Symptomatic fractures in systemic sclerosis: A case-control study.

This case-control study analyzed risk factors for symptomatic fractures in a group of 52 patients with systemic sclerosis compared with a group of 104 patients without fractures, matched for sex and age, who were attended at a single systemic sclerosis outpatient clinic from 2010 to 2020. Fractures affected predominantly vertebral (65.4%), rib (13.5%), and hip (7.7%) joints, while the mean age of fracture was 55.3 ± 9.5 years. Age at disease onset, age at diagnosis, disease duration, age at menarche, and age at menopause were similar in both groups, and 58.9% of the patients were menopausal at the time of the fracture. The presence of fractures had a significant association with densitometric osteoporosis (p < 0.001), lower weight (p = 0.032), and bone mineral index (p = 0.044), anti-RNA polymerase III (p = 0.040), use of corticosteroids (p = 0.019), and bisphosphonates (p < 0.001), as well as with densitometric T-scores of lumbar spine (p < 0.001), femoral neck (p = 0.025), and total hip (p = 0.013). Multivariate analysis showed that the variables significantly associated with fractures were high doses of corticosteroids (odds ratio = 4.10; 95% confidence interval = 1.290-13.090; p = 0.017), bisphosphonates (odds ratio = 3.91; 95% confidence interval = 1.699-8.984; p = 0.001), negative anti-Scl70 (OR = 0.34; 95% confidence interval = 0.124-0.943; p = 0.038), and lumbar T-score (odds ratio = 0.39; 95% confidence interval = 0.034-0.460; p = 0.010). In conclusion, symptomatic fractures were associated predominantly with lower bone mineral density of lumbar spine and use of high doses of corticosteroids and bisphosphonates in this cohort.

Disparity in the access to kidney transplantation for sensitized patients in the state of Sao Paulo-Brazil.

Highly sensitized (HS) patients accumulate on deceased donor kidney transplantation (DDKT) waitlists worldwide due to matching difficulty and inequity of allocation policies. Current situation of HS patients on KT waitlist in Brazil has not been published. All patients enrolled on the KT waitlist of the State of São Paulo from 2002 to 2017 were retrospectively assessed. Patients were divided into eight groups according to their degree of sensitization, PRA of 0%, >0-40%, >40-80%, >80-85%, >85-90%, >90-95%, >95-98% and > 98%. Cumulative incidence curves for transplantation or mortality/removal from waitlist were estimated by competing risk. Among 50,249 waitlisted candidates, 1247 prioritized, 2467 with age < 18 or > 75 years and 4152 submitted to living-donor KT were excluded from the analysis, remaining 42,383 patients. There were 29,664(70%) PRA 0%, 5611(13.2%) PRA > 0-40%, 3442(8.2%) PRA > 40-80%, 507(1.2%) PRA > 80-85%, 564(1.3%) PRA > 85-90%, 825(1.9%) PRA >90-95%, 859(2%) PRA > 95-98% and 911(2.2%) PRA > 98%. There was a progressive increase in the need of prioritization, waiting time for KT or on waitlist and time on dialysis as PRA increased (p < 0.001). Probability of DDKT clearly increased as PRA decreased so that PRA 0% candidates were much more likely to be transplanted compared to PRA > 98% patients(HR:13.02, p < 0.001). Waiting list mortality/removal was higher among PRA > 0-40%(HR1.05,p = 0.03), PRA > 90-95%(HR:1.10,p = 0.05), PRA > 95-98%(HR:1.26,p < 0.001) and PRA > 98%(HR:1.09,p = 0.05) patients compared to PRA zero candidates. HS patients in Sao Paulo-Brazil required greater prioritization due to lack of venous access, longer dialysis and waitlist times, lower probability of DDKT and higher rates of waitlist mortality/removal. We confirmed the disparity of access to KT among HS patients in Sao Paulo-Brazil, indicating the need of new strategies that optimize transplantation for this subcategory of patients.

Clinical and laboratory profile of juvenile-onset systemic sclerosis in a Brazilian cohort.

Objective: To characterize the clinical and laboratory profile of juvenile-onset compared to adult-onset systemic sclerosis in a large Brazilian cohort. Methods: Retrospective analysis of a cohort of 1016 systemic sclerosis patients followed at the Scleroderma Outpatient Clinic from two referral university centers in Brazil. Patients were classified as systemic sclerosis according to the 1980 American College of Rhaumatology (ACR) criteria. Juvenile-onset systemic sclerosis was defined if age at onset was <16 years. Results: Thirty-one (3.1%) patients were classified as juvenile-onset systemic sclerosis. These patients were predominantly females (90.3%), Caucasians (71.0%), and presented diffuse systemic sclerosis (51.6%), with mean age at onset of 12.71 years. Compared to the adult-onset patients, juvenile onset was associated with diffuse systemic sclerosis (p < 0.001), calcinosis (p < 0.001), myositis (p = 0.050), and lower frequency of interstitial lung disease (p = 0.050), pulmonary hypertension (p = 0.035), and esophageal (p = 0.005) involvement. Conclusion: Juvenile-onset systemic sclerosis characterized a distinct clinical pattern in this large series of systemic sclerosis patients, since it was predominantly associated with diffuse systemic sclerosis without significant organ involvement.

Different ethnic background is associated with distinct clinical profiles in the spondyloarthritides in the North and South of Brazil.

