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International audience; Objective. Juvenile Sjögren's syndrome (SS) is a rare, poorly defined, and possibly underdiagnosed condition affecting children and adolescents. The aim of this study was to characterize symptoms and clinical findings of juvenile SS and to explore the clinical application of major salivary gland ultrasonography (SGUS) in patients with juvenile SS. Methods. A cross-sectional multicenter study recruited patients with disease onset until age 18 years (n = 67). Disease characteristics were recorded, and unstimulated whole sialometry and SGUS examination of the parotid and submandibular salivary glands were performed. Results. The female:male ratio was 58:9. The mean age at first symptom was 10.2 years and 12.1 years at diagnosis. Ocular and oral symptoms were noted in 42 of 67 patients (63%) and 53 of 66 patients (80%), respectively. The American-European Consensus Group or American College of Rheumatology/European League Against Rheumatism classification criteria for primary SS were fulfilled by 42 of 67 patients (63%). Pathologic SGUS findings were observed in 41 of 67 patients (61%); 26 of 41 SGUS+ patients (63%) fulfilled primary SS criteria. Salivary gland enlargements/parotitis were noted in 37 of 58 patients and were nonsignificantly associated with SGUS+ status (P = 0.066). The mean levels of saliva were 5.6 ml/15 minutes in SGUS-patients compared to 3.3 ml/15 minutes in the SGUS+ patients (P = 0.049). A total of 36 of 41 SGUS+ patients (88%) were anti-Ro/La+ compared to 14 of 26 SGUS-patients (54%) (P = 0.001). In addition, 24 of 39 SGUS+ patients (62%) were positive for rheumatoid factor (RF), whereas only 5 of 25 SGUS-patients (20%) were RF+ (P = 0.001). Conclusion. Juvenile SS is characterized by a large spectrum of clinical symptoms and findings. Several glandular and extraglandular parameters such as hyposalivation, swollen salivary glands, and autoantibodies are associated with pathologic SGUS findings.

Made available in DSpace on 2022-04-29T08:46:03Z (GMT). No. of bitstreams: 0 Previous issue date: 2021-01-01 Objective: To evaluate if the 2019-European League Against Rheumatism/American College of Rheumatology (EULAR/ACR) criteria at diagnosis of childhood-onset systemic lupus erythematosus (cSLE) are associated with higher rates of early damage scored by Systemic Lupus International Collaborating Clinics/American College of Rheumatology (SLICC/ACR) Damage Index (SDI). Methods: This retrospective multicenter study included 670 cSLE patients with ≤5 years of disease duration. All patients fulfilled both 2019-EULAR/ACR and 1997-ACR classification criteria. Total score of 2019-EULAR/ACR criteria and each of its specific domains were assessed at diagnosis as predictors of damage accrual at the last visit, according to the presence of any organ damage (defined by SDI ≥ 1). Results: Median disease duration was 2.8 (IQR 1.8–3.8) years and 200 (29.9%) patients had at least one organ damage (SDI ≥ 1). The most frequent domains were neuropsychiatric (12%), renal (7%), and musculoskeletal (6%). There was a higher frequency of renal (58% vs 43%, p = 0.0004) and neuropsychiatric domain (21% vs 7%, p < 0.0001) of 2019-EULAR/ACR criteria in patients with damage (SDI ≥ 1) compared to those without damage (SDI = 0). Patients scoring renal or neuropsychiatric domains of the 2019-EULAR/ACR criteria at diagnosis were associated with renal damage (odds ratio 9.701, 95% confidence interval 3.773–24.941, p < 0.001) or neuropsychiatric damage (OR 9.480, 95% CI 5.481–16.399, p25 was associated with more overall (SDI ≥ 1) (38% vs 25%, p = 0.0002) and renal damage (11% vs 5%, p = 0.023). Conclusions: The 2019-EULAR/ACR criteria at diagnosis were associated with a higher rate of early damage in cSLE patients, especially for renal and neuropsychiatric damage. Of note, damage was particularly associated with high disease activity at diagnosis and 2019-EULAR/ACR score >25. Pediatric Rheumatology Unit Children’s Institute Hospital das Clinicas HCFMUSP Faculdade de Medicina Universidade de Sao Paulo Division of Rheumatology Hospital das Clinicas HCFMUSP Faculdade de Medicina Universidade de Sao Paulo Pediatric Rheumatology Unit Universidade Federal de Sao Paulo Pediatric Rheumatology Division Sao Paulo State University (UNESP) Pediatric