity at first visit (76.2% versus 89.3%, p <  0.001) and 1-year (91% versus 97.5%, p = 0.008), but similar specificities at both assessments. When compared to ACR 1997, a EULAR/ACR total score ≥ 13, resulted in no differences in sensitivity and specificity at both observation periods. CONCLUSIONS: In this cSLE population, SLICC criteria better scored at first visit and 1-year-follow-up. The adoption of a EULAR/ACR total score ≥ 13 in this study, against the initially proposed ≥10 score, was most appropriate to classify cSLE. Further studies are necessary to address if SLICC criteria might allow fulfillment of cSLE classification earlier in disease course and may be more inclusive of cSLE subjects for clinical studies.

Performance of distinct knee osteoarthritis classification criteria in the ELSA-Brasil musculoskeletal study.

OBJECTIVE: To compare the performance of different knee osteoarthritis (OA) classification criteria in a subsample of subjects of ELSA-Brasil Musculoskeletal cohort (ELSA-Brasil MSK) in comparison with the clinical-radiographic evaluation of an experienced rheumatologist. METHOD: A cross-sectional study of the diagnostic accuracy of different knee OA classification criteria: radiographic OA, symptomatic OA, clinical and clinical-radiographic criteria of the American College of Rheumatology (ACR), and the definition proposed by the National Institute for Health and Care Excellence (NICE), UK. The study also evaluated some alternative definitions of OA including a combination of criteria. In total, 250 subjects participated. Only one knee per subject was included. When both or none of the knees were affected, one knee was randomly selected for analysis. OA prevalence, sensitivity, specificity, positive and negative predictive values, and accuracy were presented (CI 95%; α = 5%). RESULTS: The mean age was 56.1 years (SD = 8.7), 48.8% female, and 39.2% presented knee OA according to the rheumatologist. The sensitivity and the specificity of radiographic OA were 51% and 96.7%, respectively, while the NICE definition showed 57.0% and 76.3%, respectively. The other OA criteria showed good levels of specificity, but the levels of sensitivity were below 30%. Considering NICE and/or X-ray result in combination, the sensitivity increased (73.4%), while the specificity reduced slightly (73.0%) in relation to the original NICE definition. CONCLUSIONS: Radiographic OA showed the best performance, followed by NICE definition, especially in combination with X-ray results.

Validade dos critérios de classificação da osteoartrite de joelho: ELSA-Brasil Musculoesquelético (ELSA-Brasil ME) / Validity of the knee osteoarthritis classification criteria: ELSA-Brasil Musculoskeletal (ELSA-Brasil ME)