The objective of this study was to analyze the clinical profile of the spondyloarthritides (SpA) in distinct Brazilian regions. A common protocol of investigation was prospectively applied to 202 SpA patients, including 138 patients from the South and 64 patients from the North. All the patients were classified as axial or peripheral SpA. Clinical and demographic variables and disease indexes were analyzed. Bonferroni correction was used to adjust the level of significance of each test; results with p value < 0.003 were considered statistically relevant. White ethnicity was associated with positive HLA-B27, while non-Whites presented higher frequency of peripheral arthritis, although not statistically significant. When comparing non-White patients from the North with those from the South, the Southerners presented significantly higher scores of Ankylosing Spondylitis Disease Activity Score using C-reactive protein (p = 0.001) and Health Assessment Questionnaire (p = 0.001). Although not statistically significant, Northern non-White patients were more frequently males, while Southerners had higher frequency of anterior uveitis and higher Bath Ankylosing Spondylitis Disease Activity Index and Ankylosing Spondylitis Quality of Life. Brazilian SpA patients present distinct patterns of disease according to the geographic region, especially regarding the non-White populations.

Quality of life in spondyloarthritis: analysis of a large Brazilian cohort.

OBJECTIVE: To analyze quality of life and demographic and clinical variables associated to its impairment in a large Brazilian cohort of patients with spondyloarthritis (SpA). METHODS: A common protocol of investigation was applied to 1465 Brazilian patients classified as SpA according to the European Spondyloarthropaties Study Group (ESSG) criteria, attended at 29 reference centers for Rheumatology in Brazil. Clinical and demographic variables were recorded. Quality of life was analyzed through the Ankylosing Spondylitis Quality of Life (ASQoL) questionnaire. RESULTS: The mean ASQoL score was 7.74 (+5.39). When analyzing the specific diseases in the SpA group, the ASQoL scores did not present statistical significance. Demographic data showed worse scores of ASQoL associated with female gender (p=0.014) and African-Brazilian ethnicity (p<0.001). The analysis of the clinical symptoms showed that buttock pain (p=0.032), cervical pain (p<0.001) and hip pain (p=0.001) were statistically associated with worse scores of ASQoL. Continuous use of nonsteroidal anti-inflammatory drugs (p<0.001) and biologic agents (p=0.044) were associated with higher scores of ASQoL, while the other medications did not interfere with the ASQoL scores. CONCLUSION: In this large series of patients with SpA, female gender and African-Brazilian ethnicity, as well as predominant axial symptoms, were associated with impaired quality of life.

Qualidade de vida nas espondiloartrites: análise de uma grande coorte brasileira / Quality of life in spondyloarthritis: analysis of a large Brazilian cohort

Resumo Objetivo: Analisar as variáveis demográficas e clínicas associadas à diminuição da qualidade de vida em uma grande coorte brasileira de pacientes com espondiloartrite (EpA). Métodos: Foi aplicado um protocolo de pesquisa único a 1.465 pacientes brasileiros classificados como tendo EpA de acordo com os critérios do European Spondyloarthropaties Study Group (ESSG), atendidos em 29 centros de referência em reumatologia do Brasil. Foram registradas as variáveis clínicas e demográficas. A qualidade de vida foi analisada por meio do questionário Ankylosing Spondylitis Quality of Life (ASQoL). Resultados: A pontuação média do ASQoL foi de 7,74 (+ 5,39). Ao analisar doenças específicas no grupo de EpA, as pontuações do ASQoL não apresentaram diferença estatisticamente significativa. Os dados demográficos mostraram piores escores de ASQoL associados ao gênero feminino (p = 0,014) e etnia negra (p < 0,001). Quanto aos sintomas clínicos, a dor na região glútea (p = 0,032), a dor cervical (p < 0,001) e a dor no quadril (p = 0,001), estiveram estatisticamente associadas a piores escores no ASQoL. O uso contínuo de fármacos anti-inflamatórios não esteroides (p < 0,001) e agentes biológicos (p = 0,044) esteve associado a escores mais elevados de ASQoL, enquanto outros medicamentos não interferiram nos escores do ASQoL. Conclusão: Nesta grande série de pacientes com EpA, o sexo feminino e a etnia negra, bem como sintomas predominantemente axiais, estiveram associados a uma qualidade de vida reduzida.

Abstract Objective: To analyze quality of life and demographic and clinical variables associated to its impairment in a large Brazilian cohort of patients with spondyloarthritis (SpA). Methods: A common protocol of investigation was applied to 1465 Brazilian patients classified as SpA according to the European Spondyloarthropaties Study Group (ESSG) criteria, attended at 29 reference centers for Rheumatology in Brazil. Clinical and demographic variables were recorded. Quality of life was analyzed through the Ankylosing Spondylitis Quality of Life (ASQoL) questionnaire. Results: The mean ASQoL score was 7.74 (± 5.39). When analyzing the specific diseases in the SpA group, the ASQoL scores did not present statistical significance. Demographic data showed worse scores of ASQoL associated with female gender (p = 0.014) and African-Brazilian ethnicity (p < 0.001). Regarding clinical symptoms, buttock pain (p = 0.032), cervical pain (p < 0.001) and hip pain (p = 0.001), were statistically associated with worse scores of ASQoL. Continuous use of nonsteroidal anti-inflammatory drugs (p < 0.001) and biologic agents (p = 0.044) were associated with higher scores of ASQoL, while the other medications did not interfere with the ASQoL scores. Conclusion: In this large series of patients with SpA, female gender and African-Brazilian ethnicity, as well as predominant axial symptoms, were associated with impaired quality of life.

Angiomirs expression profiling in diffuse large B-Cell lymphoma.