Rheumatology Unit Janeiro Federal University (IPPMG-UFRJ) Pediatric Rheumatology Unit Hospital da Criança de Brasília Jose Alencar Post-graduation Program in Medical Science and Rheumatology Unit University of Brasilia Pediatric Rheumatology Unit Ribeirao Preto Medical School University of Sao Paulo Pediatric Rheumatology Unit Hospital Geral de Fortaleza Pediatric Rheumatology Unit University of Campinas (UNICAMP) Pediatric Rheumatology Unit Pedro Ernesto University Hospital Pediatric Rheumatology Unit Irmandade da Santa Casa de Misericórdia de Sao Paulo Rheumatology Division - Universidade Federal do Rio de Janeiro Hospital Universitário Clementino Fraga Filho Pediatric Rheumatology Unit Federal University of Pará Pediatric Rheumatology Unit Federal University of Bahia Pediatric Rheumatology Unit Hospital Menino Jesus Pediatric Rheumatology Unit Federal University of Pernambuco Pediatric Rheumatology Unit Federal University of Mato Grosso do Sul Pediatric Rheumatology Division Sao Paulo State University (UNESP)

Background There is a lack of information on the role of chronic use of hydroxychloroquine during the SARS-CoV-2 outbreak. Our aim was to compare the occurrence of COVID-19 between rheumatic disease patients on hydroxychloroquine with individuals from the same household not taking the drug during the first 8 weeks of community viral transmission in Brazil. Methods This baseline cross-sectional analysis is part of a 24-week observational multi-center study involving 22 Brazilian academic outpatient centers. All information regarding COVID-19 symptoms, epidemiological, clinical, and demographic data were recorded on a specific web-based platform using telephone calls from physicians and medical students. COVID-19 was defined according to the Brazilian Ministry of Health (BMH) criteria. Mann–Whitney, Chi-square and Exact Fisher tests were used for statistical analysis and two binary Final Logistic Regression Model by Wald test were developed using a backward-stepwise method for the presence of COVID-19. Results From March 29th to May 17st, 2020, a total of 10,443 participants were enrolled, including 5166 (53.9%) rheumatic disease patients, of whom 82.5% had systemic erythematosus lupus, 7.8% rheumatoid arthritis, 3.7% Sjögren’s syndrome and 0.8% systemic sclerosis. In total, 1822 (19.1%) participants reported flu symptoms within the 30 days prior to enrollment, of which 3.1% fulfilled the BMH criteria, but with no significant difference between rheumatic disease patients (4.03%) and controls (3.25%). After adjustments for multiple confounders, the main risk factor significantly associated with a COVID-19 diagnosis was lung disease (OR 1.63; 95% CI 1.03–2.58); and for rheumatic disease patients were diagnosis of systemic sclerosis (OR 2.8; 95% CI 1.19–6.63) and glucocorticoids above 10 mg/ day (OR 2.05; 95% CI 1.31–3.19). In addition, a recent influenza vaccination had a protective effect (OR 0.674; 95% CI 0.46–0.98). Conclusion Patients with rheumatic disease on hydroxychloroquine presented a similar occurrence of COVID-19 to household cohabitants, suggesting a lack of any protective role against SARS-CoV-2 infection. Trial registration Brazilian Registry of Clinical Trials (ReBEC; RBR – 9KTWX6).

Objectives To identify potential predictors of COVID-19 vaccine hesitancy (C19-VH) in adults with immune-mediated inflammatory diseases (IMID). Methods A total of 1000 IMID patients were enrolled in this web-based cross-sectional study. A standardised and self-administered survey was designed by members of the Brazilian Society of Rheumatology Steering Committee for Infectious and Endemic diseases and distributed to IMID patients spread across Brazil. Results Of the 908 (90.8%) respondents eligible for analysis, 744 (81.9%) were willing to get vaccinated against COVID-19. In our multivariable logistic regression model, concurrent malignancy, fibromyalgia, hydroxychloroquine use, and recent corticosteroid pulse therapy were independently associated with higher odds of C19-VH. The short duration of COVID-19 vaccine clinical trials was the main reason for C19-VH. Conclusion We identified novel characteristics potentially associated with C19-VH among adults with IMID. Greater awareness on the safety and efficacy of COVID-19 vaccines is needed for both IMID patients and attending physicians.

