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Nas últimas décadas, índices compostos para avaliar atividade de doença, resposta terapêutica e dano em pacientes com doenças reumáticas foram elaborados e validados para uso na população pediátrica. A artrite idiopática juvenil (AIJ) apresentou grande avanço nesse campo. Nos anos 1990 foi elaborado o ACR Pedi para AIJ, de acordo com os critérios do ACR para a artrite reumatoide. A partir de então, foi elaborado o JADAS, escore composto de quatro variáveis, para quantificar atividade de doença na AIJ e sua versão clínica (cJADAS), onde não se contabiliza o valor de VHS/PCR. De acordo com o número de articulações ativas incluídas, obtém-se JADAS71, JADAS27 e JADAS10. Há ainda o JADAS sistêmico, que inclui um quinto item para manifestação sistêmica, e o Juvenile SpA disease activity (JSpADA), recentemente desenvolvido para avaliar atividade de doença na espondiloartrite juvenil. O dano crônico na AIJ é calculado através do Juvenile Arthritis Damage Index (JADI), composto pelos JADI-A (articular) e JADI-E (extra-articular). As ferramentas para avaliar atividade no lúpus eritematoso sistêmico juvenil (LESJ) são as mesmas utilizadas na população adulta (SLEDAI e BILAG), e apresentam boa sensibilidade na pediatria. O dano no LESJ pode ser mensurado através da versão pediátrica do SLICC/ACR-DI. Há dois escores para monitoramento de vasculites sistêmicas específicos para população pediátrica: o Paediatric Vasculitis Activity Score (PVAS), derivado do BVASv.3 modificado, e o Paediatric Vasculitis Damage Index (PVDI), derivado do VDI. Na dermatomiosite juvenil, a atividade global da doença é mensurada pelo Disease Activity Score (DAS), e a avaliação da força muscular pelo Childhood Myositis Assessment Scale (CMAS) e Manual Muscle Testing (MMT). Child Health Questionnaire – Parent Form 50 (CHQ–PF50), o Pediatric Quality of Life Inventory (PedsQL™) version 4.0 são instrumentos para avaliar qualidade de vida, validados na população pediátrica e factíveis na prática clínica. Unitermos: Métricas. Reumatologia pediátrica. Atividade de doença. Dano de doença. AIJ.

Purpose: SARS-CoV-2 infection in children is usually asymptomatic/mild. However, some patients may develop critical forms. Our aim was to evaluate the independent risk factors associated to in-hospital mortality in children with critical disease related to SARS-CoV-2. Methods: This is multicenter prospective cohort included critically ill children (1 month/18 years of age), with confirmed critical disease related to SARS-CoV-2 admitted to three tertiary Pediatric Intensive Care Units (PICU) in Brazilian Amazon, between April 2020/July 2022. Main outcome was in-hospital mortality. The independent risk factors associated with mortality were evaluated with a multivariable Cox proportional regression. Results: 208 patients were assessed. Median age was 33 months and median follow-up was 277 days (range, 2-759). Death occurred in 37 (17.8%) patients with a median follow-up of 7 (4-13) days. Most non-survivors had at least one comorbidity - 34 (91.9%). Substantial clinical features, laboratory and ventilatory parameters were associated with mortality. Independent risk factors for mortality were underweight status (HR= 6.64, p=0.01), vasoactive inotropic score (VIS) > 84 (HR=4.76, p=0.05), acute respiratory distress syndrome (HR=8.63, p=0.02) and erythrocyte sedimentation rate (ESR) >18 mm/hour (HR=3.95, p=0.03). Conclusions: This study of critically ill patients with COVID-19 and MIS-C from the Brazilian Amazon showed a high mortality rate. The risk of death was higher for underweight individuals, those with higher levels of VIS and ESR, presence of ARDS. The majority of deaths occurred within 10 days of hospitalization, highlighting the importance of prompt recognition in regard to these patients.