Despite advances in treatment, 30% of diffuse large B-cell lymphoma (DLBCL) cases are refractory or relapse after chemoimmunotherapy. Currently, the relationship between angiogenesis and angiomiRs in DLBCL is unknown. We classified 84 DLBCL cases according to stromal signatures and evaluated the expression of pro- and antiangiomiRs in paraffin embedded tissues of DLBCL and correlated them with microvascular density (MVD). 40% of cases were classified as stromal-1, 50% as stromal-2 and 10% were not classified. We observed increased expression of proangiomiRs Let-7f, miR-17, miR-18a, miR-19b, miR-126, miR-130a, miR-210, miR-296 and miR-378 in 14%, 57%, 30%, 45%, 12%, 12%, 56%, 58% and 48% of the cases, respectively. Among antiangiomiRs we found decreased expression of miR-16, miR-20b, miR-92a, miR-221 and miR-328 in, respectively, 27%, 71%, 2%, 44% and 11%. We found association between increased expression of proangiomiRs miR-126 and miR-130a and antiangiomiR miR-328 and the subtype non-GCB. We found higher levels of the antiangiomiRs miR-16, miR-221 and miR-328 in patients with low MVD and stromal-1 signature. IPI and CD34 confirmed independent impact on survival of the study group. None of the above angiomiRs showed significance as biomarker in an independent serum samples cohort of patients and controls. In conclusion, we confirmed association between antiangiomiRs miR-16, miR-221 and miR-328 and stromal-1 signature. Four angiomiRs emerged as potential therapeutic targets: proangiomiRs miR-17, miR-210 and miR-296 and antiangiomiR miR-20b. Although the four microRNAs seem to be important in DLBCL pathogenesis, they were not predictive of DLBCL onset or relapse in the serum independent cohort.

Nutritional practices and postnatal growth restriction in preterm newborns / Práticas nutricionais e restrição de crescimento pós-natal em prematuros

SUMMARY Objective: to assess the nutritional practices in neonatal intensive care units (NICU) associated with growth retardation in premature (preterm) infants. Methods: retrospective study of preterm infants weighing between 500 and 1,499g admitted to NICU. Evolution of growth and parenteral (PN) and enteral (EN) nutrition practices were analyzed. Results: among 184 preterm infants divided into G1 (500 to 990g; n=63) and G2 (1000 to 1499g; n=121), 169 received PN (G1=63, G2=106). Compared with the recommendations, PN was started late, its progress was slow and the maximum glucose, amino acid, lipid and energy supplies were low in both groups. The initial supply of amino acid and lipid and initial and maximum glucose and energy were lower in G1. The onset of EN was early (1-2 days), the time to reach exclusive EN was appropriate (11-15 days) and the use of human milk was possible in a reasonable amount of time (7-13 days). The multivariate analysis showed that respiratory distress syndrome and obtaining a supply of 120 kcal/kg/day too late increased the chance of weight loss greater than 10%. Sepsis, maximum energy supply for PN <60 kcal/kg/day and obtaining a supply of 120 kcal/kg/day too late increased the chance of regaining birth weight after 14 days, while small for gestational age (SGA) at birth reduced this chance. SGA at birth, sepsis and achieving exclusive enteral nutrition after 14 days increased the chance of being SGA at post-conceptual age of term. Conclusion: improving nutritional practices in the NICU can reduce the growth deficit in premature infants of very low birth weight.

RESUMO Objetivo: avaliar as práticas nutricionais em unidade de cuidados intensivos neonatais (UCIN) associadas a déficit de crescimento em recém-nascidos pré-termo (RNPT). Métodos: estudo retrospectivo de RNPT com peso entre 500 e 1.499 g internados em UCIN. Analisaram-se: evolução do crescimento e práticas de nutrição parenteral (NP) e enteral (NE). Resultados: dentre 184 RNPT divididos em G1 (500 a 990 g; n=63) e G2 (1.000 a 1.499 g; n=121), 169 receberam NP (G1=63; G2=106). Comparando-se com as recomendações, a NP foi iniciada tardiamente, sua progressão foi lenta e as ofertas máximas de glicose, aminoácidos, lipídios e energia foram baixas nos dois grupos. A oferta inicial de aminoácido e lipídio e a inicial e máxima de glicose e energia foram menores no G1. O início da NE foi precoce (1 a 2 dias), o tempo para atingir NE exclusiva foi adequado (11 a 15 dias), e o uso de leite humano foi possível por tempo razoável (7 a 13 dias). A análise multivariada mostrou que a síndrome do desconforto respiratório e o alcance tardio da oferta de 120 kcal/kg/dia aumentaram a chance de perda de peso superior a 10%. Sepse, oferta energética máxima por NP < 60 kcal/kg/dia e alcance tardio da oferta de 120 kcal/kg/dia aumentaram a chance de recuperar o peso de nascimento após 14 dias, enquanto a condição de pequeno para idade gestacional (PIG) ao nascimento reduziu essa chance. PIG ao nascimento, sepse e alcance de NE exclusiva após 14 dias aumentaram a chance de ser PIG na idade pós-conceptual de termo. Conclusão: aprimorar as práticas nutricionais em UCIN pode reduzir o déficit de crescimento em prematuros de muito baixo peso ao nascer.

Avaliação do desempenho do BASDAI (Bath Ankylosing Spondylitis Disease Activity Index) numa coorte brasileira de 1492 pacientes com espondiloartrites: dados do Registro Brasileiro de Espondiloartrites (RBE) / Evaluation of performance of BASDAI (Bath Ankylosing Spondylitis Disease Activity Index) in a Brazilian cohort of 1492 patients with spondyloarthritis: data from the Brazilian Registry of Spondyloarthritides (RBE)