Objective To evaluate the baseline clinical characteristics of juvenile systemic sclerosis (SSc) patients in the international juvenile SSc inception cohort, and to compare these characteristics between the classically defined juvenile diffuse cutaneous SSc (dcSSc) and limited cutaneous SSc (lcSSc) subtypes and among those with overlap features. Methods A cross-sectional study was performed using baseline visit data. Information on demographic characteristics, organ system evaluation, treatment, and patient- and physician-reported outcomes was extracted and summary statistics applied. Comparisons between juvenile dcSSc and lcSSc subtypes and patients with and without overlap features were performed using chi-square and Mann-Whitney U tests. Results At data extraction, 150 juvenile SSc patients were enrolled across 42 centers; 83% were White, 80% were female, juvenile dcSSc predominated (72%), and 17% of the cohort had overlap features. Significant differences were found between juvenile dcSSc and juvenile lcSSc regarding modified Rodnan skin thickness score, the presence of Gottron's papules, digital tip ulceration, results of the 6-minute walk test, and composite pulmonary and cardiac involvement. All of these were more frequent in dcSSc except for cardiac involvement. Juvenile dcSSc patients had significantly worse scores for physician-rated disease activity and damage. A significantly higher occurrence of Gottron's papules and musculoskeletal and composite pulmonary involvement, and a significantly lower frequency of Raynaud's phenomenon, were seen in those with overlap features. Conclusion Results from a large international juvenile SSc cohort demonstrate significant differences between juvenile dcSSc and juvenile lcSSc patients, including more globally severe disease and increased frequency of interstitial lung disease in juvenile dcSSc patients, while those with lcSSc have more frequent cardiac involvement. Those with overlap features had an unexpected higher frequency of interstitial lung disease.

Made available in DSpace on 2021-06-25T12:24:54Z (GMT). No. of bitstreams: 0 Previous issue date: 2020-11-11 Background: Concerns about the safety and efficacy of vaccines in patients with autoimmune diseases (AID) have led to contradictions and low vaccination coverage in this population, who are at a higher risk of infections, including by human papillomavirus (HPV). Although HPV vaccines have been recommended for immunocompromised patients, there is still a lack of data to support its use for AID patients, such as juvenile dermatomyositis (JDM) patients. The aim of this study was to assess the safety and immunogenicity of the quadrivalent HPV (qHPV) vaccine in a cohort of JDM patients. Methods: JDM patients aged from 9 to 20 years and healthy controls (HC) were enrolled to receive a 3-dose schedule of qHPV vaccine from March/2014 to March/2016. Study visits were performed before the first dose, 1 month after the second and third doses, and 6 months after the third dose. Participants completed a diary of possible adverse events for 14 days following each dose of vaccination (AEFV). Disease activity and current therapy were analyzed at each visit for JDM patients. In addition, serum samples from all participants were collected to test antibody concentrations against HPV16 and 18 at each visit. Participant recruitment was conducted in ten Brazilian centres. From 47 eligible JDM patients and 41 HC, 42 and 35, respectively, completed the 3-dose schedule of the vaccine, given that five JDM patients and two HC had received doses prior to their inclusion in the study. Results: The AEFVs presented by the participants were mild and in general did not differ between JDM and HC groups. No severe AEFVs were related to the vaccination. Disease activity was stable, or even improved during the