ABSTRACT Objective: To evaluate autoinflammatory diseases (AID) according to age at diagnosis and sex, and response to therapy in a large population. Methods: This is a cross-sectional observational study of a Latin American registry using a designed web system for data storage, collected between 2015 and 2018. Any altered findings during follow-up were recorded. The forms were translated into Portuguese and Spanish, including demographic, clinical, laboratory, genetic and treatment characteristics. Results: We included 152 patients, 51.3% male and 75% Caucasian. The median age at disease onset was 2.1 years (0–15.6 years) and median age at diagnosis 6.9 years (0–21.9 years); 111 (73%) were children (0–9 years old), and 41 (27%) were adolescents and young adults (AYA) (10–21 years old). Periodic fever, aphthous stomatitis, pharyngitis, and adenitis syndrome (PFAPA) occurred in 46/152 (30%), chronic non-bacterial osteomyelitis (CNO) in 32/152 (21%), and familial Mediterranean fever (FMF) in 24/152 (15.7%). PFAPA was significantly higher in young children than in AYA (38.7% vs. 7.3%, p<0.001), while CNO were lower (13.5% vs. 41.5%, p<0.001). The frequency of females was significantly higher in CNO (28.4% vs. 14.1%, p=0.031) and lower in FMF (8.1% vs. 23.1%, p=0.011). The most used drugs were glucocorticoids, non-steroidal anti-inflammatory drugs (NSAID), and colchicine. Glucocorticoids and colchicine treatment were used in all AID with good to moderate response. However, cryopyrin-associated periodic syndromes (CAPS) seemed unresponsive to glucocorticoids. NSAIDs and methotrexate were the main medications used to treat CNO. Conclusions: Differences among AID patients were observed in the LA population regarding sex and age at disease diagnosis. Objective (AID Methods crosssectional cross sectional storage 201 2018 followup follow up recorded Spanish demographic clinical laboratory characteristics Results 15 513 51 3 51.3 75 Caucasian 21 2 1 2. 0–15.6 0156 0 6 (0–15. 69 9 6. 0–21.9 0219 (0–21. 11 73% 73 (73% 0–9 09 (0– old, old , old) 4 27% 27 (27% (AYA 10–21 1021 10 (10–2 old. . stomatitis pharyngitis (PFAPA 46152 46 46/15 30%, 30 30% (30%) nonbacterial non bacterial (CNO 32152 32 32/15 21%, 21% (21%) (FMF 24152 24 24/15 15.7%. 157 15.7% 7 (15.7%) 38.7% 387 38 (38.7 vs 7.3% p<0.001, p0001 p p<0.001 001 p<0.001) 13.5% 135 13 5 (13.5 415 41.5% p<0.001. 28.4% 284 28 (28.4 141 14 14.1% p=0.031 p0031 031 8.1% 81 8 (8.1 231 23 23.1% p=0.011. p0011 p=0.011 011 p=0.011) glucocorticoids nonsteroidal steroidal antiinflammatory anti inflammatory NSAID, NSAID (NSAID) However cryopyrinassociated cryopyrin associated CAPS (CAPS Conclusions 20 51. 0–15. 015 (0–15 0–21. 021 (0–21 (73 0– (0 (27 10–2 102 (10– 4615 46/1 (30% 3215 32/1 (21% 2415 24/1 15.7 (15.7% 38.7 (38. 7.3 p000 p<0.00 00 13.5 (13. 41.5 28.4 (28. 14.1 p=0.03 p003 03 8.1 (8. 23.1 p001 p=0.01 01 (NSAID 0–15 (0–1 0–21 02 (0–2 (7 ( (2 10– (10 461 46/ (30 321 32/ (21 241 24/ 15. (15.7 38. (38 7. p00 p<0.0 13. (13 41. 28. (28 14. p=0.0 8. (8 23. 0–1 0–2 (1 (3 (15. p0 p<0. p=0. (15 p<0 p=0 p< p=