Objetivo Avaliar os resultados da aplicação do Índice de Atividade de Doença da Espondilite Anquilosante de Bath (BASDAI) numa série de pacientes brasileiros com EpA e estabelecer suas correlações com as variáveis específicas do grupo. Métodos Um protocolo comum de investigação foi prospectivamente aplicado em 1.492 pacientes brasileiros classificados como EpA pelos critérios do Grupo Europeu de Estudo das Espondiloartropatias (ESSG), acompanhados em 29 centros de referência em reumatologia no Brasil. Variáveis clínicas, demográficas e índices de doença foram colhidos. Os valores totais do BASDAI foram comparados com a presença das diferentes variáveis. Resultados O valor médio do BASDAI foi de 4,20 ± 2,38. Os escores médios do BASDAI foram mais elevados nos pacientes com forma clínica combinada, comparado às formas axiais e periféricas isoladas, nos pacientes do sexo feminino e nos sedentários. Com relação ao componente axial, valores mais altos do BASDAI estiveram significativamente associados à lombalgia inflamatória, à dor alternante em nádegas, à dor cervical e ao acometimento de coxofemorais. Houve associação estatística entre os valores do BASDAI e o comprometimento periférico, relacionado ao número de articulações inflamadas, tanto dos membros inferiores quanto dos membros superiores, e às entesites. A positividade do HLA-B27 e a presença de manifestações extra-articulares não estiveram correlacionadas com os valores médios do BASDAI. Valores mais baixos do BASDAI estiveram associados ao uso de agentes biológicos (p < 0,001). Conclusão Nesta série heterogênea de pacientes brasileiros com EpA, o BASDAI conseguiu demonstrar “atividade de doença” tanto nos pacientes com acometimento axial quanto naqueles com envolvimento periférico. .

Objective To analyze the results of the application of the Bath Ankylosing Spondylitis Disease Activity Index (BASDAI) in a large series of Brazilian patients with the diagnosis of SpA and establish its correlations with specific variables into the group. Methods A common protocol of investigation was prospectively applied to 1492 Brazilian patients classified as SpA according to the European Spondyoarthropathies Study Group (ESSG), attended at 29 referral centers of Rheumatology in Brazil. Clinical and demographic variables, and disease indices (BASDAI, Basfi, Basri, Mases, ASQol) were applied. The total values of BASDAI were compared to the presence of the different variables. Results The mean score of BASDAI was 4.20 ± 2.38. The mean scores of BASDAI were higher in patients with the combined (axial + peripheral + entheseal) (4.54 ± 2.38) clinical presentation, compared to the pure axial (3.78 ± 2.27) or pure peripheral (4.00 ± 2.38) clinical presentations (P < 0.001). BASDAI also presented higher scores associated with the female gender (P < 0.001) and patients who did not practice exercises (P < 0.001). Regarding the axial component, higher values of BASDAI were significantly associated with inflammatory low back pain (P < 0.049), alternating buttock pain (P < 0.001), cervical pain (P < 0.001) and hip involvement (P < 0.001). There was also statistical association between BASDAI scores and the peripheral involvement, related to the lower (P = 0.004) and upper limbs (P = 0.025). The presence of enthesitis was also associated to higher scores of BASDAI (P = 0.040). Positive HLA-B27 and the presence of cutaneous psoriasis, inflammatory bowel disease, uveitis and urethritis were not correlated with the mean scores of BASDAI. Lower scores of BASDAI were associated with the use of biologic agents (P < 0.001). Conclusion In this heterogeneous Brazilian series of SpA patients, BASDAI was able to demonstrate “disease ...

Nutritional practices and postnatal growth restriction in preterm newborns.

OBJECTIVE: to assess the nutritional practices in neonatal intensive care units (NICU) associated with growth retardation in premature (preterm) infants. METHODS: retrospective study of preterm infants weighing between 500 and 1,499 g admitted to NICU. Evolution of growth and parenteral (PN) and enteral (EN) nutrition practices were analyzed. RESULTS: among 184 preterm infants divided into G1 (500 to 990 g; n=63) and G2 (1000 to 1499 g; n=121), 169 received PN (G1=63, G2=106). Compared with the recommendations, PN was started late, its progress was slow and the maximum glucose, amino acid, lipid and energy supplies were low in both groups. The initial supply of amino acid and lipid and initial and maximum glucose and energy were lower in G1. The onset of EN was early (1-2 days), the time to reach exclusive EN was appropriate (11-15 days) and the use of human milk was possible in a reasonable amount of time (7-13 days). The multivariate analysis showed that respiratory distress syndrome and obtaining a supply of 120 kcal/kg/day too late increased the chance of weight loss greater than 10%. Sepsis, maximum energy supply for PN <60 kcal/kg/day and obtaining a supply of 120 kcal/kg/day too late increased the chance of regaining birth weight after 14 days, while small for gestational age (SGA) at birth reduced this chance. SGA at birth, sepsis and achieving exclusive enteral nutrition after 14 days increased the chance of being SGA at post-conceptual age of term. CONCLUSION: improving nutritional practices in the NICU can reduce the growth deficit in premature infants of very low birth weight.

[Evaluation of performance of BASDAI (Bath Ankylosing Spondylitis Disease Activity Index) in a Brazilian cohort of 1,492 patients with spondyloarthritis: data from the Brazilian Registry of Spondyloarthritides (RBE)]. / Avaliação do desempenho do BASDAI (Bath Ankylosing Spondylitis Disease Activity Index) numa coorte brasileira de 1.492 pacientes com espondiloartrites: dados do Registro Brasileiro de Espondiloartrites (RBE).