follow-up. One month after the third dose of the vaccine the JDM group presented seropositivity of 100% for HPV16 and 97% for HPV18, similarly to the HC group, who presented 100% for both serotypes (p = 1.000). Six months after the third dose the seropositivity for the patient group was 94% for both HPV types. Conclusions: The HPV vaccination in this cohort of JDM patients was safe and immunogenic. Since the seropositivity against HPV16 and 18 was very high after the 3-dose schedule, this regimen should be recommended for JDM patients. Univ Med Ctr Utrecht, Wilhelmina Childrens Hosp, Dept Paediat Immunol & Rheumatol, Utrecht, Netherlands Univ Fed Parana, Hosp Clin, Dept Pediat, Gen Carneiro St 68,181, BR-80060900 Curitiba, Parana, Brazil Univ Sao Paulo, Fac Med Ribeirao Preto, Dept Paediat, Sao Paulo, Brazil Univ Fed Sao Paulo, Dept Prevent Med, Sao Paulo, Brazil Univ Sao Paulo, Fac Med, Hosp Clin, Dept Paediat Rheumatol,Inst Crianca, Sao Paulo, Brazil Univ Fed Sao Paulo, Dept Paediat Rheumatol, Sao Paulo, Brazil Hosp Estadual Infantil Nossa da Senhora, Dept Paediat Rheumatol, Vitoria, ES, Brazil Univ Fed Rio de Janeiro, Inst Puericultura & Pediat Martagao Gesteira IPPM, Dept Paediat Rheumatol, Rio De Janeiro, Brazil Univ Estado Rio De Janeiro, Dept Paediat Rheumatol, Rio De Janeiro, Brazil Hosp Infantil Albert Sabin, Dept Paediat Rheumatol, Fortaleza, Ceara, Brazil Univ Florida, Dept Paediat Immunol & Rheumatol, Coll Med, Gainesville, FL 32611 USA Hosp Crianca Brasilia Jose Alencar, Dept Paediat Rheumatol, Brasilia, DF, Brazil Univ Estadual Campinas, Dept Paediat Rheumatol, Sao Paulo, Brazil Univ Fed Rio de Janeiro, Dept Paediat Rheumatol, Rio De Janeiro, Brazil Univ Estadual Paulista, Dept Paediat Rheumatol, Botucatu, SP, Brazil Univ Sao Paulo, Fac Med Ribeirao Preto, Dept Paediat Rheumatol, Sao Paulo, Brazil Natl Inst Publ Hlth & Environm RIVM, Ctr Infect Dis Control, Bilthoven, Netherlands Hosp Canc Barretos, Fac Ciencias Saude Dr Paulo Prata FACISB, Sao Paulo, Brazil Hosp Canc Barretos, Inst Ensino & Pesquisa IEP, Sao Paulo, Brazil Univ Estadual Paulista, Dept Paediat Rheumatol, Botucatu, SP, Brazil

To assess childhood-onset systemic lupus erythematosus-related antiphospholipid syndrome(cSLE-APS) in a large Brazilian population.A retrospective observational cohort study was carried-out in 27 Pediatric Rheumatology university centers, including 1519 cSLE patients.cSLE-APS was observed in 67/1519 (4%) and was diagnosed at disease onset in 39/67 (58%). The median disease duration was 4.9 (0-17) years. Thrombosis recurrences were evidenced in 18/67 (27%) cSLE-APS patients. The most frequent thrombosis sites in cSLE-APS patients were: venous thrombosis in 40/67 (60%), especially deep vein thrombosis in 29/40 (72%); arterial thrombosis in 35/67 (52%), particularly stroke; small vessels thrombosis in 9/67 (13%) and mixed thrombosis in 3/67 (4%). Pregnancy morbidity was observed in 1/67 (1%). Non-thrombotic manifestation associated to cSLE-APS occurred in 21/67 (31%), mainly livedo reticularis in 14/67 (21%), valvar thickening in 4/67 (6%) and valvar vegetations not related to infections in 2/67 (3%). None of them had catastrophic APS. Further analysis demonstrated that the median of SLICC/ACR-DI [1(0-5) vs. 0(0-7),p 0.0001] was significantly higher in cSLE-APS patients compared to cSLE without APS. The frequencies of cerebrovascular disease (40% vs. 1%,p 0.0001), polyneuropathy (9% vs. 1%,p 0.0001), SLICC/ACR-DI ≥1 (57% vs. 27%, p 0.0001) and intravenous cyclophosphamide use (59% vs. 37%, p 0.0001) were significantly higher in the former group.Our large multicenter study demonstrated that cSLE-APS was a rare condition, occurring during disease course with a high accrual damage. Central and peripheral neuropsychiatric involvements were distinctive features of this autoimmune thrombosis.