Introdução: Vários critérios de classificação de osteoartrite (OA) de joelhos estão disponíveis na literatura, geralmente divididos em clínicos, clínico-radiográficos e radiográficos. A existência de diferentes critérios de classificação de OA de joelhos dificulta uniformizar e comparar resultados em estudos epidemiológicos, assim como a investigação de fatores de risco e manifestações clínicas associados à OA de joelhos. O conhecimento sobre a capacidade desses diferentes critérios em discriminar indivíduos com e sem OA, assim como conhecer a força de associação de fatores de risco, da presença de dor e de limitação funcional com os diferentes critérios, é essencial para se compreender as vantagens e limitações do uso de cada um deles. Objetivo: avaliar a validade de diferentes critérios de classificação de OA de joelhos em uma amostra de servidores públicos acompanhados pelo Estudo Longitudinal de Saúde do Adulto, ELSA-Brasil Musculoesquelético (ELSA-Brasil ME). Método: trata-se de um estudo transversal de validação de diferentes critérios de classificação de OA de joelho, a saber: OA sintomática, OA radiográfica, OA pelo American College of Rheumatology (ACR clínico e clínico-radiográfico) e OA pela definição do National Institute for Health and Care Excellence (NICE). Os participantes do estudo foram provenientes da coorte ELSA-Brasil ME, um estudo ancilar ao Estudo Longitudinal de Saúde do Adulto (ELSA-Brasil). O ELSA-Brasil ME acompanha 2901 servidores públicos, ativos e aposentados, em um dos seis centros de investigação do ELSA-Brasil, localizado no estado de Minas Gerais, Brasil. O presente estudo foi realizado em uma subamostra de conveniência com 250 participantes do ELSA-Brasil ME, de ambos os sexos, com idade entre 39 e 78 anos. A avaliação foi feita por uma reumatologista (RCCM) no período de fevereiro de 2014 a junho de 2015. Apenas um joelho por indivíduo foi incluído, aquele com OA pela reumatologista. Quando ambos ou nenhum dos joelhos apresentava OA, foi feita a seleção por sorteio aleatório simples. Avaliou-se o desempenho dos critérios de classificação de OA descritos anteriormente, tendo como padrão-referência a avaliação clínico-radiográfica da reumatologista. Foram apresentados dados de prevalência, sensibilidade, especificidade, valores preditivos positivo e negativo e acurácia (IC 95%;α=5%). Posteriormente, a validade de construto convergente desses critérios foi avaliada a partir da associação entre a presença de OA de joelho identificada pelos critérios e as seguintes variáveis explicativas: índice de massa corporal (IMC), dor atual e limitação funcional (subjetiva e objetiva). A presença da dor atual em joelhos e a limitação funcional subjetiva foram avaliadas pelo Western Ontario and McMaster Universities Osteoarthritis Index (WOMAC) e a limitação funcional objetiva pelo teste sentar e levantar repetido. Associações foram testadas por modelos de regressão logística binária e multinomial (IC 95%;α=5%). Resultados: a idade média foi de 56,1 anos (DP=8,7), 51,2% eram homens. A maior prevalência de OA ocorreu na avaliação da reumatologista (39,2%), seguido de OA pelo NICE (36,8%) e pelo critério OA radiográfica (22,0%). OA radiográfica demonstrou sensibilidade e especificidade de 51,0% e 96,7%, respectivamente. A definição pelo NICE apresentou sensibilidade de 57,0% e especificidade de 76,3%. Os demais critérios mostraram boa especificidade, mas sensibilidade menor que 30,0%. Na avaliação de validade de construto convergente, IMC, dor atual e limitação funcional subjetiva se associaram à OA de joelho identificada por todos os critérios, sendo a magnitude da associação particularmente forte entre dor atual e OA segundo o critério ACR clínico (OR 21,7; IC95% 7,12-66,12) e entre limitação funcional subjetiva e OA segundo o NICE (OR 32,5; IC95% 13,4-79,0). Limitação funcional objetiva apresentou associação com os critérios OA sintomática e ACR clínico e clínico-radiográfico. Conclusões: o presente estudo demonstrou que dentre os critérios avaliados a OA radiográfica demonstrou melhor desempenho quanto à sensibilidade e especificidade, seguido pela definição de OA pelo NICE. Os critérios OA sintomática e OA pelo ACR clínico e clínico-radiográfico não se mostraram adequados para estudos que objetivam avaliar prevalência, devido à baixa sensibilidade dos mesmos. Entretanto, podem ser uma alternativa em estudos longitudinais nos quais é favorável o uso de critérios com boa especificidade. Quanto à validade de construto convergente, de forma geral, os resultados encontrados oferecem suporte para todos os critérios investigados, já que houve associação de IMC, dor atual e limitação funcional subjetiva com todos os critérios e a limitação funcional objetiva apenas não se associou à OA radiográfica e ao NICE. É importante ressaltar que a escolha de qual critério utilizar em um estudo requer levar em conta qual combinação melhor atende aos objetivos preconizados pelo estudo.