Objectives Toassess whether 18F-fluordeoxiglucose (18F-FDG) PET/MRI) with angiographic sequences can contribute to detecting vessel wall inflammation in patients with childhood-onset Takayasu’s arteritis (c-TA) under immunosuppressive therapy. Methods A three-centre cross-sectional study was conducted. 18F-FDG PET/MRI scans were performed in c-TA patients and in oncologic patients, who served as the control group. Clinical and laboratory characteristics were also analysed. Results Seventeen c-TA patients (65% females) between the ages of 6 and 21 years with a mean disease duration of 9.4 years were recruited. Only one patient presented clinical disease activity and six (35.6%) had increased ESR and/or CRP levels. The most frequent magnetic resonance angiography (MRA) findings were stenosis and thickening, observed in 82.4 and 70.6% of c-TA patients, respectively. 18F-FDG PET revealed 18F-FDG uptake greater than the liver in at least one arterial segment in 15 (88.2%) patients in a qualitative analysis and a median maximum standardized uptake value (SUVmax) of 3.22 (interquartile range 2.76–3.69) in a semi-quantitative analysis. c-TA patients presented significantly higher SUVmax values than oncologic patients (P Conclusion A state-of-the-art imaging modality was used in c-TA patients and revealed a strong arterial FDG uptake even in patients in apparent remission. We suppose that this finding may represent silent activity in the vessel wall; however, we cannot exclude the possibility of arterial remodelling. Importantly, a negative imaging scan may help in immunosuppression withdrawal in daily clinical practice.

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.

Lupus nephritis (LN) is a frequent manifestation of childhood-onset systemic lupus erythematosus (cSLE) with a potential risk for kidney failure and poor outcomes. This study aimed to evaluate stages III, IV, and V of chronic kidney disease (CKD) and investigate risk factors for CKD in cSLE patients.We performed a nationwide observational cohort study in 27 pediatric rheumatology centers, including medical charts of 1528 cSLE patients. Data were collected at cSLE diagnosis, during follow-up, and at last visit or death, between September 2016 and May 2019.Of 1077 patients with LN, 59 (5.4%) presented with CKD, 36/59 (61%) needed dialysis, and 7/59 (11.8%) were submitted for kidney transplantation. After Bonferroni's correction for multiple comparisons (p 0.0013), determinants associated with CKD were higher age at last visit, urinary biomarker abnormalities, neuropsychiatric involvement, higher scores of disease activity at last visit and damage index, and more frequent use of methylprednisolone, cyclosporine, cyclophosphamide, and rituximab. In the regression model analysis, arterial hypertension (HR = 15.42, 95% CI = 6.12-38.83, p ≤ 0.001) and biopsy-proven proliferative nephritis (HR = 2.83, 95%CI = 1.70-4.72, p ≤ 0.001) increased the risk of CKD, while children using antimalarials had 71.0% lower CKD risk ((1.00-0.29) × 100%) than children not using them. The Kaplan-Meier comparison showed lower survival in cSLE patients with biopsy-proven proliferative nephritis (p = 0.02) and CKD (p ≤ 0.001).A small number of patients manifested CKD; however, frequencies of dialysis and kidney transplantation were relevant. This study reveals that patients with cSLE with hypertension, proliferative nephritis, and absence of use of antimalarials exhibited higher hazard rates of progression to CKD. A higher resolution version of the Graphical abstract is available as Supplementary information.

Background: Some children can develop severe forms of SARS-CoV-2 infection either acutely or later, as represented by multisystemic inflammatory syndrome in children (MIS- C). To identify the risk factors for worse outcomes in hospitalized children and adolescents with severe acute SARS-CoV-2 infection and MIS-C. Methods: This multicenter cohort study included all children and adolescents with confirmed or suspected critical SARS-CoV-2 infection admitted to the PICU between April 2020 and September 2021. The exclusion criteria were incomplete vaccinal status, immunocompromised status, and end-of-life decision. The main variables analyzed were epidemiological, clinical, and laboratory data, and ventilator settings at admission and after 72 h. The patients were divided into three groups (G): confirmed coronavirus disease (COVID-19) with MIS-C criteria (G1), confirmed COVID-19 without MIS-C criteria (G2), and MIS-C criteria without confirmed COVID-19. Results: The median age of the patients was 28 months in G1, with comorbidities in 40 patients (72.7%) (p < 0.0001). The duration of exposure (median 23 days; p = 0.004) and fever were longer in G1 (12 days; p = 0.001). Moreover, invasive mechanical ventilation (IMV) was required in 44 patients (80%, p < 0.0001), and cardiogenic shock occurred in 26 patients (54.2%, p < 0.0001) in G1. Subnutrition was most frequent in G1 in 55 cases (57.3%; p = 0.01). Under nutrition (< 2 SD for weight), longer exposure time (odds ratio [OR]: 2.11; 95% confidence interval [CI]: 1.37–3.25; p = 0.001), IMV time (OR: 2.6; 95% CI: 1.15–5.85; p = 0.03), and length of hospital stay (OR: 10.94; 95% CI: 1.93–63.1; p = 0.007) were associated with critical MIS-C in G1. Conclusions: In the Brazilian Amazon area, specifically in the Pará state, we identified a cluster of more severe forms of pediatric acute or late SARS-CoV-2 infection.