RESUMO Objetivo: Avaliar as doenças autoinflamatórias (DAI) de acordo com sexo e idade no momento do diagnóstico e a resposta terapêutica em uma grande população. Métodos: Este é um estudo observacional transversal de um registro latino-americano que usou um sistema de dados coletados entre 2015 e 2018. Quaisquer achados alterados ao longo do acompanhamento foram registrados. Os formulários foram traduzidos para os idiomas português e espanhol, incluindo características demográficas, clínicas, laboratoriais, genéticas e de tratamento. Resultados: Incluímos 152 pacientes, sendo 51,3% do sexo masculino e 75% da raça branca. A média de idade de início da doença foi de 2,1 anos (0–15,6 anos) e a média de idade de diagnóstico 6,9 anos (0–21,9 anos); 111 (73%) eram crianças (0–9 anos) e 41 (27%) adolescentes/adultos jovens (10–21 anos). A síndrome de febre periódica, estomatite aftosa, faringite e adenite (PFAPA) ocorreu em 46/152 (30%), osteomielite não bacteriana crônica (CNO) em 32/152 (21%) e febre familiar do Mediterrâneo (FMF) em 24/152 (15,7%). A PFAPA foi significativamente maior em crianças pequenas (38,7 vs. 7,3%, p<0,001), e a CNO, em adolescentes/adultos jovens (13,5 vs. 41,5%, p<0,001). A frequência do sexo feminino foi significativamente maior na CNO (28,4 vs. 14,1%, p=0,031) e menor na FMF (8,1 vs. 23,1%, p=0,011). Os medicamentos mais utilizados foram glicocorticoides, anti-inflamatórios não esteroidais (AINE) e colchicina. O tratamento com glicocorticoides e colchicina foi usado em todas as DAI com resposta boa a moderada. No entanto, as síndromes periódicas associadas à criopirina (CAPS) pareciam não responder aos glicocorticoides. AINE e metotrexato foram os principais medicamentos utilizados no tratamento da CNO. Conclusões: Diferenças de pacientes com DAI foram observadas na população latino-americana em pacientes agrupados por sexo e idade ao diagnóstico da doença. Objetivo (DAI Métodos latinoamericano latino americano 201 2018 registrados espanhol demográficas clínicas laboratoriais Resultados 15 513 51 3 51,3 75 branca 21 2 1 2, 0–15,6 0156 0 6 (0–15, 69 9 6, 0–21,9 0219 (0–21, 11 73% 73 (73% 0–9 09 (0– 4 27% 27 (27% adolescentesadultos adolescentes adultos 10–21 1021 10 (10–2 anos. . periódica aftosa (PFAPA 46152 46 46/15 30%, 30 30% , (30%) (CNO 32152 32 32/15 21% (21% (FMF 24152 24 24/15 15,7%. 157 15,7% 7 (15,7%) 38,7 387 38 (38, vs 7,3% p<0,001, p0001 p p<0,001 001 p<0,001) 13,5 135 13 5 (13, 415 41,5% p<0,001. 28,4 284 28 (28, 141 14 14,1% p=0,031 p0031 031 8,1 81 8 (8, 231 23 23,1% p=0,011. p0011 p=0,011 011 p=0,011) antiinflamatórios anti inflamatórios (AINE moderada entanto CAPS (CAPS Conclusões latinoamericana americana 20 51, 0–15, 015 (0–15 0–21, 021 (0–21 (73 0– (0 (27 10–2 102 (10– 4615 46/1 (30% 3215 32/1 (21 2415 24/1 15,7 (15,7% 38, (38 7,3 p000 p<0,00 00 13, (13 41,5 28, (28 14,1 p=0,03 p003 03 8, (8 23,1 p001 p=0,01 01 0–15 (0–1 0–21 02 (0–2 (7 ( (2 10– (10 461 46/ (30 321 32/ 241 24/ 15, (15,7 (3 7, p00 p<0,0 (1 41, 14, p=0,0 23, 0–1 0–2 (15, p0 p<0, p=0, (15 p<0 p=0 p< p=

Abstract Background Increased malignancy frequency is well documented in adult-systemic lupus erythematosus (SLE), but with limited reports in childhood-onset SLE (cSLE) series. We explored the frequency of malignancy associated with cSLE, describing clinical and demographic characteristics, disease activity and cumulative damage, by the time of malignancy diagnosis. Method A retrospective case-notes review, in a nationwide cohort from 27 Pediatric Rheumatology centres, with descriptive biopsy-proven malignancy, disease activity/damage accrual, and immunosuppressive treatment were compiled in each participating centre, using a standard protocol. Results Of the 1757 cSLE cases in the updated cohort, 12 (0.7%) developed malignancy with median time 10 years after cSLE diagnosis. There were 91% females, median age at cSLE diagnosis 12 years, median age at malignancy diagnosis 23 years. Of all diagnosed malignancies, 11 were single-site, and a single case with concomitant multiple sites; four had haematological (0.22%) and 8 solid malignancy (0.45%). Median (min–max) SLEDAI-2 K scores were 9 (0–38), median (min–max) SLICC/ACR-DI (SDI) score were 1 (1–5) Histopathology defined 1 Hodgkin's lymphoma, 2 non-Hodgkin's lymphoma, 1 acute lymphoblastic leukaemia; 4 gastrointestinal carcinoma, 1 squamous cell carcinoma of the tongue and 1 anal carcinoma; 1 had sigmoid adenocarcinoma and 1 stomach carcinoid; 3 had genital malignancy, being 1 vulvae, 1 cervix and 1 vulvae and cervix carcinomas; 1 had central nervous system oligodendroglioma; and 1 testicle germ cell teratoma. Conclusion Estimated malignancy frequency of 0.7% was reported during cSLE follow up in a multicentric series. Median disease activity and cumulative damage scores, by the time of malignancy diagnoses, were high; considering that reported in adult series. adultsystemic systemic SLE, , (SLE) childhoodonset childhood onset (cSLE series characteristics casenotes notes review centres biopsyproven biopsy proven activitydamage accrual centre protocol 175 07 0 7 (0.7% 91 females malignancies singlesite, singlesite site, site single-site sites 0.22% 022 22 (0.22% 0.45%. 045 0.45% . 45 (0.45%) min–max minmax min max (min–max SLEDAI2 SLEDAI SLEDAI- 0–38, 038 0–38 38 (0–38) SLICC/ACRDI SLICCACRDI SLICC/ACR DI SLICC ACR SDI (SDI 1–5 15 5 (1–5 Hodgkins Hodgkin s lymphoma nonHodgkins non leukaemia carcinoid carcinomas oligodendroglioma teratoma 0.7 diagnoses high (SLE 17 (0.7 0.22 02 (0.22 04 0.45 (0.45% 03 0–3 (0–38 ACRDI SLICCACR 1– (1– 0. (0. 0.2 (0.2 0.4 (0.45 0– (0–3 (1 (0 (0.4 (0– (

