Abstract Purpose: To evaluate the quality of referrals for a first Rheumatology consultation at a tertiary care center in a southern Brazilian capital (Porto Alegre, RS), having as background findings from a similar survey performed in 2007/2008. Since then, our state has implemented referral protocols and a triage system with teleconsulting support exclusively for referrals from locations outside the capital, permitting a comparison between patients screened and not screened by the new system. Methods: Physicians of the Rheumatology Service at Hospital Nossa Senhora da Conceição prospectively collected information regarding first visits over a 6-month period (Oct 2017 to March 2018). We recorded demographic characteristics, diagnostic hypotheses, date of referral, and the municipality of origin (within the state of Rio Grande do Sul). We considered adequate referrals from primary health care when a systemic autoimmune inflammatory disease (SIRD) was suspected at first evaluation by the attending rheumatologist. Results: Three hundred fifty-seven patients/appointments were eligible for analysis (193 from the capital and 164 from small and medium towns). In 2007/2008, suspected SIRD occurred in 76/260 (29.2%) and 73/222 (32.9%) among patients from the capital and outside counties, respectively (P = 0.387). In 2017/2018, suspected SIRD occurred in 75/193 (38.9%) and 111/164 (67.7%) in patients from the capital and outside counties, respectively (difference: 28.8, 95% CI: 19.0 to 38.9, P < 0.001), indicating a marked improvement in referrals submitted to the new triage system. Conclusion: The quality of Rheumatology referrals in our state improved over the 10-year interval under study, particularly among patients from locations submitted to referral protocols and teleconsulting support.

OBJECTIVE: To describe and analyze a new protocol for the extraction of platelet-rich plasma (PRP) for use in clinical practice and compare this technique with methods that have been previously described in the medical literature. METHODS: We extracted PRP from 20 volunteers using four different protocols (single spin at 1600 ×g, single spin at 600 ×g, double spin at 300 and 700 ×g, and double spin at 600 and 900 ×g). In another group of 12 individuals, we extracted PRP with our new technique (named ‘turn down-turn up’) consisting of a double spin (200 ×g and 1600 ×g) closed system using standard laboratory equipment (including an ordinary benchtop centrifuge), where the blood remained in the same tube during all processes, reducing the risk of contamination. Platelet counts adjusted to baseline values were compared using analysis of covariance (ANCOVA). RESULTS: Using the four previously described protocols (mentioned above), we obtained concentrations of platelets that were 1.15-, 2.07-, 2.18-, and 3.19-fold greater than the baseline concentration, respectively. With the turn down-turn up technique, we obtained a platelet count that was 4.17-fold (95% confidence interval (CI): 3.09 to 5.25) greater than the baseline platelet count (p=0.063 compared with the double spin at 600 and 900 ×g method). The total cost of the disposable materials used in the extraction process was less than US$10.00 per individual. CONCLUSION: In the present study, we described a simple and safe method for obtaining PRP using low-cost devices.

Abstract Background: Imbalance and disfuntion in regulatory T-cells (Tregs) and IL-17 producer lymphocytes (Th17) have been implicated in the pathogenesis of rheumatoid arthritis (RA). Gray scale synovial proliferation (GS), power Doppler signal (pD) and bone erosions seen on high resolution muskuloskeletal ultrasound (MSUS) are hallmarks of destructive articular disease. Objective: To evaluate the association of peripheral Tregs and Th17 with MSUS findings in RA. Methods: RA patients (1987 ACR criteria) treated with disease-modifying antirheumatic drugs (DMARDs) were included. Lymphocytes were isolated and immunophenotyped by flow cytometry to investigate regulatory FoxP3+ T cells and IL-17+ cells. MSUS (MyLab 60, Esaote, Genova, Italy, linear probe 6-18 MHz) was performed on hand joints, and a 10-joint US score was calculated for each patient. Results: Data on lymphocytes subsets were avaiable for 90 patients. The majority of patients were Caucasian women with a median disease duration of 6 years (interquartile range: 2-13 years). Mean DAS28 was 4.28 (SD ± 1.64) and mean HAQ score was 1.11 (SD ± 0.83). There was no significant correlation of 10-joint GS score (rS = 0.122, 95% CI: - 0.124 to 0.336, P = 0.254) and 10-joint pD score (rS = 0.056, 95% CI: - 0.180 to 0.273, P = 0.602) with the mean percentage of peripheral Treg cells. Also, 10-joint GS score (rS = 0.083, 95% CI: - 0.125 to 0.302, P = 0.438) and 10-joint pD score 10 (rS = - 0.060, 95% CI: - 0.271 to 0.150, P = 0.575); did not correlate to Th17 profile. No association of bone erosions on MSUS with Treg and Th17 profiles (P = 0.831 and P = 0.632, respectively) was observed. Conclusion: In this first study addressing MSUS features and lymphocytes subtypes in established RA, data did not support an association of circulating Tregs and Th17 lymphocytes with inflammatory and structural damage findings on MSUS.