OBJECTIVE: To analyze the results of the application of the Bath Ankylosing Spondylitis Disease Activity Index (BASDAI) in a large series of Brazilian patients with the diagnosis of SpA and establish its correlations with specific variables into the group. METHODS: A common protocol of investigation was prospectively applied to 1492 Brazilian patients classified as SpA according to the European Spondyoarthropathies Study Group (ESSG), attended at 29 referral centers of Rheumatology in Brazil. Clinical and demographic variables, and disease indices (BASDAI, Basfi, Basri, Mases, ASQol) were applicated. The total values of BASDAI were compared to the presence of the different variables. RESULTS: The mean score of BASDAI was 4.20 ± 2.38. The mean scores of BASDAI were higher in patients with the combined (axial + peripheral + entheseal) (4.54 ± 2.38) clinical presentation, compared to the pure axial (3.78 ± 2.27) or pure peripheral (4.00 ± 2.38) clinical presentations (p<0.001). BASDAI also presented higher scores associated with the female gender (p<0.001) and patients who did not practice exercises (p < 0.001). Regarding the axial component, higher values of BASDAI were significantly associated with inflammatory low back pain (p<0.049), alternating buttock pain (p<0.001), cervical pain (p<0.001) and hip involvement (p<0.001). There was also statistical association between BASDAI scores and the peripheral involvement, related to the lower (p=0.004) and upper limbs (p=0.025). The presence of enthesitis was also associated to higher scores of BASDAI (p=0.040). Positive HLA-B27 and the presence of cutaneous psoriasis, inflammatory bowel disease, uveitis and urethritis were not correlated with the mean scores of BASDAI. Lower scores of BASDAI were associated with the use of biologic agents (p<0.001). CONCLUSION: In this heterogeneous Brazilian series of SpA patients, BASDAI was able to demonstrate "disease activity" in patients with axial as well as peripheral disease.

[Epidemiologic profile of juvenile-onset compared to adult-onset spondyloarthritis in a large Brazilian cohort]. / Perfil epidemiológico da espondiloartrite de início juvenil comparada com a espondiloartrite de início na vida adulta em uma grande coorte brasileira.

OBJECTIVE: To analyze the clinical and epidemiologic characteristics of juvenile-onset spondyloarthritis (SpA) (< 16 years) and compare them with a group of adult-onset (≥ 16 years) SpA patients. PATIENTS AND METHODS: Prospective, observational and multicentric cohort with 1,424 patients with the diagnosis of SpA according to the European Spondyloarthropathy Study Group (ESSG) submitted to a common protocol of investigation and recruited in 29 reference centers participants of the Brazilian Registry of Spondyloarthritis (RBE - Registro Brasileiro de Espondiloartrites). Patients were divided in two groups: age at onset<16 years (JOSpA group) and age at onset ≥ 16 years (AOSpA group). RESULTS: Among the 1,424 patients, 235 presented disease onset before 16 years (16.5%). The clinical and epidemiologic variables associated with JOSpA were male gender (p<0.001), lower limb arthritis (p=0.001), enthesitis (p=0.008), anterior uveitis (p=0.041) and positive HLA-B27 (p=0.017), associated with lower scores of disease activity (Bath Ankylosing Spondylitis Disease Activity Index - BASDAI; p=0.007) and functionality (Bath Ankylosing Spondylitis Functional Index - BASFI; p=0.036). Cutaneous psoriasis (p<0.001), inflammatory bowel disease (p=0.023), dactylitis (p=0.024) and nail involvement (p=0.004) were more frequent in patients with adult-onset SpA. CONCLUSIONS: Patients with JOSpA in this large Brazilian cohort were characterized predominantly by male gender, peripheral involvement (arthritis and enthesitis), positive HLA-B27 and lower disease scores.

Perfil epidemiológico da espondiloartrite de início juvenil comparada com a espondiloartrite de início na vida adulta em uma grande coorte brasileira / Epidemiologic profile of juvenile-onset compared to adult-onset spondyloarthritis in a large Brazilian cohort

Objetivo Analisar as características clínicas e epidemiológicas das espondiloartrites (EpA) de início juvenil (< 16 anos) e compará-las com um grupo de pacientes com EspA de início na vida adulta (≥ 16 anos). Pacientes e métodos Coorte prospectiva, observacional e multicêntrica com 1.424 pacientes com diagnóstico de EspA de acordo com o European Spondyloarthropathy Study Group (ESSG) submetidos a um protocolo comum de investigação e recrutados em 29 centros de referência participantes do Registro Brasileiro de Espondiloartrites (RBE). Os pacientes foram divididos em dois grupos: idade no início<16 anos (grupo EspAiJ) e idade no início ≥ 16 anos. Resultados Entre os 1.424 pacientes, 235 manifestaram o início da doença antes dos 16 anos (16,5%). As variáveis clínicas e epidemiológicas associadas com a EspAiJ foram: gênero masculino (p<0,001), artrite em membro inferior (p=0,001), entesite (p=0,008), uveíte anterior (p=0,041) e HLA-B27 positivo (p=0,017), em associação com escores mais baixos de atividade da doença (Bath Ankylosing Spondylitis Disease Activity Index – BASDAI; p=0,007) e de capacidade funcional (Bath Ankylosing Spondylitis Functional Index – BASFI; p=0,036). A psoríase cutânea (p<0,001), a doença inflamatória intestinal (p=0,023), a dactilite (p=0,024) e o envolvimento ungueal (p=0,004) foram mais frequentes em pacientes com EspA de início na vida adulta. Conclusões Nessa grande coorte brasileira, os pacientes com EspAiJ se caracterizavam predominantemente pelo gênero masculino, envolvimento periférico (artrite e entesite), HLA-B27 positivo e escores de doença mais baixos. .