ABSTRACT Objective: To describe the clinical features and outcomes of patients with uveitis associated with juvenile idiopathic arthritis (JIA) and idiopathic uveitis. Methods: This was an observational, retrospective study, conducted in a tertiary center. Patients under 18 years old who experienced at least one episode of uveitis and followed between 2000 and 2019 were included. Results: A total of 82 patients were included, of whom 43 had idiopathic uveitis and 39 had uveitis associated with JIA. Anterior uveitis was the primary site of ocular inflammation (76.8%) and occurred in 24 and 39 patients with idiopathic uveitis and uveitis associated with JIA arthritis, respectively (p=0.02). The complete response to corticotherapy was more frequent in uveitis associated with JIA (p=0.001). Total and partial responses to biological disease modifying antirheumatic drugs were more frequent in uveitis associated with JIA (p=0.025) and idiopathic uveitis (p=0.045), respectively. There were 203 complications: cataracts were more frequently present in idiopathic uveitis (p=0.05), while synechiae was more frequent in uveitis associated with JIA (p=0.02). Conclusion: Idiopathic uveitis and uveitis associated JIA frequently follow a chronic course and an increased risk of visual loss in childhood. The uveitis associated with JIA showed better response to systemic corticotherapy and total response to biologic disease modifying antirheumatic drugs more frequently. Objective (JIA Methods observational study center 1 200 201 included Results 8 4 3 76.8% 768 76 (76.8% 2 p=0.02. p002 p p=0.02 . 0 02 (p=0.02) p=0.001. p0001 p=0.001 001 (p=0.001) p=0.025 p0025 025 (p=0.025 p=0.045, p0045 p=0.045 , 045 (p=0.045) 20 complications p=0.05, p005 p=0.05 05 (p=0.05) Conclusion childhood 76.8 7 (76.8 p00 p=0.0 (p=0.02 p000 p=0.00 00 (p=0.001 p004 p=0.04 04 (p=0.045 (p=0.05 76. (76. p0 p=0. (p=0.0 (p=0.00 (p=0.04 (76 p=0 (p=0. (7 p= (p=0 ( (p= (p

RESUMO Objetivos: Descrever as características clínicas e desfechos dos pacientes com uveíte associada à Artrite Idiopática Juvenil (AIJ) e da Uveíte Idiopática. Métodos: Este foi um estudo retrospectivo observacional conduzido em um centro terciário. Foram incluídos pacientes abaixo dos 18 anos de idade que apresentaram pelo menos um episódio de uveíte e que estiveram em acompanhamento médico entre os anos de 2000 e 2019. Resultados: Foram incluídos 82 pacientes, sendo 43 com uveíte idiopática e 39 com uveíte associada à AIJ. A uveíte anterior foi o sítio primário de acometimento (76,8%) em 24 e 39 pacientes com uveíte idiopática e uveíte associada à AIJ, respectivamente (p=0.02). Resposta total à corticoterapia foi mais frequente na uveíte associada à AIJ (p=0.001). Respostas total e parcial às drogas antirreumáticas modificadoras de doença biológicas foram mais frequentes na uveíte associada à AIJ (p=0.025) e na uveíte idiopática (p=0.045), respectivamente. Foram encontradas 203 complicações: a catarata foi mais frequente na uveíte idiopática (p=0.05), enquanto a sinéquia foi mais frequente na uveíte associada à AIJ (p=0.02). Conclusão: A uveíte idiopática e a uveíte associada à AIJ frequentemente apresentam um curso crônico e um risco elevado de perda visual na infância. A uveíte associada à AIJ apresentou melhor resposta à corticoterapia sistêmica e resposta total às drogas modificadoras de doença reumática biológicas mais frequentemente. Objetivos (AIJ Métodos terciário 1 200 2019 Resultados 8 4 3 76,8% 768 76 (76,8% 2 p=0.02. p002 p p=0.02 . 0 02 (p=0.02) p=0.001. p0001 p=0.001 001 (p=0.001) p=0.025 p0025 025 (p=0.025 p=0.045, p0045 p=0.045 , 045 (p=0.045) 20 complicações p=0.05, p005 p=0.05 05 (p=0.05) Conclusão infância 201 76,8 7 (76,8 p00 p=0.0 (p=0.02 p000 p=0.00 00 (p=0.001 p004 p=0.04 04 (p=0.045 (p=0.05 76, (76, p0 p=0. (p=0.0 (p=0.00 (p=0.04 (76 p=0 (p=0. (7 p= (p=0 ( (p= (p