Abstract Nailfold capillaroscopy (NFC) is a reproducible, simple, low-cost, and safe imaging technique used for morphological analysis of nail bed capillaries. It is considered to be extremely useful for the investigation of Raynaud's phenomenon and for the early diagnosis of systemic sclerosis (SSc). The capillaroscopic pattern typically associated with SSc, scleroderma (“SD”) pattern, is characterized by dilated capillaries, microhemorrhages, avascular areas and/or capillary loss, and distortion of the capillary architecture. The aim of these recommendations is to provide orientation regarding the relevance of NFC, and to establish a consensus on the indications, nomenclature, the interpretation of NFC findings and the technical equipments that should be used. These recommendations were formulated based on a systematic literature review of studies included in the database MEDLINE (PubMed) without any time restriction.

INTRODUÇÃO: Estudos têm relatado um aumento da expressão das células natural killer (NK) no sangue periférico de pacientes com esclerose sistêmica (ES). Essas células fazem parte da imunidade inata, reconhecendo células infectadas por meio dos receptores killer immunoglobulin-like receptor (KIR), que apresentam acentuado polimorfismo. Um novo modelo foi proposto prevendo a atividade das células NK, avaliando o excesso de ativação (EA), excesso de inibição (EI) ou se a célula está funcionalmente em equilíbrio (balance, B) (neutra). OBJETIVO: Avaliar a atividade das células NK em pacientes com ES e comparar com grupo-controle. MÉTODO: Cento e dez pacientes com ES e 115 controles foram estudados. Foi aplicado um novo modelo que prevê a atividade das células NK. Para esse método, considerou-se cada célula com seu respectivo ligante KIR/HLA-C e Bw4. A nomenclatura utilizada foi EA, EI e B. RESULTADOS: Nossos resultados mostraram que 63,5% dos controles saudáveis apresentavam o fenótipo KIR caracterizado por EI, em comparação com 39,1% dos pacientes com ES (P = 0,001). Considerando-se somente indivíduos com presença de KIR2DL2 (KIR2DL2+), encontramos 34,7% de EI em controles sadios e 10,9% em pacientes com ES (P < 0,001). CONCLUSÃO: Em nosso estudo, o modelo que prevê a ação das células NK mostrou que controles sadios têm maior frequência de EI quando comparados a pacientes com ES, sugerindo um efeito protetor do EI contra o desenvolvimento da ES. Outros estudos, porém, devem ser realizados para confirmar nossos dados.