Objective: To translate and validate the Brazilian Portuguese version of the Transition Readiness Assessment Questionnaire in a population of adolescents and young adults with chronic rheumatologic disorders. This questionnaire evaluates the patient's readiness for making the transition from the pediatric health service to adult care. Methods: The four‐phase methodology for the translation and validation of generic questionnaires was followed, including translation, back‐translation, pilot testing and clinical validation of the final tool. The confirmatory factor analysis was used for clinical validation and the Cronbach's alpha coefficient was used to assess the overall internal consistency of the final tool. Results: A total of 150 patients with a mean age of 17.0 years (SD = 2.2 years, range 14–21 years) were enrolled for the final tool validation. Of those, 71 patients had juvenile systemic lupus erythematosus (47.3%), 64 had juvenile idiopathic arthritis (42.7%), and 15 had juvenile dermatomyositis (10%). During the confirmatory factor analysis, the dimension “Talking with providers” consisting of two questions, was considered as not fitting the translated questionnaire due to a very high ceiling effect and was therefore excluded. All other translated items favorably contributed to the overall consistency of the final tool; removing that dimension did not result in a substantial increase in Cronbach's alpha, which was 0.776. Conclusions: The Brazilian Portuguese version of the Transition Readiness Assessment Questionnaire was validated in a population of transitional patients with chronic rheumatologic disorders, after one dimension from the original questionnaire was excluded. It is a non‐specific disease questionnaire; thus, it can be used to evaluate the transition readiness of Brazilian patients with other chronic diseases. Resumo: Objetivo: Traduzir para o português brasileiro e validar o Questionário de Avaliação do Preparo para a Transição em uma população de adolescentes e adultos jovens com doenças reumáticas crônicas. Este questionário avalia o preparo do paciente para realizar a transição do serviço de saúde pediátrico para a assistência ao adulto. Métodos: Seguimos a metodologia de quatro etapas para a tradução e validação de questionários genéricos que inclui tradução, retrotradução, teste piloto e validação clínica do instrumento final. Utilizamos Análise Fatorial Confirmatória e Coeficiente Alfa de Cronbach para testar a validade do instrumento e sua consistência interna. Resultados: Responderam ao questionário traduzido e adaptado 150 pacientes. A média de idade foi de 17,0 anos (DP = 2,2 anos, variação 14‐21 anos). Tinham o diagnóstico de lúpus eritematoso sistêmico juvenil 71 pacientes (47,3%), 64 (42,7%) artrite idiopática juvenil e 15 (10%) dermatomiosite juvenil. Durante a análise fatorial confirmatória, a dimensão “Falando com a Equipe Médica” contendo duas questões teve que ser removida devido à presença de expressivo efeito teto. Todas as outras questões restantes contribuíram favoravelmente para aumentar a consistência interna do questionário, obteve‐se um Coeficiente Alfa de Cronbach de 0,776. Conclusões: O Questionário de Avaliação do Preparo para a Transição na sua versão em português brasileiro pode ser validado em uma população de pacientes com doenças reumáticas crônicas em transição, com a exclusão de uma dimensão do questionário original. Por ser um questionário não específico para doenças reumáticas, poderá ser utilizado para avaliar o preparo para a transição de outros pacientes brasileiros com doenças crônicas. Keywords: Transition readiness tool, TRAQ, Brazil, Rheumatology, Adolescent health services, Research in health services, Palavras‐chave: Avaliação do preparo para a transição, TRAQ, Brasil, Reumatologia, Serviços de saúde do adolescente, Pesquisa em serviços de saúde