Abstract Objectives Adherence problems have a great impact on auto-immune Rheumatic Diseases (AIRD). The COVID-19 pandemic may have worsened treatment adherence. The aims of this study were to measure treatment adherence to identify an earlier risk of poor adherence and measure families’ satisfaction with the health service during the pandemic. Methods Prospective observational study with 50 parents/children and adolescents with recent AIRD diagnosis. Initially, they answered questions (demographic data, disease) and completed the Pediatric Rheumatology Adherence Questionnaire (PRAQ), after 6 months they completed the Morisky-Green Test (MGT), Brief Medication Questionnaire (BMQ), Compliance Questionnaire for Rheumatology (CQR-19) and Pediatric Quality of Life Inventory Questionnaire 3.0 (PedsQlTM-SSS). The patient's medical records from the previous 12 weeks were reviewed for global and medication adherence data. Results The mean global adherence score was 94.3 ± 10.0, for medication adherence 97.3 ± 9.3, and for PRAQ questionnaire 5.2 ± 1.5. The authors observed agreement between MGT, BMQ, CQR-19, PedsQLTM-SSS scores and medication adherence rate, but not with global adherence rate. There were no associations between demographic characteristics, disease diagnosis, and adherence. No associations between PRAQ scores and values and global/medication adherence rates were observed. No variables were shown to be predictors of good adherence. The mean PedsQLTM-SSS rate was 92.1 ± 6.8. Conclusion The high values of MGT, BMQ, CQR-19 questionnaire scores were in agreement with the medication adherence rate. Despite the pandemic, the global and medication adherence rates were good. It was not possible to demonstrate the PRAQ's predictive power. The authors weren't able to establish an association between families’ satisfaction and treatment adherence rates. autoimmune auto immune AIRD. . (AIRD) COVID19 COVID 19 COVID-1 families 5 parentschildren parents children diagnosis Initially data PRAQ, , (PRAQ) MoriskyGreen Morisky Green MGT (MGT) BMQ (BMQ) CQR19 CQR (CQR-19 30 3 0 3. PedsQlTMSSS. PedsQlTMSSS PedsQlTM SSS (PedsQlTM-SSS) patients patient s 1 943 94 94. 100 10 10.0 973 97 97. 93 9 9.3 52 2 5. 15 1.5 CQR19, 19, PedsQLTMSSS PedsQLTM characteristics globalmedication 921 92 92. 68 8 6.8 CQR-1 PRAQs power werent weren t (AIRD COVID1 COVID- (PRAQ (MGT (BMQ CQR1 (CQR-1 (PedsQlTM-SSS 10. 9. 1. 6. CQR- (CQR- (CQR

Abstract Objective Advances in medicine have increased the life expectancy of pediatric patients with chronic illnesses, and challenges with the guided transition of adolescents and young adults from pediatric clinics to adult clinics have grown. The aim of this study was to better understand readiness and factors related to this transition process in Brazil. Method In this cross-sectional study of 308 patients aged from 16 to 21 years under follow-up in pediatric specialties, the degree of readiness for transition was assessed using the Transition Readiness Assessment Questionnaire (TRAQ) and its domains. Associations with demographic data, clinical data, socio-economic level, medication adherence, family functionality, and parental satisfaction with health care were evaluated. Results The median TRAQ score was 3.7 (3.2 - 4.2). Better readiness was associated with female patients, socio-economic class A-B, current active employment, higher level of education, not failing any school year, attending medical appointments alone, functional family, and a good knowledge of disease and medications. A low correlation was observed between TRAQ and age. TRAQ presented good internal consistency (alpha-Cronbach 0.86). In the multiple linear regression, TRAQ score showed a significant association with female gender, advanced age, socio-economic class A-B, better knowledge of disease and medications, and independence to attend appointments alone. Conclusion TRAQ instrument can guide healthcare professionals to identify specific areas of approach, in order to support adolescents with chronic disease to set goals for their own personal development and improve their readiness to enter into the adult healthcare system. In this study, some factors were related to better TRAQ scores. illnesses grown Brazil crosssectional cross sectional 30 1 2 followup follow up specialties (TRAQ domains data socioeconomic socio economic adherence functionality evaluated 37 3 7 3. 3.2 32 (3. 4.2. 42 4.2 . 4 4.2) AB, AB B, B A-B employment education year alone medications age alphaCronbach alpha Cronbach 0.86. 086 0.86 0 86 0.86) regression gender approach system scores (3 4. 08 0.8 8 ( 0.