Introduction Juvenile localized scleroderma (jlSc) usually responds well to treatment with methotrexate or mycophenolate. In case of nonresponse or partial response, tocilizumab (TOC) appears to be a promising option. Methods Participants of the Pediatric Rheumatology Email Board were asked to report patients with jlSc treated with TOC. Results Six centers responded and reported 11 patients. The mean age at disease onset was 5.5 years. Disease duration at time of the initiation of TOC was 4.5 years. A total of 5 patients had linear subtype, 2 Parry Romberg syndrome, and 1 morphea en coup de sabre. Three had a generalized subtype, 2 a mixed subtype, and 1 a limited subtype/morphea. Before starting TOC, 10 of 11 patients received methotrexate, 7/11 combination methotrexate and mycophenolate, 1 abatacept, and 1 antitumor necrosis factor therapy. The indications for starting TOC were: (i) an increase in the score of the Localized Scleroderma Activity Index (mLoSSI) in 9 patients; and (ii) evidence of increased extra-cutaneous activity in 2 patients. The mean duration of TOC therapy was 14.75 months. Three of 11 patients received TOC as monotherapy, and 8/11 as combination therapy. Therapy success was reflected by a decreased mLoSSI in 9/11 patients, no new lesion occurrence, and – with Parry Romberg syndrome subtype – no increase in facial atrophy. In 8/8 patients, both the physicians’ and patients’ global assessment of disease activity decreased. In 3/3 patients, the number of active joints decreased. The mean modified Rodnan skin score decreased from 8.7 to 5.6. Conclusions In this small cohort of patients, TOC seems to be a promising rescue medication.

Background To our knowledge the influence of ethnic background in childhood-onset SLE (cSLE) presentation was not evaluated in a large population of Latin American country. Objectives To assess demographic data, clinical manifestations, laboratory abnormalities and disease activity score in cSLE patients according to ethnic groups at diagnosis Methods This multicenter study included cSLE patients(ACR criteria) followed in 27 Pediatric Rheumatology services of Brazil. Ethnicities were classified in four groups according to the parents’ and all four grandparents’ self-reported ethnicity. The statistical analysis was performed using the Bonferroni’s correction(p Results According to ethnic groups, 1,537 cSLE patients were classified in: Caucasian (n=786), African-Latin American(n=526), Asian(n=8) and others/unknown(n=217). Comparisons between 1,312 African-Latin American and Caucasian revealed similar median age at cSLE diagnosis[12.2(2.6-18) vs. 12.1(0.3-18) years,p=0.234], time interval to diagnosis[0.25(0-12) vs. 0.3(0-10) years,p=0.034] and SLEDAI-2K score[14(0-55) vs. 14(0-63),p=0.781] in both groups. The mean number of diagnostic criteria according to SLICC(6.47±1.911 vs. 5.81±1.631, p Conclusion Our study demonstrated that disease presentation severity of African-Latin American cSLE patients is comparable to Caucasian. Mucocutaneous manifestations and autoantibodies profile were the only distinctive features of the former group. The unique mixed background of Brazilian patients probably minimized race diversity spectrum of these patients. Disclosure of Interests None declared

Made available in DSpace on 2019-10-06T16:49:50Z (GMT). No. of bitstreams: 0 Previous issue date: 2019-01-01 Objective: To evaluate the influence of ethnicity in presentation of childhood-onset systemic lupus erythematosus (cSLE) patients. Methods: This multicenter study included cSLE patients (American College of Rheumatology criteria) followed in 27 Pediatric Rheumatology services of Brazil. Ethnicities were classified in four groups according to the parents’ and all four grandparents’ self-reported ethnicity. The statistical analysis was performed using the Bonferroni’s correction (p < 0.0027). Results: According to ethnic groups, 1537 cSLE patients were classified in Caucasian (n = 786), African-Latin American (n = 526), Asian (n = 8), and others/unknown (n = 217). Comparisons between 1312 African-Latin American and Caucasian revealed similar median age at cSLE diagnosis [12.2(2.6–18) vs. 12.1(0.3–18) years, p = 0.234], time interval to diagnosis [0.25(0–12) vs. 0.3(0–10) years, p = 0.034], and SLEDAI-2K score [14(0–55) vs. 14(0–63), p = 0.781] in both groups. The mean number of diagnostic criteria according to SLICC (6.47 ± 1.911 vs. 5.81 ± 1.631, p < 0.0001) and frequencies of maculopapular lupus rash (8% vs. 3%, p < 0.0001), palate oral ulcers (17% vs. 11%, p = 0.001), tongue oral ulcers (4% vs. 1%, p = 0.001), and nonscarring alopecia (29% vs. 16%, p < 0.0001) were significantly higher in African-Latin American, whereas malar rash (45% vs. 58%, p < 0.0001) was more frequent in Caucasian. The presence of anti-phospholipid antibody (23% vs. 12%, p < 0.0001), low complement levels (58% vs. 41%, p < 0.0001), and isolated direct Coombs test (10% vs. 5%, p = 0.001) was also significantly higher in the former group. Conclusions: Our study demonstrated that disease presentation severity of African-Latin American cSLE patients is comparable with Caucasian. Mucocutaneous manifestations and autoantibodies profile were the only distinctive features of the former group. The unique mixed background of Brazilian patients probably minimized race diversity spectrum of these patients.Key Points• Our study demonstrated that disease presentation severity of African-Latin American cSLE patients is comparable with Caucasian.• Mucocutaneous manifestations and autoantibodies profile were the only distinctive features of African-Latin American cSLE patients.• African-Latin American cSLE patients had more often anti-phospholipid antibodies and hypocomplementemia.• The unique mixed background of Brazilian patients probably minimized race diversity spectrum of these patients. Pediatric Rheumatology Unit Children’s Institute Hospital das Clinicas HCFMUSP Faculdade de Medicina Universidade de Sao Paulo, Av. Dr. Eneas Carvalho Aguiar, 647 - Cerqueira César Pediatric Rheumatology Unit Hospital Jose Alencar Division of Rheumatology Hospital das Clinicas HCFMUSP Faculdade de Medicina Universidade de Sao Paulo Pediatric Rheumatology Unit Universidade Federal de Sao Paulo Pediatric Rheumatology Division Sao Paulo State University (UNESP) Pediatric Rheumatology Unit University of Campinas (UNICAMP) Pediatric Rheumatology Unit Ribeirao Preto Medical School – University of Sao Paulo Pediatric Rheumatology Unit Hospital Geral de Fortaleza Pediatric Rheumatology Unit Rio de Janeiro Federal University (IPPMG-UFRJ) Pediatric Rheumatology Unit Pedro Ernesto University Hospital Pediatric Rheumatology Unit Albert Sabin Children’s Hospital Pediatric Rheumatology Unit Hospital Darcy Vargas Pediatric Rheumatology Unit Irmandade da Santa Casa de Misericórdia de Sao Paulo Rheumatology Division - Universidade Federal do Rio de Janeiro Hospital Universitário Clementino Fraga Filho Pediatric Rheumatology Unit Lauro Vanderley University Hospital Pediatric Rheumatology Unit Federal University of Pará Pediatric Rheumatology Unit Obras Sociais Irmã Dulce Pediatric Rheumatology Unit Hospital Evangélico de Curitiba Pediatric Rheumatology Unit Hospital Criança Conceição Pediatric Rheumatology Unit Federal University of Pernambuco Pediatric Rheumatology Unit Federal University of Mato Grosso do Sul Pediatric Rheumatology Unit Federal University of Bahia Pediatric Rheumatology Unit University of Brasilia Pediatric Rheumatology Unit Federal University of Minas Gerais Pediatric Rheumatology Unit Pontifícia Catholic University of Sorocaba Pediatric Rheumatology Division Sao Paulo State University (UNESP)

To identify the underlying mechanism of amenorrhea in juvenile systemic lupus erythematosus (JSLE) patients, thirty-five (11.7%) JSLE patients with current or previous amenorrhea were consecutively selected among the 298 post-menarche patients followed in 12 Brazilian pediatric rheumatology centers. Pituitary gonadotrophins [follicle-stimulating hormone (FSH) and luteinizing hormone (LH)] and estradiol were evaluated in 32/35 patients, and prolactin and total testosterone in 29/35 patients. Patient's medical records were carefully reviewed according to demographic, clinical and therapeutic findings. The mean duration of amenorrhea was 7.2 +/- A 3.6 months. Low FSH or LH was observed in 7/32 (22%) JSLE patients and normal FSH or LH in 25 (78%). Remarkably, low levels of FSH or LH were associated with higher frequency of current amenorrhea (57% vs. 0%, P = 0.001), higher median disease activity (SLEDAI) and damage (SLICC/ACR-DI) (18 vs. 4, P = 0.011; 2 vs. 0, P = 0.037, respectively) and higher median current dose of prednisone (60 vs. 10 mg/day, P = 0.0001) compared to normal FSH or LH JSLE patients. None of them had decreased ovarian reserve and premature ovarian failure. Six of 29 (21%) patients had high levels of prolactin, and none had current amenorrhea. No correlations were observed between levels of prolactin and SLEDAI, and levels of prolactin and SLICC/ACR-DI scores (Spearman's coefficient). We have identified that amenorrhea in JSLE is associated with high dose of corticosteroids indicated for active disease due to hypothalamic-pituitary-ovary axis suppression.