Introduction: the classification criteria for knee osteoarthritis (OA) available in the literature are usually divided into three main groups; clinical, clinical-radiographic and radiographic. The existence of different knee OA classification criteria makes it difficult to standardize and compare results in epidemiological studies, and also to investigate risk factors and clinical manifestations associated with knee OA. It is essential to understand the extent to which these different criteria are able to discriminate between subjects with and without OA. It is also important to know the strength of association of the following items with the different criteria: risk factors, the presence of pain and the functionality Objective: To evaluate the validity of different knee osteoarthritis (OA) classification criteria: radiographic OA, symptomatic OA, clinical and clinical-radiographic criteria of the American College of Rheumatology (ACR) and the OA definition proposed by the National Institute for Health and Care Excellence (NICE). Method: A cross-sectional study of the validity of different knee OA classification criteria, radiographic OA, symptomatic OA, clinical and clinical-radiographic criteria of the American College of Rheumatology (ACR) and the OA definition proposed by the National Institute for Health and Care Excellence (NICE). The subjects were from the ELSA-Brasil Musculoskeletal cohort (ELSA-Brasil MSK), an ancillary investigation on musculoskeletal disorders of the Brazilian Longitudinal Study of Adult Health (ELSA-Brasil). The ELSA- Brasil MSK follows 2901 active and retired civil servants at one of the six ELSA-Brasil investigations centers, located in the State of Minas Gerais, Brazil. The present study was carried out using a convenience subsample with 250 male and female subjects aged between 39 and 78 selected from ELSA-Brasil MSK. The assessment was made by a rheumatologist (RCCM) between February 2014 and June 2015. Only one knee per subject was included i.e., the one that had OA according to the rheumatologist and, when both or none of the knees were affected, one knee was randomly selected for analysis. The performance of the classification criteria of knee OA described before was evaluated, using as reference-standard the clinicalradiographic evaluation of the rheumatologist. OA prevalence, sensitivity, specificity, positive and negative predictive values and accuracy were presented (CI 95%; α = 5%). After that the construct validity of the criteria was evaluated by the relationship between the presence of knee OA identified by the criteria and the following explanatory variables: body mass index (BMI), pain and the functional limitation. The presence of current pain in knees and the subjective functional limitation were evaluated using Western Ontario and McMaster Universities Osteoarthritis Index (WOMAC) and the objective functional limitation was evaluated by the five-times sitto-stand test (FTSTS). Associations were tested by bivariate and multinomial logistic regression models (CI 95% ;α=5%). Mean age was 56.1 years (SD=8.7); 51.2% were male. The highest knee OA prevalence was observed in OA according to the rheumatologist, followed by the NICE definition (36,8%) and radiographic OA (22.0%).The sensitivity and the specificity of radiographic OA were 51% and 96.7%, respectively, while the NICE definition showed 57.0% and 76.3%, respectively. The other OA criteria showed good levels of specificity, but the levels of sensitivity were below 30%. In the convergent construct validity evaluation, BMI, current pain and subjective functional limitation were associated with knee OA identified by all criteria. The magnitude of the estimates were particularly strong between current pain and OA according to the ACR clinical criteria (OR 21.7; 95%CI 7.12-66.12) and between subjective functional limitation and OA according to NICE (OR 32.5; 95%CI 13.4- 79.0). The objective functional limitation was associated with symptomatic OA and with clinical and clinical-radiographic ACR criteria. Conclusions: the present study demonstrated that among the evaluated criteria, radiographic OA showed the best performance, followed by the NICE OA definition. The other criteria, namely symptomatic OA, clinical ACR and clinical-radiographic ACR were not adequate for studies that aim to evaluate prevalence, due to the low sensitivity of these criteria. However, they may be an alternative in longitudinal studies in which it is appropriate to use criteria with good specificity. Regarding the convergent construct validity, overall, our results offer support for all of the investigated criteria, since there was an association of BMI, pain and subjective functional limitation with all the criteria and the objective functional limitation was not radiographic OA and NICE. It is important to emphasize that the choice of criteria for a study requires deciding which the combination best meets the study objectives.