Abstract Objective To determine the frequency of radiographic changes in the temporomandibular joint, in a representative population of patients with Juvenile Idiopathic Arthritis (JIA) and to compare with findings in healthy controls matched by sex and age. Patients and Methods One hundred and thirty-seven panoramic radiographies (PR) from JIA patients of a pediatric rheumatology outpatient clinic were prospectively evaluated and compared to 137 PR from healthy individuals. Results 102 (74.5%) JIA patients and 47 (34.3%) controls showed at least one radiological alteration (p < 0.001). The following radiographic alterations were more frequently observed in JIA patients than in controls: erosion (p < 0.001), altered condylar morphology (p < 0.001), disproportion between condylar process and the coronoid process (p < 0.001) and accentuated curve in the antegonial notch (p = 0.002). Twenty patients (14.6%) presented the four radiographic alterations simultaneously compared to only two controls (1.5%) (p < 0.001). Conclusion Due to the difference in the frequency of findings in the PR of patients and controls, we concluded that PR has value as a screening tool. In the presence of major changes in the mandible head in the PR of patients with a confirmed diagnosis of JIA, MRI should be considered to detect an active inflammatory process in this joint. joint (JIA age thirtyseven thirty seven (PR 13 individuals 10 74.5% 745 74 5 (74.5% 4 34.3% 343 34 3 (34.3% p 0.001. 0001 0.001 . 0 001 0.001, , 0.002. 0002 0.002 002 0.002) 14.6% 146 14 6 (14.6% 1.5% 15 1 (1.5% tool 74.5 7 (74.5 34.3 (34.3 000 0.00 00 14.6 (14.6 1.5 (1.5 74. (74. 34. (34. 0.0 14. (14. 1. (1. (74 (34 0. (14 (1 (7 (3 (

Abstract Background Juvenile Dermatomyositis (JDM) is the most common idiopathic inflammatory myopathy in children. Imaging exams are useful for muscle assessment, with ultrasonography (US) being a promising tool in detecting disease activity and tissue damage. There are few studies about muscle elastography. Objectives Our aim was to associate clinical, laboratory, and nailfold capillaroscopy (NC) assessments with US in JDM patients; and to compare the findings of US and Strain Elastography (SE) from patients and healthy controls. Methods An analytic cross-sectional study was performed with JDM patients and healthy controls. Patients underwent clinical exam to access muscle strength and completed questionnaires about global assessment of the disease and functional capacity. Patients were submitted to NC and measurement of muscle enzymes. All subjects underwent US assessment, using gray scale, Power Doppler (PD), and SE. Results Twenty-two JDM patients and fourteen controls, aged between 5 and 21 years, matched for age and sex were assessed. In qualitative and semi-quantitative gray scale, we observed a higher frequency of alterations in patients (p < 0.001), while in PD, there was a higher frequency of positivity in patients’ deltoids and anterior tibialis (p < 0.001). Active disease was associated with an important change in the semi-quantitative gray scale in deltoids (p = 0.007), biceps brachii (p = 0.001) and quadriceps femoris (p = 0.005). The SE demonstrated a high negative predictive value of 87.2. Conclusion US was able, through gray scale, to differentiate JDM patients from controls, while PD achieved such differentiation only for deltoids and anterior tibialis. The semi-quantitative gray scale showed disease activity in proximal muscles. SE was not able to differentiate patients from controls. (JDM children (US damage elastography laboratory (NC (SE controls crosssectional cross sectional capacity enzymes , (PD) Twentytwo Twenty two 2 years assessed semiquantitative semi quantitative p 0.001, 0001 0.001 0 001 0.001. . 0.007, 0007 0.007 007 0.007) 0.005. 0005 0.005 005 0.005) 872 87 87.2 muscles (PD 000 0.00 00 8 87. 0.0 0.

Abstract Background: The observation that 2-deoxy-2[18F]fluoro-D-glucose-positron emission tomography/magnetic resonance imaging ([18F]F-FDG-PET/MRI) revealed high-grade arterial wall FDG uptake, without arterial wall thickening with contrast-enhancement, in a considerable number of c-TA patients in our previous study, encouraged us to compare patients with both PET and MR angiography (MRA) positives, with those with PET positive but MRA negative. Our aim was to evaluate the relevance of these two imaging modalities together. Methods: A three-center cross-sectional study with 17 patients who fulfilled the EULAR/PRINTO/PReS criteria for c-TA and who underwent [18F]F-FDG-PET/MRI was previously performed. Herein we compared patients/vessels with positive PET (arterial wall 18F-FDG uptake higher than liver) and positive MRA (arterial wall thickening with contrast-enhancement)—group 1, with those with positive PET but negative MRA—group 2. Results: Median disease duration of 17 c-TA patients was 10.4 years. Nine patients were classified as group 1 and six as group 2. Median of metabolic inflammatory volume (MIV) of all arterial segments was significantly higher in group 1 (2346 vs. 1177 cm3; p = 0.036). Fifty-four (19%) from 284 available arterial segments presented positive findings in vessel wall in one or both images. Positive findings were concordant between PET and MRA in only 13% arterial segments (group 1); most changes (28–59.6%) that were discordant between both images, were positive in PET and negative in MRA (group 2). Conclusions: Our study demonstrated that [18F]F-FDG-PET/MRI added information about inflammation in vessel wall of c-TA patients. Prospective multicenter studies are needed in order to get solid data to guide immunosuppressive tapering and withdrawal.