Submitted by Guilherme Lemeszenski (guilherme@nead.unesp.br) on 2013-08-22T19:02:59Z No. of bitstreams: 1 S0482-50042009000500007.pdf: 368380 bytes, checksum: 9876471f4eb6a07ccdfd6857c9a16c03 (MD5) Made available in DSpace on 2013-08-22T19:02:59Z (GMT). No. of bitstreams: 1 S0482-50042009000500007.pdf: 368380 bytes, checksum: 9876471f4eb6a07ccdfd6857c9a16c03 (MD5) Previous issue date: 2009-10-01 Made available in DSpace on 2013-09-30T20:04:20Z (GMT). No. of bitstreams: 2 S0482-50042009000500007.pdf: 368380 bytes, checksum: 9876471f4eb6a07ccdfd6857c9a16c03 (MD5) S0482-50042009000500007.pdf.txt: 73931 bytes, checksum: 50b651341d444747ef2d1e5db5caa2ec (MD5) Previous issue date: 2009-10-01 Submitted by Vitor Silverio Rodrigues (vitorsrodrigues@reitoria.unesp.br) on 2014-05-20T15:16:32Z No. of bitstreams: 2 S0482-50042009000500007.pdf: 368380 bytes, checksum: 9876471f4eb6a07ccdfd6857c9a16c03 (MD5) S0482-50042009000500007.pdf.txt: 73931 bytes, checksum: 50b651341d444747ef2d1e5db5caa2ec (MD5) Made available in DSpace on 2014-05-20T15:16:32Z (GMT). No. of bitstreams: 2 S0482-50042009000500007.pdf: 368380 bytes, checksum: 9876471f4eb6a07ccdfd6857c9a16c03 (MD5) S0482-50042009000500007.pdf.txt: 73931 bytes, checksum: 50b651341d444747ef2d1e5db5caa2ec (MD5) Previous issue date: 2009-10-01 Crianças e adolescentes com doenças reumatológicas apresentam maior prevalência de doenças infecciosas quando comparados com a população em geral, em decorrência de atividade da doença, possível deficiência imunológica secundária à própria doença, ou uso de terapia imunossupressora. A vacinação é uma medida eficaz para a redução da morbidade e mortalidade nesses pacientes. O objetivo deste artigo foi realizar um consenso de eficácia e segurança das vacinas em crianças e adolescentes com doenças reumatológicas infantis baseadas em níveis de evidência científica. Imunização passiva para os pacientes e orientações para as pessoas que convivem com doentes imunodeprimidos também foram incluídas. Os 32 pediatras reumatologistas membros do Departamento de Reumatologia da Sociedade de Pediatria de São Paulo (SPSP) e/ou da Comissão de Reumatologia Pediátrica da Sociedade Brasileira de Reumatologia elaboraram o consenso, sendo que alguns desses profissionais estão envolvidos em pesquisas e publicações científicas nesta área. A pesquisa dos termos eficácia e/ou segurança das diferentes vacinas em crianças e adolescentes com doenças reumatológicas foi realizada nas bases de Medline e Scielo, de 1966 até março de 2009, incluindo revisões, estudos controlados e relatos de casos. O grau de recomendação e o nível científico de evidências dos estudos foram classificados em quatro níveis para cada vacina. de um modo geral, as vacinas inativadas e de componentes são seguras nos pacientes com doenças reumatológicas, mesmo em uso de terapias imunossupressoras. Entretanto, vacinas com agentes vivos atenuados são, em geral, contraindicadas para os pacientes imunossuprimidos. Incidence of infectious diseases is higher in children and adolescents with rheumatic diseases than in the general population due to disease activity, possible immune deficiency secondary to the disease itself, or the use of immunosuppressive drugs. Vaccination is effective in reducing morbidity and mortality in those patients. The objective of this study was to establish an evidence-based consensus on the efficacy and safety of vaccination in children and adolescents with rheumatic diseases. Passive immunization of patients and guidelines for people who live with immunosuppressed patients were also included. The 32 pediatric rheumatologists of the Rheumatology Department of the Pediatrics Society of São Paulo, (SPSP, from the Portuguese), São Paulo, SP, Brazil, and/or the Commission on Pediatrics