INTRODUCTION: Previous studies have shown an increased expression of natural killer (NK) cells in the peripheral blood of patients with systemic sclerosis (SSc). NK cells are part of innate immunity, recognizing infected cells through killer immunoglobulin-like receptors (KIR), which show marked polymorphism. A novel model has been proposed predicting the activity of NK cells, evaluating whether there is excessive activation (EA), excessive inhibition (EI) or balance (B) (neutral). OBJECTIVE: To evaluate the activity of NK cells in patients with SSc and compare it with that of a control group. METHOD: This study comprised 110 patients with SSc and 115 healthy controls. A novel model that predicts the activity of NK cells was used. For that, cells with their respective KIR/HLA-C and Bw4 ligands were considered. The activity of NK cells was defined as EA, EI, or B. RESULTS: Our results showed that 63.5% of healthy controls had the KIR phenotype characterized by EI, as compared with 39.1% of the patients with SSc (P = 0.001). Considering only KIR2DL2-positive individuals, 34.7% of EI was found in healthy controls and 10.9% in patients with SSc (P < 0.001). CONCLUSION: In our study, the model that predicts the action of NK cells showed that healthy controls have higher frequency of EI as compared with SSc patients, suggesting a protective effect of the EI profile against the development of SSc. These results suggest a potential role of NK cells in the pathogenesis of SSc, but further studies should be conducted to confirm our data.

OBJETIVO: avaliar o estado nutricional na artrite idiopática juvenil (AIJ), a influência da atividade inflamatória e o uso de glicocorticóide. MÉTODOS: foram estudados 116 pacientes com AIJ diagnosticados segundo os critérios da ILAR/1997. Subtipo e atividade da doença foram determinados por reumatologistas pediátricos, e a dose cumulativa de glicocorticóide foi revisada nos prontuários dos pacientes. Foram determinados os percentis do índice de massa corporal (IMC) e da prega cutânea tricipital (PCT) e o escore Z da estatura, de acordo com a OMS. Considerou-se baixo peso e baixa adiposidade quando o IMC e a PCT apresentavam-se abaixo do percentil 5. Baixa estatura foi definida por escore Z de estatura para idade menor do que -2. O nível sérico de IGF-1 foi medido por meio de radioimunoensaio. RESULTADOS: as prevalências de baixo peso, baixa adiposidade e baixa estatura foram 16,4%, 20,7% e 10,4%, respectivamente. Níveis séricos reduzidos de IGF-1 foram observados em 14 pacientes (12,1%). Os fatores negativamente associados com o escore Z da estatura na análise de regressão multivariável foram: duração da doença (coeficiente parcial de correlação, intervalo de confiança de 95%: -0,370, -0,527 a -0,188: p< 0,001), velocidade de hemossedimentação (VHS) (-0,357, -0,516 a -0,174; p< 0,001), subtipo da doença sistêmico ou poliarticular (-0,290, -0,459 a -0,100; p= 0,003), enquanto não houve associação significante com dose cumulativa de glicocorticóide (0,086, -0,111 a 0,277: p= 0,391). Nenhuma dessas variáveis foi significativamente associada com os percentis de IMC e PCT, porém, pacientes com os subtipos sistêmico e poliarticular tenderam a apresentar percentis de IMC mais baixos (p= 0,051). CONCLUSÕES: pacientes com AIJ apresentam freqüentemente comprometimento do estado nutricional. A duração e subtipo da doença e o VSG são fatores independentemente associados à baixa estatura. A dose cumulativa de glicocorticóide não esteve associada de maneira independente com a baixa estatura ou a outras variáveis nutricionais.

OBJECTIVE: To assess the nutritional status in juvenile idiopathic arthritis (JIA) and the influence of inflammatory activity and glucocorticoid use. METHODS: One hundred and sixteen patients were evaluated. Disease subtype and disease activity were defined by the attending physician, and the cumulative glucocorticoid dose was recorded by chart review. The percentiles of body mass index (BMI) and triceps skinfold (TSF) and the Z-score for height were determined: low weight and low adiposity were diagnosed when BMI and TSF were below the 5th percentile. Short stature was defined by a Z-score of height for age < -2. The serum level of IGF-I was measured by radioimmunoassay. RESULTS: The prevalences of low weight, low adiposity and short stature were 16.4%, 20.7% and 10.4%, respectively. Low IGF-1 serum level was found in 14 patients (12.1%). The factors negatively associated with the Z-score of height in multivariable regression analysis were disease duration (partial correlation coefficient, 95% confidence interval: -0.370, -0.527 to -0.188; p < 0.001), erythrocyte sedimentation rate (ESR) (-0.357, -0.516 to -0.174; p < 0.001), polyarticular or systemic subtype (-0.290, -0.459 to -0.100; p = 0.003), while there was no significant association with the cumulative dose of glucocorticoids (0.086, -0.111 to 0.277; p = 0.391). None of these variables were significantly associated with the percentiles of BMI or TSF, but patients with a systemic or polyarticular subtype tended to present lower percentiles of BMI (p = 0.051). CONCLUSIONS: Nutritional status is frequently compromised in JIA. The duration and subtype of the disease and the ESR are factors independently associated with short stature. The cumulative dose of glucocorticoids was not independently associated with short stature or other nutricional variables.