Resumo: Introdução: As relações entre o desempenho dos alunos nos exames de residência médica e testes de progresso e os estágios no internato médico não estão bem estabelecidas. Objetivo: Este estudo teve como objetivos medir as correlações entre as notas nos testes de progresso e as notas no internato e o resultado final do exame de residência médica, e determinar qual desempenho teve a maior correlação com o exame final da residência médica. Método: Trata-se de um estudo retrospectivo e longitudinal com análises de correlação de notas em provas de progresso do primeiro ao sexto ano do curso de Medicina, coeficiente de desempenho de estágios do internato (quinto e sexto anos) e notas do exame final de residência médica em uma coorte de alunos matriculados em uma Faculdade de Medicina de uma instituição pública federal, usando análise fatorial. Foram incluídos os alunos que realizaram os testes de progresso do primeiro ao sexto ano. Resultado: Dos 123 alunos matriculados no primeiro ano do curso de Medicina em 2009, 114 (92,7%) realizaram os testes de progresso durante os seis anos letivos e foram incluídos. As notas médias nos testes de progresso de 1 a 10 foram 2,67 (primeiro ano), 3,01 (segundo ano), 4,19 (terceiro ano), 4,01 (quarto ano), 5,19 (quinto ano) e 6,38 (sexto ano). As notas médias nos estágios foram 8,32 (quinto ano) e 8,26 (sexto ano). A nota média no exame teórico da residência médica foi 7,53; e a média no exame final da residência, 8,5. A análise fatorial detectou três domínios com maior força de correlação que responderam por 76,3% da variância do modelo. O componente 1 foi identificado como coeficiente de rendimento acadêmico (CAP) 5º, CAP 6º e o resultado final do exame de residência médica, o componente 2 foi formado pelas notas das provas de progresso do quinto e sextos anos, e o terceiro componente compreendeu as notas do progresso do segundo, terceiro e quarto anos. Conclusão: As notas das provas de progresso, as avaliações do internato e o exame final de residência médica apresentaram correlações significantes. Além disso, o desempenho durante o internato apresentou maior correlação com as notas do exame final de residência médica.

Abstract: Introduction: The relationships between the students’ performance on medical residency exams and progress tests and medical clerkship rotations are not well established. Objective: The objective of this study was to measure the correlations between grades on progress tests and clerkship rotations assessments and the medical residency exam and determine which performance had the strongest correlation with the final medical residency exam. Methods: This was a retrospective and longitudinal study with correlation analyses of grades on progress tests from the 1st to 6th year of medical school, the clerkship rotations performance coefficient (5th and 6th years of school) and the final medical residency exam in a cohort of students enrolled in a federal public medical school using factor analysis. Students who performed the progress tests from the 1st to 6th year were included. Results: Of 123 students enrolled in the first year of medical school in 2009, 114 (92.7%) performed the progress tests during the six years and were included. The average grades on the progress tests from 1 to 10 were 2.67 (1st year), 3.01 (2nd year), 4.19 (3rd year), 4.01 (4th year), 5.19 (5th year), and 6.38 (6th year). The average grades in the clerkship rotations were 8.32 (5th year) and 8.26 (6th year). The average score on the theoretical medical residency exam was 7.53 and the final result of the medical residency exam was 8.05. Factor analysis detected three domains with greater correlation strength that accounted for 76.3% of the model variance. Component 1 was identified as the coefficient of academic performance (CAP) 5th, CAP 6th and final medical residency exam grades, whereas component 2 was constituted by the grades of the 5th and 6th years progress tests and the third component comprised the progress tests of the 2nd, 3rd and 4th years. Conclusions: Grades on the progress tests, the clerkship rotations assessments and the final medical residency exam were correlated. Moreover, the performance during the medical clerkship rotations showed the strongest correlations with medical residency exam grades.