Objective To analyze the clinical and epidemiologic characteristics of juvenile-onset spondyloarthritis (SpA) (< 16 years) and compare them with a group of adult-onset (≥ 16 years) SpA patients. Patients and methods Prospective, observational and multicentric cohort with 1,424 patients with the diagnosis of SpA according to the European Spondyloarthropathy Study Group (ESSG) submitted to a common protocol of investigation and recruited in 29 reference centers participants of the Brazilian Registry of Spondyloarthritis (RBE – Registro Brasileiro de Espondiloartrites). Patients were divided in two groups: age at onset<16 years (JOSpA group) and age at onset ≥ 16 years (AOSpA group). Results Among the 1,424 patients, 235 presented disease onset before 16 years (16.5%). The clinical and epidemiologic variables associated with JOSpA were male gender (p<0.001), lower limb arthritis (p=0.001), enthesitis (p=0.008), anterior uveitis (p=0.041) and positive HLA-B27 (p=0.017), associated with lower scores of disease activity (Bath Ankylosing Spondylitis Disease Activity Index – BASDAI; p=0.007) and functionality (Bath Ankylosing Spondylitis Functional Index – BASFI; p=0.036). Cutaneous psoriasis (p<0.001), inflammatory bowel disease (p=0.023), dactylitis (p=0.024) and nail involvement (p=0.004) were more frequent in patients with adult-onset SpA. Conclusions Patients with JOSpA in this large Brazilian cohort were characterized predominantly by male gender, peripheral involvement (arthritis and enthesitis), positive HLA-B27 and lower disease scores. .

FOXP3 and CTLA4 overexpression in multiple myeloma bone marrow as a sign of accumulation of CD4(+) T regulatory cells.

INTRODUCTION: Multiple myeloma (MM) development involves a series of genetic abnormalities and changes in the bone marrow (BM) microenvironment, favoring the growth of the tumor and failure of local immune control. T regulatory (Treg) cells play an important role in dampening anti-tumor immune responses while T-helper-17 (Th17) cells seem to be critical for the eradication of malignant cells. The aim of our study was to characterize the expression of Treg- and Th17-related genes in total myeloma BM samples to assess their role as biomarkers, prognostic factors, and possible therapeutic targets in this incurable disease. METHODS: Expression of markers for Treg (FOXP3, CTLA4) and Th17 cells (RORγt) was determined by quantitative real-time PCR in BM aspirates of 46 MM patients, four patients with monoclonal gammopathy of undetermined significance, five solitary plasmacytomas, and five healthy BM donors. Gene expression was evaluated regarding an influence on the patients' overall survival (OS). RESULTS: FOXP3 and CTLA4 presented a sixfold (p = 0.02) and 30-fold higher expression (p = 0.03), respectively, in MM patients than in controls. RORγt expression was similar in MM patients and controls. Median OS of MM patients was 16.8 (range 4.5-29.1) months, and international staging system was the only independent prognostic factor for patients survival. CONCLUSIONS: Overexpression of FOXP3 and CTLA4 in total BM samples suggests a local accumulation of immunosuppressive Tregs, the MM tumor environment, possibly dampening anti-tumor host immune responses. Therapeutic approaches targeting Treg cells and restoring local anti-tumor immunity may provide new treatment strategies for this incurable malignancy.

Assessment of fatigue in a large series of 1492 Brazilian patients with Spondyloarthritis.

BACKGROUND: The aim of the present study was to analyze the score of fatigue in a large cohort of Brazilian patients with SpA, comparing different disease patterns and its association with demographic and disease-specific variables. METHODS: A common protocol of investigation was prospectively applied to 1492 Brazilian patients classified as SpA according to the European Spondyloarthropathies Study Group (ESSG) criteria, attended at 29 reference centers. Clinical and demographic variables were recorded. Fatigue was evaluated using the first item of the Bath Ankylosing Spondylitis Disease Activity Index (BASDAI) questionnaire. RESULTS: The mean BASDAI fatigue score was 4.20 ± 2.99. There was no significant difference in the fatigue score between the different SpA. Fatigue was higher in female patients (p < 0.001), with mixed (axial + peripheral) involvement (p < 0.001) and in those who did not practice exercises (p < 0.001). Higher scores of fatigue were significantly associated with inflammatory low back pain (p = 0.013), alternating buttock pain (p = 0.001), cervical pain (p = 0.001), and hip involvement (p = 0.005). Fatigue presented a moderate positive statistical correlation with Bath Ankylosing Spondylitis Functional Index (BASFI) (0.469; p < 0.001) and Ankylosing Spondylitis Quality of Life (0.462; p < 0.001). CONCLUSION: In this large series of Brazilian SpA patients, higher fatigue scores were associated with female gender, sedentary, worse functionality, and quality of life.

Profile of the use of disease modifying drugs in the Brazilian Registry of Spondyloarthritides.

INTRODUCTION: Few studies have evaluated the profile of use of disease modifying drugs (DMD) in Brazilian patients with spondyloarthritis (SpA). METHODS: A common research protocol was applied prospectively in 1505 patients classified as SpA by criteria of the European Spondyloarthropathies Study Group (ESSG), followed at 29 referral centers in Rheumatology in Brazil. Demographic and clinical variables were obtained and evaluated, by analyzing their correlation with the use of DMDs methotrexate (MTX) and sulfasalazine (SSZ). RESULTS: At least one DMD was used by 73.6% of patients: MTX by 29.2% and SSZ by 21.7%, while 22.7% used both drugs. The use of MTX was significantly associated with peripheral involvement, and SSZ was associated with axial involvement, and the two drugs were more administered, separately or in combination, in the mixed involvement (p < 0.001). The use of a DMD was significantly associated with Caucasian ethnicity (MTX , p = 0.014), inflammatory back pain (SSZ, p = 0.002) , buttock pain (SSZ, p = 0.030), neck pain (MTX, p = 0.042), arthritis of the lower limbs (MTX, p < 0.001), arthritis of the upper limbs (MTX, p < 0.001), enthesitis (p = 0.007), dactylitis (MTX, p < 0.001), inflammatory bowel disease (SSZ, p < 0.001) and nail involvement (MTX, p < 0.001). CONCLUSION: The use of at least one DMD was reported by more than 70% of patients in a large cohort of Brazilian patients with SpA, with MTX use more associated with peripheral involvement and the use of SSZ more associated with axial involvement.