Comparison among ACR1997, SLICC and the new EULAR/ACR classification criteria in childhood-onset systemic lupus erythematosus

Abstract Background: To date there are no specific classification criteria for childhood-onset systemic lupus erythematosus (cSLE). This study aims to compare the performance among the American College of Rheumatology (ACR) 1997, the Systemic Lupus International Collaborating Clinics criteria (SLICC) and the new European League Against Rheumatism (EULAR)/ACR criteria, in a cSLE cohort. Methods: We conducted a medical chart review study of cSLE cases and controls with defined rheumatic diseases, both ANA positive, to establish each ACR1997, SLICC and EULAR/ACR criterion fulfilled, at first visit and 1-year-follow-up. Results: Study population included 122 cSLE cases and 89 controls. At first visit, SLICC criteria had higher sensitivity than ACR 1997 (89.3% versus 70.5%, p < 0.001), but similar specificity (80.9% versus 83.2%, p = 0.791), however performance was not statistically different at 1-year-follow-up. SLICC better scored in specificity compared to EULAR/ACR score ≥ 10 at first visit (80.9% versus 67.4%, p = 0.008) and at 1-year (76.4% versus 58.4%, p = 0.001), although sensitivities were similar. EULAR/ACR criteria score ≥ 10 exhibited higher sensitivity than ACR 1997 (87.7% versus 70.5%, p < 0.001) at first visit, but comparable at 1-year, whereas specificity was lower at first visit (67.4% versus 83.2%, p = 0.004) and 1-year (58.4% versus 76.4%, p = 0.002). A EULAR/ACR score ≥ 13 against a score ≥ 10, resulted in higher specificity, positive predictive value, and cut-off point accuracy. Compared to SLICC, a EULAR/ACR score ≥ 13 resulted in lower sensitivity at first visit (76.2% versus 89.3%, p < 0.001) and 1-year (91% versus 97.5%, p = 0.008), but similar specificities at both assessments. When compared to ACR 1997, a EULAR/ACR total score ≥ 13, resulted in no differences in sensitivity and specificity at both observation periods. Conclusions: In this cSLE population, SLICC criteria better scored at first visit and 1-year-follow-up. The adoption of a EULAR/ACR total score ≥ 13 in this study, against the initially proposed ≥10 score, was most appropriate to classify cSLE. Further studies are necessary to address if SLICC criteria might allow fulfillment of cSLE classification earlier in disease course and may be more inclusive of cSLE subjects for clinical studies.

Performance of the classification criteria in patients with late-onset axial spondyloarthritis.

AIM: To evaluate the performance of four different classification criteria for spondyloarthritis (SpA) in patients with late-onset symptoms and to compare the clinical, laboratory and radiographic outcomes among the patients with symptoms before and after 45 years of age. PATIENTS AND METHODS: A total of 329 patients with SpA were enrolled in this prospective cohort. Patients with psoriatic arthritis, reactive arthritis, colitis associated arthritis and peripheral or undifferentiated SpA were excluded. The remaining individuals were divided into two groups based on their ages at the time of onset of symptoms: from 16 to 45 years of age (adult-onset, A-O) and after 45 years of age (late-onset, L-O). The clinical data were collected, including BASDAI, BASFI, BASMI, mSASSS, ASDAS, as were concomitant diseases and medications, efficacy and safety data. The performance of four SpA classification criteria, including modified New York, ESSG, Amor and ASAS, was evaluated in both groups. p value <.05 was considered as significant. RESULTS: Thirty-two patients (9.72%) had L-O axial SpA. Mean age of diagnosis and symptoms were 57.6 (8.0) years and 7.6 (5.1) years, respectively. L-O patients had statistically worse functional impairment and higher disease activity. However, they had lower radiographic sacroiliac and spine damage (p < .001). CONCLUSION: Our data showed that almost 10% of the patients with SpA had late-onset of symptoms. Moreover, they had higher disease activity, worse physical function and lower spine radiographic damage than A-O SpA patients. Additionally, the ASAS classification criteria had the best performance and might be used in clinical practice.