Abstract Background: Rheumatic diseases are associated with an increase in overall risks of tuberculosis (TB). The aim of this study was to evaluate the frequency of TB and the frequency of latent TB infection (LTBI), in clinical practice, for juvenile idiopathic arthritis (JIA) patients from high and low risk of TB incidence endemic countries. Methods: This is an international, multicenter, cross-sectional, observational study of data collection from Brazil and Registry of Portugal at REUMA.PT. The inclusion criteria were patients with Juvenile Idiopathic Arthritis (JIA) with age ≤ 18 years who underwent screening for Mycobacterium tuberculosis infection [tuberculin skin test (TST) and/or interferon gamma release assay (IGRA)]. Chest X-rays and history of exposure to TB were also assessed. Results: 292 JIA patients were included; mean age 14.3 years, mean disease duration 7.5 years, 194 patients (66.4%) performed only TST, 14 (4.8%) only IGRA and 84 (28.8%) both. The frequency of LTBI (10.6%) and TB was similar between the two countries. The reasons for TB screening were different; in Brazil it was performed more often at JIA onset while in Portugal it was performed when starting Disease Modified Anti-Rheumatic Drugs (DMARD) treatment (p < 0.001). Isoniazid therapy was prescribed in 40 (13.7%) patients (31 with LTBI and 9 with epidemiologic risks and/or due to contact with sick people). Only three patients (1%) developed active TB. Conclusion: We found nearly 10% of patients with LTBI, a small percentage of patients with treatment due to epide-miologic risks and only 1% with active TB. Distinct reasons and screening methods for LTBI were observed between the two countries.

Abstract Background: Paediatric inflammatory multisystem syndrome (PIMS) associated with severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) has been described since mid-April 2020 with the first reports coming from Europe. Our objective was to describe the characteristics of patients among the Brazilian population. Methods: A multicenter retrospective study was conducted with the participation of five pediatric rheumatology centers in Brazil during the period from March to November 2020. Children and adolescents with PIMS temporally associated with SARS-CoV-2 (TS) who met the definition criteria for the disease according to the Royal College of Paediatrics and Child Health were included. Demographic, clinical, laboratory, therapeutic characteristics and molecular and serological diagnosis of SARS-CoV-2 infection were described. Results: Fifty-seven children and adolescents with PIMS-TS were evaluated, 54% female, with a median age of 8 (3–11) years. Most (86%) were previously healthy, with asthma being the main comorbidity, present in 10% of the patients. Fever was the main manifestation, present in all patients, followed by mucocutaneous and gastrointestinal features, present in 89% and 81% of the patients, respectively. Myocarditis occurred in 21% of the patients and in 68% of them required intensive care. The Kawasaki disease phenotype occurred in most patients (77%). All patients had elevated inflammatory markers, with elevated CRP being the most found (98%). Anemia and lymphopenia were present in 79% and 72%, respectively. Laboratory evidence of SARS-CoV-2 was found in 77% of the patients, with 39% positive RT-PCR and 84% positive serology for SARS-CoV-2. An immunomodulatory treatment was performed in 91% of the patients, with 67% receiving intravenous immunoglobulin (IVIG) associated with glucocorticoid, 21% receiving IVIG, and 3.5% receiving glucocorticoid. The median length of hospitalization was 10 days. Conclusions: This study showed a high morbidity of PIMS-TS in Brazilian children, with a prolonged length of hospitalization and a high rate of admission to pediatric intensive care unit. Multicenter prospective studies are needed to assess the morbidity of the disease in the medium and long term.

Abstract Objective: To present an updated and evidence-based guideline for the use of dual-energy x-ray absorptiometry (DXA) to assess body composition in clinical practice. Materials and methods: This Official Position was developed by the Scientific Committee of the Brazilian Association of Bone Assessment and Metabolism ( Associação Brasileira de Avaliação Óssea e Osteometabolismo , ABRASSO) and experts in the field who were invited to contribute to the preparation of this document. The authors searched current databases for relevant publications in the area of body composition assessment. In this second part of the Official Position, the authors discuss the interpretation and reporting of body composition parameters assessed by DXA and the use of DXA for body composition evaluation in special situations, including evaluation of children, persons with HIV, and animals. Conclusion: This document offers recommendations for the use of DXA in body composition evaluation, including indications, interpretation, and applications, to serve as a guiding tool in clinical practice and research for health care professionals in Brazil.

Abstract Background: The reactivation rate of tuberculosis in patients with chronic inflammatory arthritis (CIA) on TNFα inhibitors (TNFi) and baseline negative screening for latent tuberculosis infection (LTBI) is higher than in the general population. Aim: To compare the performance of tuberculin skin test (TST), TST-Booster, ELISPOT (T-SPOT.TB) and QuantiFERON-TB Gold in tube (QFT-IT) to detect LTBI in patients with CIA on TNFi. Patients and methods: A total of 102 patients with CIA [rheumatoid arthritis (RA), n = 40; ankylosing spondylitis (AS), n = 35; psoriatic arthritis (PsA), n = 7; and juvenile idiopathic arthritis (JIA), n = 20] were prospectively followed-up for 24 months to identify incident LTBI cases. Epidemiologic data, TST, T-SPOT.TB, QFT-IT and a chest X-ray were performed at baseline and after 6 months of LTBI treatment. Results: Thirty six percent (37/102) of patients had positive TST or Interferon Gamma Release Assays (IGRAs) tests. Agreement among TST and IGRAs was moderate (k = 0.475; p = 0.001), but high between T-SPOT.TB and QFT-IT (k=0.785; p < 0.001). During the 24-Month follow-up, 15 (18.5%) incident cases of LTBI were identified. In comparison to TST, the IGRAs increased the LTBI diagnosis power in 8.5% (95% CI 3.16-17.49). TST-Booster did not add any value in patients with negative TST at baseline. After 6-Month isoniazid therapy, IGRAs results did not change significantly. Conclusions: Almost 20% of CIA patients had some evidence of LTBI, suggesting higher conversion rate after exposition to TNFi. TST was effective in identifying new cases of LTBI, but IGRAs added diagnostic power in this scenario. Our findings did not support the repetition of IGRAs after 6-Month isoniazid therapy and this approach was effective to mitigate active TB in 2 years of follow-up.