OBJETIVO: Estudar a associação entre alterações capilaroscópicas e lesões em órgãos-alvo na esclerose sistêmica (ES). MÉTODOS: Noventa e um pacientes com ES foram avaliados através de entrevista, exame físico, capilaroscopia periungueal (CPU), sorologia, provas de função pulmonar, cintilografia de trânsito esofágico, ecocardiografia com Doppler e tomografia computadorizada de alta resolução pulmonar (TCAR). A deleção capilar na CPU foi avaliada usando o escore de deleção vascular de Lee; consideraram-se alterações severas na CPU um escore médio de deleção >1 ou número médio de megacapilares por dedo >1 para fins de análise. Hipertensão arterial pulmonar (HAP) foi definida como pressão sistólica na artéria pulmonar >40 mmHg. RESULTADOS: Pacientes com alterações capilaroscópicas severas apresentaram maior prevalência de áreas de opacidades em vidro-fosco (OVF) (P=0,016), redução da capacidade difusional pulmonar (P=0,026) e disfunção esofágica (P=0,001). HAP ocorreu somente em pacientes com alterações severas na CPU (P=0,114). Naqueles com duração de doença <5 anos, OVF estavam presentes em 14 de 19 pacientes com alterações severas na CPU, mas não estavam presentes nos 8 pacientes com alterações capilaroscópicas leves ou ausentes (P<0,001). Nesse subgrupo, nenhuma outra variável clínica ou laboratorial associou-se à presença de OVF. O uso de curvas ROC mostrou uma boa capacidade da CPU em discriminar pacientes com e sem o desfecho combinado de HAP ou doença intersticial pulmonar em pacientes com duração de doença < 5 anos. CONCLUSÕES: A severidade das anormalidades da CPU está associada com dano em órgãos-alvo na ES. A CPU pode indicar a presença de doença pulmonar ativa (representada por áreas de opacidades em vidro-fosco na TCAR) na ES de duração relativamente curta.

OBJECTIVE: To evaluate the association of capillaroscopic alterations with end-organ damage in systemic sclerosis (SSc). METHODS: Ninety-one SSc patients were studied through interview, physical examination, nailfold capillary microscopy (NCM), serology, pulmonary function tests, esophageal transit scintigraphy, Doppler echocardiography and pulmonary highresolution computed tomography (HRCT). Capillary loss on NCM was evaluated using the avascular score (AS); patients with mean AS =1 or mean number of megacapillaries per finger =1 were considered to have severe capillaroscopic alterations for analytical purposes. Pulmonary arterial hypertension (PAH) was defined as systolic pulmonary arterial pressure =40 mmHg. RESULTS: Patients with severe NCM alterations had greater prevalence of ground-glass opacities (GGO) areas (P=0.016), reduced pulmonary diffusing capacity (P=0.026) and esophageal dysfunction (P=0.001). PAH was present only in patients with severe NCM abnormalities (P=0.114). In those with disease duration =5 years, GGO were present in 14 of 19 patients with severe NCM alterations, but were absent in all patients (n=8) with no or mild NCM alterations (P<0.001). In this subgroup, no other clinical or laboratory variable was associated with the presence of GGO. ROC curves showed a good ability of NCM to discriminate between patients with and without the combined end-point of PAH or interstitial disease in patients with disease duration =5 years. CONCLUSIONS: The severity of NCM abnormalities is associated with end-organ damage in SSc. NCM may indicate the presence of active pulmonary disease (represented by ground-glass opacities areas in HRCT) in SSc of relatively short duration.