Performance and agreement between classification criteria and rheumatologist's diagnosis of the most widely used SpA classification criteria in a Colombian group of patients with chronic low back pain / Acuerdo entre los criterios más utilizados para la clasificación de las espondiloartritis (SpA) y el diagnóstico clínico hecho por un reumatólogo experto en un grupo de pacientes jóvenes con dolor lumbar coránico

Abstract Objective: To evaluate the performance of the most widely used SpA classification criteria in a Colombian group of patients with chronic low back pain. Methods: We assessed the ASAS and the European Spondyloarthropathy Study Group (ESSG) classification criteria in a group of 133 patients who attended consecutively over a period of six months at outpatient clinic of low back pain. All the patients were evaluated with the same protocol. The patients were divided into two groups according to the diagnosis. The diagnosis was compared with the diagnosis made by a expert rheumatologist blinded to patient information. Results: 81 patients with SpA and 52 with other diagnoses were included. There were no differences in age and age of onset of symptoms between the two groups. The SpA group included 55 males and more common clinical findings were: enthesitis, arthritis, sacroiliitis, HLA-B27-positive, previous infection, and dactylitis. The sensitivity and specificity of criteria were: ASAS criteria 96% of sensibility and 80% of specificity, and ESSG criteria 95% and 100% respectively. The agreement between the classification criteria and the diagnosis established by the rheumatologist showed a Cohen's kappa index of 0.938 for ESSG criteria (95% CI: 0.877-0.998) and 0.790 for the ASAS criteria (95% CI: 0.682-0.898). Conclusion: In a Colombian group of SpA patients, the new ASAS classification criteria have a good concordance with clinical diagnosis but are not superior to the ESSG criteria.

Resumen Objetivo: Evaluar el acuerdo entre los criterios de clasificación para SpA y el diagnóstico de un experto reumatólogo en un grupo de pacientes con dolor lumbar. Métodos: Se evaluó el comportamiento de los criterios de ASAS y del ESSG en 133 pacientes que acudieron de forma consecutiva durante seis meses a la clínica ambulatoria de dolor lumbar. Todos los pacientes se evaluaron con el mismo protocolo. Los pacientes fueron divididos en dos grupos de acuerdo con el diagnóstico. Posteriormente se estableció del acuerdo diagnóstico con el de un experto reumatólogo ciego a la información previa de los pacientes. Resultados: 81 pacientes con SpA y 52 con otros diagnósticos fueron incluidos. No hubo diferencias en la edad y la edad de aparición de los síntomas. El grupo SpA incluyó a 55 varones y los hallazgos más comunes fueron: entesitis, artritis, sacroileitis, infección previa, HLA-B27, y dactilitis. La sensibilidad y especificidad de los criterios fueron: ASAS 96% de sensibilidad y 80% de especificidad, y ESSG 95% y 100% respectivamente. El acuerdo entre los criterios de clasificación y el diagnóstico del reumatólogo mostró un índice kappa de 0,938 con criterios ESSG (IC del 95%: 0,877-0,998) y 0,790 para los criterios ASAS (IC del 95%: 0,682-0,898). Conclusión: En un grupo de pacientes colombianos SpA, los nuevos criterios de clasificación ASAS tienen una buena concordancia con el diagnóstico clínico, pero no son superiores a los criterios ESSG.