Perfil do uso de drogas modificadoras de doenca no Registro Brasileiro de Espondiloartrites / Profile of the use of disease modifying drugs in the Brazilian Registry of Spondyloarthritides

Introdução: Poucos estudos avaliaram o perfil do uso de drogas modificadoras de doença (DMD) em pacientes brasileiros com diagnóstico de espondiloartrite (EpA). Métodos: Um protocolo comum de investigação foi prospectivamente aplicado em 1505 pacientes classificados como EpA pelos critérios do Grupo Europeu de Estudo das Espondiloartrites (ESSG), acompanhados em 29 centros de referência em Reumatologia no Brasil. Variáveis clínicas e demográficas foram obtidas e avaliadas, analisando-se suas correlações com o uso das DMD metotrexato (MTX) e sulfasalazina (SSZ). Resultados: Pelo menos uma DMD foi utilizada por 73,6% dos pacientes, sendo MTX por 29,2% e SSZ por 21,7%, enquanto 22,7% utilizaram ambas as drogas. O uso do MTX foi significativamente associado ao acometimento periférico, e a SSZ foi associada ao comprometimento axial, sendo que as duas drogas foram mais utilizadas, isoladas ou combinadas, no comprometimento misto (p < 0,001). O uso de uma DMD esteve significativamente associado à etnia branca (MTX; p = 0,014), lombalgia inflamatória (SSZ; p = 0,002), dor em nádegas (SSZ; p = 0,030), cervicalgia (MTX; p = 0,042), artrite de membros inferiores (MTX; p < 0,001), artrite de membros superiores (MTX; p < 0,001), entesite (p = 0,007), dactilite (MTX; p < 0,001), doença inflamatória intestinal (SSZ; p < 0,001) e acometimento ungueal (MTX; p < 0,001). Conclusão: O uso de pelo menos uma DMD foi referido por mais de 70% dos pacientes numa grande coorte brasileira de pacientes com EpA, sendo o uso do MTX mais associado ao acometimento periférico e o uso da SSZ mais associado ao acometimento axial. .

Introduction: Few studies have evaluated the profile of use of disease modifying drugs (DMD) in Brazilian patients with spondyloarthritis (SpA). Methods: A common research protocol was applied prospectively in 1505 patients classified as SpA by criteria of the European Spondyloarthropathies Study Group (ESSG), followed at 29 referral centers in Rheumatology in Brazil. Demographic and clinical variables were obtained and evaluated, by analyzing their correlation with the use of DMDs methotrexate (MTX) and sulfasalazine (SSZ). Results: At least one DMD was used by 73.6 % of patients: MTX by 29.2 % and SSZ by 21.7%, while 22.7 % used both drugs. The use of MTX was significantly associated with peripheral involvement, and SSZ was associated with axial involvement, and the two drugs were more administered, separately or in combination, in the mixed involvement (p < 0.001). The use of a DMD was significantly associated with Caucasian ethnicity (MTX , p = 0.014), inflammatory back pain (SSZ, p = 0.002) , buttock pain (SSZ, p = 0.030), neck pain (MTX, p = 0.042), arthritis of the lower limbs (MTX, p < 0.001), arthritis of the upper limbs (MTX, p < 0.001), enthesitis (p = 0.007), dactylitis (MTX, p < 0.001), inflammatory bowel disease (SSZ, p < 0.001) and nail involvement (MTX, p < 0.001). Conclusion: The use of at least one DMD was reported by more than 70% of patients in a large cohort of Brazilian patients with SpA, with MTX use more associated with peripheral involvement and the use of SSZ more associated with axial involvement. .

Artrite enteropática no Brasil: dados do registro brasileiro de espondiloartrites / Enteropathic arthritis in Brazil: data from the brazilian registry of spondyloarthritis

As doenças inflamatórias intestinais (doença de Crohn e retocolite ulcerativa) apresentam manifestações extraintestinais em um quarto dos pacientes, sendo a mais comum a artrite enteropática. MÉTODOS: Estudo prospectivo, observacional e multicêntrico, realizado com pacientes de 29 centros de referência participantes do Registro Brasileiro de Espondiloartrites (RBE), que se incorpora ao grupo RESPONDIA (Registro Ibero-americano de Espondiloartrites). Dados demográficos e clínicos de 1472 pacientes foram colhidos, e aplicaram-se questionários padronizados de avaliação de mobilidade axial, de qualidade de vida, de envolvimento entesítico, de atividade de doença e de capacidade funcional. Exames laboratoriais e radiográficos foram realizados. Objetivamos, neste presente artigo, comparar as características clínicas, epidemiológicas, genéticas, imagenológicas, de tratamento e prognóstico de enteroartríticos com os outros espondiloartríticos nesta grande coorte brasileira. RESULTADOS: Foram classificados como enteroartrite 3,2% dos pacientes, sendo que 2,5% tinham espondilite e 0,7%, artrite (predomínio periférico). O subgrupo de indivíduos com enteroartrite apresentava maior prevalência de mulheres (P < 0,001), menor incidência de dor axial inflamatória (P < 0,001) e de entesite (P = 0,004). O HLA-B27 foi menos frequente no grupo de enteroartríticos (P = 0,001), mesmo se considerado apenas aqueles com a forma axial pura. Houve menor prevalência de sacroiliíte radiológica (P = 0,009) e também menor escore radiográfico (BASRI) (P = 0,006) quando comparado aos pacientes com as demais espondiloartrites. Também fizeram mais uso de corticosteroides (P < 0,001) e sulfassalasina (P < 0,001) e menor uso de anti-inflamatórios não hormonais (P < 0,001) e metotrexato (P = 0,001). CONCLUSÃO: Foram encontradas diferenças entre as enteroartrites e as demais espondiloartrites, principalmente maior prevalência do sexo feminino, menor frequência do HLA-B27, associados a uma menor gravidade do acometimento axial.