Resumo Objetivos: Avaliar a presença de síndrome das pernas inquietas, movimento periódico das pernas e distúrbios do sono em adolescentes do sexo feminino com dor musculoesquelética idiopática por meio da escala do sono e da polissonografia e comparar esses dados em adolescentes sem histórico de dor. Método: Foram recrutados 26 adolescentes diagnosticados com dor musculoesquelética idiopática acompanhados em um ambulatório de dor e 25 controles saudáveis pareados por idade e escolaridade. Avaliamos os critérios da síndrome das pernas inquietas de acordo com o Grupo Internacional de Estudos de Síndrome das Pernas Inquietas, a Escala de Distúrbios do Sono em Crianças, a polissonografia noturna e os sintomas de ansiedade. Resultados: A idade média dos adolescentes com dor musculoesquelética idiopática foi 13,9 ± 1,6 anos e dos controles foi 14,4 ± 1,4 anos. Um adolescente no grupo de controle (4%) e nove pacientes com dor musculoesquelética idiopática (34,6%) atenderam aos critérios da síndrome das pernas inquietas (p = 0,011). Não observamos diferenças significativas nos escores da Escala de Distúrbios do Sono em Crianças entre os grupos em todos os componentes: distúrbios do início e da manutenção do sono (p = 0,290), distúrbios respiratórios do sono (p = 0,576), distúrbios do despertar (p = 0,162), distúrbios da transição sono-vigília (p = 0,258), sonolência diurna excessiva (p = 0,594) e hiperidrose do sono (p = 0,797). Os parâmetros neurofisiológicos, respiratórios e o movimento periódico das pernas foram semelhantes nos dois grupos. Ansiedade não foi associada à síndrome das pernas inquietas (p = 0,11). Três pacientes com dor musculoesquelética idiopática (11,5%) apresentaram síndrome das pernas inquietas e movimento periódico das pernas simultaneamente, situação ausente no grupo de controle. Conclusão: As adolescentes do sexo feminino com dor musculoesquelética idiopática apresentaram critérios para síndrome das pernas inquietas com mais frequência do que as adolescentes saudáveis. Contudo, não observamos mudanças relevantes nas variáveis do sono objetivas e subjetivas.

Abstract Objectives: To assess the presence of restless legs syndrome, periodic leg movement, and sleep disorders in female adolescents with idiopathic musculoskeletal pain through a sleep scale and polysomnography, and to compare these data in adolescents without pain history. Method: Twenty-six adolescents diagnosed with idiopathic musculoskeletal pain followed in a pain outpatient clinic and 25 healthy controls matched by age and education were recruited. The restless legs syndrome criteria were evaluated according to the International Restless Legs Syndrome Study Group, the Sleep Disturbance Scale for Children was completed, nocturnal polysomnography was performed, and anxiety symptoms were recorded. Results: The mean age of idiopathic musculoskeletal pain adolescents was 13.9 ± 1.6 years; in controls, it was 14.4 ± 1.4 years. One adolescent in the control group (4 %) and nine patients with idiopathic musculoskeletal pain (34.6 %) fulfilled the restless legs syndrome criteria (p = 0.011). The authors did not observe significant differences in Sleep Disturbance Scale for Children scores between the groups in all components: disorders of initiating and maintaining sleep (p = 0.290), sleep breathing disorders (p = 0.576), disorders of arousal (p = 0.162), sleep-wake transition disorders (p = 0.258), disorder of excessive daytime somnolence (p = 0.594), and sleep hyperhidrosis (p = 0.797). The neurophysiological, respiratory, and periodic leg movement parameters were similar in both groups. Having anxiety was not associated with restless legs syndrome (p = 0.11). Three patients with idiopathic musculoskeletal pain (11.5 %) presented restless legs syndrome and periodic leg movement simultaneously, which was absent in the control group. Conclusion: Female adolescents with idiopathic musculoskeletal pain present criteria for RLS more frequently than healthy adolescents. However, this study did not observe relevant changes in objective and subject sleep variables.