EpiFibro (Brazilian Fibromyalgia Registry): data on the ACR classification and diagnostic preliminary criteria fulfillment and the follow-up evaluation / EpiFibro (Registro Brasileiro de Fibromialgia): dados sobre a classificação do ACR e preenchimento dos critérios diagnósticos preliminares e avaliação de seguimento

Abstract Introduction: EpiFibro (Brazilian Epidemiological Study of Fibromyalgia) was created to study Fibromyalgia patients. Patients were included since 2011 according to the 1990 American College of Rheumatology Classification Criteria for Fibromyalgia (ACR1990). Objectives: To determine how many patients still fulfill the ACR1990 and the ACR2010 criteria in 2014; to determine the correlation between the impact of FM and to describe data on the follow-up evaluation. Methods: This is a cross sectional study in a multicenter cohort of patients. The data was collected between 2013 and 2015. Physician included patients that fulfilled the ACR1990 criteria on the date of entry. The follow-up data were considered only for patients with at least two evaluations. A minimally significant change was considered to be a 30% variation of parameters scores. Results: 810 patients’ data were analyzed. Patients presented a mean age of 51.8 ± 11.5 years old. There were 786 female. Most patients met both criteria. There was a greater fulfilling of the ACR2010. There was a moderate correlation between Polysymptomatic Distress Scale and Fibromyalgia Impact Questionnaire. Three hundred fourteen patients with more than one assessment were found, but 88 patients were excluded. Thus, 226 patients with one follow-up monitoring parameter were considered (Fibromyalgia Impact Questionnaire: 222; Polysymptomatic Distress Scale: 199; both: 195). The mean follow-up time was 9.1 ± 7.5 months (1–44). Most patients became stable. Conclusion: InEpiFibro, most patients fulfill simultaneously the ACR1990 and ACR2010. A larger number of patients fulfill the ACR2010 at the time of the evaluation. There was a moderate correlation between the Polysymptomatic Distress Scale and the Fibromyalgia Impact Questionnaire. Most patients remained stable over time.

Resumo Introdução: O EpiFibro (Estudo Epidemiológico Brasileiro de Fibromialgia) foi criado para estudar pacientes com fibromialgia. Foram incluídos pacientes desde 2011 de acordo com os critérios de classificação para a fibromialgia do American College of Rheumatology de 1990 (ACR1990). Objetivos: Determinar quantos pacientes ainda atendem aos critérios ACR1990 e ACR2010 em 2014; determinar a correlação entre o impacto da FM medido pelo Questionário de Impacto da Fibromialgia (FIQ) e pela Polysymptomatic Distress Scale (PDS) e descrever dados sobre a avaliação de seguimento. Métodos: Estudo transversal em uma coorte multicêntrica de pacientes. Os dados foram coletados entre 2013 e 2015. O médico incluiu pacientes que atenderam aos critérios ACR1990 no momento da entrada. Consideraram-se os dados de seguimento apenas dos pacientes com pelo menos duas avaliações. Uma variação de 30% nos escores dos parâmetros foi considerada uma alteração minimamente significativa. Resultados: Analisaram-se os dados de 810 pacientes. Os pacientes apresentaram média de 51,8 ± 11,5 anos. Havia 786 mulheres. A maior parte dos pacientes atendeu a ambos os critérios. Houve um maior atendimento aos critérios ACR2010. Houve uma correlação moderada entre a PDS e o FIQ. Encontraram-se 314 pacientes com mais de uma avaliação, mas 88 pacientes foram excluídos. Assim, foram considerados 226 pacientes com um parâmetro de monitoramento no seguimento. (FIQ: 222; PDS: 199; ambos: 195). O tempo médio de seguimento foi de 9,1 ± 7,5 meses (1 a 44). A maior parte dos pacientes tornou-se estável. Conclusão: No EpiFibro, a maior parte dos pacientes atendia simultaneamente ao ACR1990 e ao ACR2010. Uma maior quantidade de pacientes atendia ao ACR2010 no momento da avaliação. Houve uma correlação moderada. A maior parte dos pacientes manteve-se estável ao longo do tempo.

EpiFibro (Brazilian Fibromyalgia Registry): data on the ACR classification and diagnostic preliminary criteria fulfillment and the follow-up evaluation.