Background It is currently unknown whether patients with childhood-onset Takayasu disease (c-TA) are prone to physical inactivity and poor aerobic capacity. In this study, we assessed physical activity levels and cardiorespiratory fitness along with health-related quality of life (HRQL) and various traditional and non-traditional risk factors in patients with c-TA vs. healthy controls. Methods c-TA patients with non-active disease (n = 17) and age- and sex-matched healthy controls (n = 17) were enrolled in the study. We assessed physical activity levels, aerobic capacity, body composition, systemic inflammation, cardiometabolic markers, disease-related parameters, and HRQL. Results c-TA patients showed greater time spent in sedentary behavior (P = 0.010), and lower moderate-to-vigorous physical activity (P > 0.001) and lower step counts per day (P > 0.001). VO2peak (P P = 0.016) were significantly lower in patients with c-TA and they had worse HRQL in physical domain (P P ≤ 0.05). Conclusions c-TA patients exhibited reduced physical activity levels and aerobic capacity, worse cardiometabolic risk factors and HRQL parameter compared with healthy peers. Physical inactivity and aerobic deconditioning emerge as potentially novel risk factors for c-TA. The role of physical activity interventions in preventing poor outcomes and improving HRQL in c-TA remains to be explored.

Made available in DSpace on 2019-10-06T15:31:10Z (GMT). No. of bitstreams: 0 Previous issue date: 2018-11-23. Added 1 bitstream(s) on 2021-07-15T15:23:32Z : No. of bitstreams: 1 S2523-31062018000100228.pdf: 598953 bytes, checksum: c510a60909892e9f6d7f26d4469f267b (MD5) Conselho Nacional de Desenvolvimento Científico e Tecnológico (CNPq) OBJECTIVE: To evaluate prevalence, clinical manifestations, laboratory abnormalities and treatment in a multicenter cohort study including 847 childhood-onset systemic lupus erythematosus (cSLE) patients with and without diffuse alveolar hemorrhage (DAH), as well as concomitant parameters of severity. METHODS: DAH was defined as the presence of at least three respiratory symptoms/signs associated with diffuse interstitial/alveolar infiltrates on chest x-ray or high-resolution computer tomography and sudden drop in hemoglobin levels. Statistical analysis was performed using Bonferroni correction (p

ResumoObjetivoDescrever as características clínicas e angiográficas da arterite de Takayasu em crianças e adolescentes brasileiros.MétodosFoi feita coleta retrospectiva de dados de 71 crianças e adolescentes acompanhados em 10 centros brasileiros de referência em reumatologia pediátrica. A avaliação foi feita em três tempos: início dos sintomas até o diagnóstico, do 6° ao 12° mês de diagnóstico e última consulta.ResultadosDos 71 pacientes selecionados, 51 (71,8%) eram meninas. As médias de idade de início dos sintomas e de tempo até diagnóstico foram 9,2 anos (± 4,2) e 1,2 anos (± 1,4), respectivamente. No fim do estudo, 20 pacientes estavam em atividade de doença, 39 em remissão e cinco haviam evoluído a óbito. Os sintomas mais frequentes nas avaliação inicial, segunda avaliação e avaliação final foram, respectivamente, os constitucionais, os musculoesqueléticos e os neurológicos. A redução de pulsos periféricos foi o sinal cardiovascular mais frequente e a elevação da velocidade de hemossedimentação foi o achado laboratorial mais frequente nos três períodos de avaliação. O teste tuberculínico foi reagente em 41%. A estenose foi a lesão angiográfica mais encontrada, a artéria abdominal foi o segmento mais afetado e tipo angiográfico IV o mais frequente. A maioria (90%) fez terapia com glicocorticoides, 85,9% necessitaram de outro imunossupressor e 29,6% foram submetidos à angioplastia.ConclusãoEste é o maior estudo de arterite de Takayasu juvenil e nós observar elevado número de pacientes com idade inferior a 10 anos e a predominância de sintomas constitucionais no início da doença.AbstractObjectiveTo describe the clinical and angiographic characteristics of Takayasu's arteritis in Brazilian children and adolescents.MethodsA retrospective data collection was performed in 71 children and adolescents followed in 10 Brazilian reference centers in Pediatric Rheumatology. The evaluation was carried out in three different time points: from onset of symptoms to diagnosis, from the 6th to 12th month of diagnosis, and in the last visit.ResultsOf 71 selected patients, 51 (71.8%) were girls. The mean age of onset of symptoms and of time to diagnosis was 9.2 (± 4.2) years and 1.2 (± 1.4) years, respectively. At the end of the study, 20 patients were in a state of disease activity, 39 in remission and 5 had evolved to death. The most common symptoms in baseline assessment, second evaluation, and final evaluation were, respectively: constitutional, musculoskeletal, and neurological symptoms. A decrease in peripheral pulses was the most frequent cardiovascular signal, and an increase in erythrocyte sedimentation rate was the most frequent laboratory finding in all three evaluation periods. The tuberculin test was positive in 41% of those tested. Stenosis was the most frequent angiographic lesion, abdominal artery was the most affected segment, and angiographic type IV the most frequent. Most (90%) participants were treated with glucocorticoids, 85.9% required another immunosuppressive drug, and 29.6% underwent angioplasty.ConclusionThis is the largest study on juvenile‐onset Takayasu arteritis, and a high number of patients under the age of 10 years, with predominance of constitutional symptoms early in the disease, was observed.