Inflammatory bowel diseases (Crohn's disease and ulcerative rectocolitis) have extraintestinal manifestations 25% of the patients, with the most common one being the enteropathic arthritis. METHODS: Prospective, observational, multicenter study with patients from 29 reference centers participating in the Brazilian Registry of Spondyloarthritis (RBE), which incorporates the RESPONDIA (Ibero-American Registry of Spondyloarthritis) group. Demographic and clinical data were collected from 1472 patients and standardized questionnaires for the assessment of axial mobility, quality of life, enthesitic involvement, disease activity and functional capacity were applied. Laboratory and radiographic examinations were performed. The aim of this study is to compare the clinical, epidemiological, genetic, imaging, treatment and prognosis characteristics of patients with enteropathic arthritis with other types of spondyloarthritis in a large Brazilian cohort. RESULTS: A total of 3.2% of patients were classified as having enteroarthritis, 2.5% had spondylitis and 0.7%, arthritis (peripheral predominance). The subgroup of individuals with enteroarthritis had a higher prevalence in women (P < 0.001), lower incidence of inflammatory axial pain (P < 0.001) and enthesitis (P = 0.004). HLA-B27 was less frequent in the group with enteroarthritis (P = 0.001), even when considering only those with the pure axial form. There was a lower prevalence of radiographic sacroiliitis (P = 0.009) and lower radiographic score (BASRI) (P = 0.006) when compared to patients with other types of spondyloarthritis. They also used more corticosteroids (P < 0.001) and sulfasalazine (P < 0.001) and less non-steroidal anti-inflammatory drugs (P < 0.001) and methotrexate (P = 0.001). CONCLUSION: There were differences between patients with enteroarthritis and other types of spondyloarthritis, especially higher prevalence of females, lower frequency of HLA-B27, associated with less severe axial involvement.

Effect of enthesitis on 1505 Brazilian patients with spondyloarthritis.

OBJECTIVE: To analyze the clinical effect of enthesitis in a large Brazilian cohort of patients with spondyloarthritis (SpA). METHODS: A common protocol of investigation was prospectively applied to 1505 patients with SpA in 29 centers in Brazil. Clinical and demographic variables and disease indexes were investigated. The Maastricht Ankylosing Spondylitis Enthesitis Score was used to investigate the enthesitis component. Ankylosing spondylitis was the most frequent disease in the group (65.4%). Others were psoriatic arthritis (18.4%), undifferentiated SpA (6.7%), reactive arthritis (3.3%), and enteropathic arthritis (3.2%). RESULTS: At least 1 affected enthesis was observed in 54% of the patients with SpA, with a mean of 2.12 ± 2.98 entheses affected. According to the clinical presentation, enthesitis was significantly more frequent in patients with axial + peripheral joint involvement compared to isolated axial or peripheral involvement (p < 0.001). There was a statistical association between the presence of enthesites and axial symptoms (buttock pain, cervical pain, and hip pain), and peripheral symptoms (lower limb arthritis, number of painful and swollen joints; p < 0.05). Patients with enthesitis also presented higher mean scores of Bath Ankylosing Spondylitis Functional Index (BASFI; p < 0.001), Bath Ankylosing Spondylitis Disease Activity Index (p < 0.001), and Ankylosing Spondylitis Quality of Life (ASQoL; p < 0.001). Multivariate logistic regression showed that BASFI (p < 0.0001; OR 74.839), ASQoL (p = 0.0001; OR 14.645), and Achilles tendonitis (p = 0.0059; OR 7.593) were associated with work incapacity. CONCLUSION: The clinical presence of enthesitis in this large cohort of patients with SpA was frequent and was associated with a significant increase in disease activity and decline in functional capacity and quality of life.

Enteropathic arthritis in Brazil: data from the Brazilian Registry of Spondyloarthritis.

UNLABELLED: Inflammatory bowel diseases (Crohn's disease and ulcerative rectocolitis) have extraintestinal manifestations 25% of the patients, with the most common one being the enteropathic arthritis. METHODS: Prospective, observational, multicenter study with patients from 29 reference centers participating in the Brazilian Registry of Spondyloarthritis (RBE), which incorporates the RESPONDIA (Ibero-American Registry of Spondyloarthritis) group. Demographic and clinical data were collected from 1472 patients and standardized questionnaires for the assessment of axial mobility, quality of life, enthesitic involvement, disease activity and functional capacity were applied. Laboratory and radiographic examinations were performed. The aim of this study is to compare the clinical, epidemiological, genetic, imaging, treatment and prognosis characteristics of patients with enteropathic arthritis with other types of spondyloarthritis in a large Brazilian cohort. RESULTS: A total of 3.2% of patients were classified as having enteroarthritis, 2.5% had spondylitis and 0.7%, arthritis (peripheral predominance). The subgroup of individuals with enteroarthritis had a higher prevalence in women (P < 0.001), lower incidence of inflammatory axial pain (P < 0.001) and enthesitis (P = 0.004). HLA-B27 was less frequent in the group with enteroarthritis (P = 0.001), even when considering only those with the pure axial form. There was a lower prevalence of radiographic sacroiliitis (P = 0.009) and lower radiographic score (BASRI) (P = 0.006) when compared to patients with other types of spondyloarthritis. They also used more corticosteroids (P < 0.001) and sulfasalazine (P < 0.001) and less non-steroidal anti-inflammatory drugs (P < 0.001) and methotrexate (P = 0.001). CONCLUSION: There were differences between patients with enteroarthritis and other types of spondyloarthritis, especially higher prevalence of females, lower frequency of HLA-B27, associated with less severe axial involvement.