INTRODUCTION: EpiFibro (Brazilian Epidemiological Study of Fibromyalgia) was created to study Fibromyalgia patients. Patients were included since 2011 according to the 1990 American College of Rheumatology Classification Criteria for Fibromyalgia (ACR1990). OBJECTIVES: To determine how many patients still fulfill the ACR1990 and the ACR2010 criteria in 2014; to determine the correlation between the impact of FM and to describe data on the follow-up evaluation. METHODS: This is a cross sectional study in a multicenter cohort of patients. The data was collected between 2013 and 2015. Physician included patients that fulfilled the ACR1990 criteria on the date of entry. The follow-up data were considered only for patients with at least two evaluations. A minimally significant change was considered to be a 30% variation of parameters scores. RESULTS: 810 patients' data were analyzed. Patients presented a mean age of 51.8±11.5 years old. There were 786 female. Most patients met both criteria. There was a greater fulfilling of the ACR2010. There was a moderate correlation between Polysymptomatic Distress Scale and Fibromyalgia Impact Questionnaire. Three hundred fourteen patients with more than one assessment were found, but 88 patients were excluded. Thus, 226 patients with one follow-up monitoring parameter were considered (Fibromyalgia Impact Questionnaire: 222; Polysymptomatic Distress Scale: 199; both: 195). The mean follow-up time was 9.1±7.5 months (1-44). Most patients became stable. CONCLUSION: InEpiFibro, most patients fulfill simultaneously the ACR1990 and ACR2010. A larger number of patients fulfill the ACR2010 at the time of the evaluation. There was a moderate correlation between the Polysymptomatic Distress Scale and the Fibromyalgia Impact Questionnaire. Most patients remained stable over time.

Analysis and performance of various classification criteria sets in a Colombian cohort of patients with spondyloarthritis.

The objective of this study was to investigate the performance of classification criteria sets (Assessment of SpondyloArthritis international Society (ASAS), European Spondylarthropathy Study Group (ESSG), and Amor) for spondyloarthritis (SpA) in a clinical practice cohort in Colombia and provide insight into how rheumatologists follow the diagnostic path in patients suspected of SpA. Patients with a rheumatologist's diagnosis of SpA were retrospectively classified according to three criteria sets. Classification rate was defined as the proportion of patients fulfilling a particular criterion. Characteristics of patients fulfilling and not fulfilling each criterion were compared. The ASAS criteria classified 81 % of all patients (n = 581) as having either axial SpA (44 %) or peripheral SpA (37 %), whereas a lower proportion met ESSG criteria (74 %) and Amor criteria (53 %). There was a high degree of overlap among the different criteria, and 42 % of the patients met all three criteria. Patients fulfilling all three criteria sets were older (36 vs. 30 years), had more SpA features (3 vs. 1 features), and more frequently had a current or past history of back pain (77 vs. 43 %), inflammatory back pain (47 vs. 13 %), enthesitis (67 vs. 26 %), and buttock pain (37 vs. 13 %) vs. those not fulfilling any criteria. HLA-B27, radiographs, and MRI-SI were performed in 77, 59, and 24 % of the patients, respectively. The ASAS criteria classified more patients as having SpA in this Colombian cohort when the rheumatologist's diagnosis is used as an external standard. Although physicians do not perform HLA-B27 or imaging in all patients, they do require these tests if the clinical symptoms fall short of confirming SpA and suspicion remains.

Concordancia de los nuevos criterios ACR/EULAR y los ACR 1980 para la clasificación de la esclerosis sistémica / Concordance of the new ACR/EULAR criteria and the ACR 1980 for the classification of systemic sclerosis