Background The Henoch-Schönlein Purpura (HSP) orIgA vasculitis is the most common vasculitis of childhood and may occur with renal involvement, with hematuria and / or proteinuria, and may cause severe and non-reversible sequelae. Objectives To establish the profile of patients with renal involvement due to IgA vasculitisand to describe our experience with the use of azathioprine to treat patients with nephritis. Methods Clinical data were retrospectively collected from medical records of patients with IgA vasculitiswho attended the pediatric rheumatology unit between 1995 and 2017. Patients were separated into two groups based on whether or notthey weretreated with non-glucocorticoid immunosuppressants. Results From the178 patients with IgA vasculitis,nephritis was found in67 patients (37.6%), 13 of whom receivedtreatment with non-glucocorticoid immunosuppressants. Ten patients responded well to azathioprine and 1 patient to cyclosporine. Forty patients received oral glucocorticoids, whilst 16received intravenous glucocorticoids. Conclusion Azathioprine may be beneficial in the treatment of IgA vasculitis with renal involvement.

Juvenile-Takayasu arteritis (j-TA) is a difficult diagnosis and some patients develop uncommon manifestations and associated diseases that may contribute to the delayed diagnosis. Our aim was to identify the misdiagnoses, the associated diseases and the atypical manifestations observed in a j-TA Brazilian multicentre study. 71 children and adolescents who met the classification criteria for j-TA were included. The misdiagnoses, the associated diseases and the atypical manifestations were evaluated. 19 (26.8%) patients had misdiagnoses. The most common of them was aortic coarctation in six (8.4%) patients, followed by rheumatic fever in five (7.0%) and one patient presented with both former diagnoses. Limb pain (two patients), spondyloarthropathy, juvenile idiopathic arthritis (JIA), spinal arteriovenous malformation, polyarteritis nodosa (PAN) and fever of unknown origin (FUO) were other misdiagnoses. Patients who had misdiagnoses previously to j-TA diagnosis presented a trend to have a longer diagnosis delay. 11 (15.5%) patients had 14 TA-associated diseases, such as pulmonary tuberculosis (5 patients), rheumatic fever (2 patients), spondyloarthropathy, polyarticular JIA, Crohn's disease, Prader-Willi disease, diabetes mellitus, Moyamoya and primary immunodeficiency. 7 (9.9%) patients presented 10 atypical manifestations, such as pyoderma gangrenosum, erythema nodosum, myositis, chorea, enthesitis, episcleritis, uveitis, hepatomegaly, splenomegaly and necrosis of extremities. Our study emphasizes the main misdiagnoses, associated diseases and atypical manifestations that occur in patients with j-TA and warns of the features that may alert paediatricians to this diagnosis, such as constitutional symptoms and elevated inflammatory markers.