Objetivo: Evaluar cumplimiento, y así mismo concordancia y discordancia de los criterios de clasificación de Esclerosis Sistémica (ES) ACR/EULAR 2013 y ACR 1980 en pacientes con diagnóstico clínico de la enfermedad. Método: Se incluyeron 169 pacientes con diagnóstico de Esclerosis Sistémica. Resultados: El 72,2 por ciento cumplía los criterios ACR 1980, y el 99,4 por ciento (168 pacientes) cumplía los criterios ACR/EULAR 2013. La concordancia absoluta de toda la muestra fue 72,7 por ciento, para el subtipo limitado 35,2 por ciento, y 100 por ciento el difuso. Se subanalizaron los pacientes con limitada que sólo cumplían criterios ACR/EULAR 2013, y se comparó con el resto de las limitadas. Los primeros presentaron en forma estadísticamente significativa menor esclerodactilia distal a MCF, menor presencia de úlceras digitales y pitting scars, menor afectación intersticial pulmonar, y mayor daño microvascular en la capilaroscopia. Conclusión: Los nuevos criterios de clasificación de Esclerosis Sistémica serían más adecuados para detectar esclerodermias limitadas, siendo dicho hallazgo estadísticamente significativo.

Objective: To evaluate the performance, and likewise concordance and discordance of the classification criteria of Systemic Sclerosis ACR/EULAR 2013 and ACR 1980 in a group of patients with clinical diagnosis of SSc. Methods: We enrolled 169 patients with diagnosis of Systemic Sclerosis. Results: 72.2 percent met the 1980 ACR criteria, and 99.4 percent met the ACR/EULAR 2013 criteria. The absolute agreement of the entire sample was 72.7 percent, 35.2 percent for the limited subtype, and 100 percent for the diffuse. Those patients with limited subtype who only met the ACR/EULAR 2013 criteria were compared with the rest of limited patients. The first group had statistically significantly lower sclerodactyly distal to MCF, lower presence of digital ulcers and pitting scars, less interstitial lung involvement, and greater abnormal nail fold capillaries. Conclusion: The new classification criteria for systemic sclerosis seem to be more suitable for detecting limited scleroderma. In the present study, statistically significant discrepancy was found in the limited subtype.

Espondiloartropatía juvenil: una visión desde la pediatría / Juvenile spondyloarthropathy: a view from pediatrics

La espondiloartropatía juvenil (EAPj) representa un grupo de artropatías crónicas que se inician en la infancia y que corresponden a entidades cuyas clasificaciones se han modificado en el transcurso de las décadas. Las clasificaciones actuales las incluyen sólo parcialmente. Las manifestaciones clínicas incluyen compromiso articular periférico asimétrico, entesis, sacroilíaco y menos frecuentemente de columna han permitido agruparlas en cinco categorías entre el que se encuentra la forma anquilosante juvenil relacionada con HLA B27 (+), el prototipo de EAPj y que podría representar a la forma de inicio de espondiloartropatía anquilosante del adulto. Los recientes avances en los estudios genéticos, en la patogenia, el desarrollo de mejores técnicas de imagenología tales como la ecografía musculo-esquelética y resonancia magnética aplicada a la Reumatología pediátrica podrían contribuir a generar criterios de clasificación de manera tal que faciliten la comunicación científica con los Reumatólogos de adultos. Un diagnóstico precoz, la aplicación de medidas de actividad de la enfermedad validadas y el oportuno manejo terapéutico obtendrán un pronóstico más favorable. Los resultados terapéuticos en EAPj presentan evidencia limitada aún requiriéndose mayor tiempo de evolución para obtener resultados a largo plazo.

Juvenile spondyloarthropathy (EAPj) represents a heterogeneous group of juvenile articular inflammatory entities and their classification have been changed during the last decades. The current classifications include only partially. The clinical manifestations of diseases involves peripheral joints, enthesis, sacroiliac and less frequently spine and they are classified in five specific subgroups among which is the juvenile ankylosing HLA B27 (+); the EAPj’s prototype and that may represent one of ankylosing spondyloarthropathy adult diseases. Recently, novel insights into the epidemiology, pathogenesis, and development of the imaging techniques such as muscle-skeletal ultrasound and magnetic resonance applied to pediatric rheumatology could be contributing to new classification criteria in order to facilitate the scientific communication with Rheumatologist of adult patients. An early diagnosis a validated measures of disease activity and treatment can change the course and outcome of disease.