Objective To evaluate symptomatic polyautoimmunity (PA) at childhood-onset systemic lupus erythematosus(cSLE) diagnosis, and its association with demographic data, disease activity, clinical manifestations and laboratorial abnormalities in a large Brazilian cSLE population. Methods A multicenter retrospective study was performed in 1463 cSLE(ACR criteria) patients from 27 Pediatric Rheumatology services. Symptomatic PA was defined according to the presence of more than one concomitant autoimmune disease(AD) and symptomatic multiple autoimmune syndrome(MAS) was defined as three or more AD. An investigator meeting was held to define the protocol. Demographic data, SLICC classification criteria and SLEDAI-2K were evaluated. Results At cSLE diagnosis symptomatic PA was observed in 144/1463(9.8%) and symptomatic MAS occurred in solely 10/1463(0.7%). In the former group the more frequently observed associated AD were Hashimoto thyroiditis n = 42/144(29%), antiphospholipid syndrome n = 42/144(29%), autoimmune hepatitis n = 26/144(18%) and type 1 diabetes mellitus n = 23/144(15.9%). Further comparisons between cSLE patients with and without PA showed a higher median age(p = 0.016) and lower mean SLICC criteria (p = 0.039) in those with PA. Additionally, these cSLE patients had less renal involvement(35% vs. 44%, p = 0.038) and red blood cell cast(6% vs. 12%, p = 0.042) and more antiphospholipid antibodies(29% vs. 15%, p Conclusions Approximately 10% of cSLE had symptomatic PA at diagnosis, particularly endocrine autoimmune disorders and antiphospholipid syndrome. Lupus was characterized by a mild disease onset and MAS was infrequently evidenced. Further studies are necessary to determine if this subgroup of cSLE patients have a distinct genetic background with a less severe disease and a better long-term outcome.

Made available in DSpace on 2018-12-11T17:16:07Z (GMT). No. of bitstreams: 0 Previous issue date: 2017-11-01 Objectives To assess clinical digital vasculitis (DV) as an initial manifestation of childhood‐onset systemic lupus erythematosus (cSLE) within a large population. Methods Multicenter cross‐sectional study including 852 cSLE patients (ACR criteria) followed in ten Pediatric Rheumatology centers in São Paulo State, Brazil. Results DV was observed in 25/852 (3%) cSLE patients. Periungual hemorrhage was diagnosed in 12 (48%), periungual infarction in 7 (28%), tip finger ulceration in 4 (16%), painful nodules in 1 (4%) and gangrene in 1 (4%). A poor outcome, with digital resorption, occurred in 5 (20%). Comparison of patients with and without DV revealed higher frequency of malar rash (80% vs. 53%, p = 0.008), discoid rash (16% vs. 4%, p = 0.017), photosensitivity (76% vs. 45%, p = 0.002) and other cutaneous vasculitides (80% vs. 19%, p < 0.0001), whereas the frequency of overall constitutional features (32% vs. 61%, p = 0.003), fever (32% vs. 56%, p = 0.020) and hepatomegaly (4% vs. 23%, p = 0.026) were lower in these patients. Frequency of female gender, severe multi‐organ involvement, autoantibodies profile and low complement were alike in both groups (p > 0.05). SLEDAI‐2 K median, DV descriptor excluded, was significantly lower in patients with DV compared to those without this manifestation [10(0‐28) vs. 14(0‐58), p = 0.004]. Visceral vasculitis or death were not observed in this cSLE cohort. The frequency of cyclophosphamide use (0% vs. 18%, p = 0.014) was significantly lower in the DV group. Conclusion Our large multicenter study identified clinical DV as one of the rare initial manifestation of active cSLE associated with a mild multisystemic disease, in spite of digital resorption in some of these patients. Universidade Federal de São Paulo (Unifesp) Unidade de Reumatologia Pediátrica Universidade de São Paulo (USP) Faculdade de Medicina Unidade de Reumatologia Pediátrica Universidade de São Paulo (USP) Faculdade de Medicina Divisão de Reumatologia Universidade Estadual Paulista (Unesp) Faculdade de Medicina de Botucatu Hospital das Clínicas de Botucatu Universidade Estadual Paulista (Unesp) Faculdade de Medicina de Botucatu Hospital das Clínicas de Botucatu