Rheumatology of the Brazilian Society of Rheumatology are responsible for this consensus; some of those professionals are involved on research and scientific publications in this field. The words efficacy and/or safety of different vaccines in children and adolescents with rheumatologic diseases were searched in Medline and Scielo data bases from 1966 to March 2009, including reviews, controlled studies, and case reports. The degree of recommendation and the scientific evidence of the studies were classified in four levels for each vaccine. As a rule, inactive and protein components vaccines are safe for patients with rheumatologic diseases, even in the presence of immunosuppressive therapy. However, live attenuated vaccines are, in general, contraindicated for immunosuppressed patients. Universidade de São Paulo Faculdade de Medicina Instituto da Criança Universidade de São Paulo USP Faculdade de Medicina de Ribeirão Preto FMUSP Universidade Estadual Paulista Faculdade de Medicina de Botucatu Santa Casa de Misericórdia de São Paulo Hospital Municipal Infantil Menino Jesus Universidade Estadual de Campinas Hospital Servidor Público Universidade Federal da Bahia (UFBA) Hospital Materno Infantil de Goiás Universidade Federal do Pará (UFPA) Universidade Federal de Mato Grosso do Sul Universidade Federal do Rio de Janeiro (UFRJ) UFRJ Instituto de Puericultura Martagão Gesteira Universidade Estadual Paulista Faculdade de Medicina de Botucatu

In this study, we evaluate the distribution of nitric oxide (NO) in the serum of juvenile idiopathic arthritis (JIA) patients, correlating it with parameters of the severity of the disease. Ninety-seven patients with mean age 11.7 years and disease duration 4.8 years, showing active disease or not, grouped as oligoarticular (n = 34), polyarticular (n = 29) and systemic (n = 34) group, presenting uveitis and positive RF with erosive arthritis or active disease and erosions had significantly high levels of NO than the inactive ones. NO correlated with TNF-α in the oligoarticular subtype (P < 0.03), with pain in the polyarticular subtype with active disease (P < 0.04) and with ESR in the systemic subtype with active disease (P < 0.03). TNF-α concentration was high in all patients with active disease, accompanying NO production. The data confirm the production of NO in JIA patients, indicating a possible positive correlation between the production of NO and severity of the disease.

BACKGROUND: Most data for treatment of dermatomyositis and juvenile dermatomyositis are from anecdotal, non-randomised case series. We aimed to compare, in a randomised trial, the efficacy and safety of prednisone alone with that of prednisone plus either methotrexate or ciclosporin in children with new-onset juvenile dermatomyositis.METHODS: We did a randomised trial at 54 centres in 22 countries. We enrolled patients aged 18 years or younger with new-onset juvenile dermatomyositis who had received no previous treatment and did not have cutaneous or gastrointestinal ulceration. We randomly allocated 139 patients via a computer-based system to prednisone alone or in combination with either ciclosporin or methotrexate. We did not mask patients or investigators to treatment assignments. Our primary outcomes were the proportion of patients achieving a juvenile dermatomyositis PRINTO 20 level of improvement (20% improvement in three of six core set variables at 6 months), time to clinical remission, and time to treatment failure. We compared the three treatment groups with the Kruskal-Wallis test and Friedman's test, and we analysed survival with Kaplan-Meier curves and the log-rank test. Analysis was by intention to treat. Here, we present results after at least 2 years of treatment (induction and maintenance phases). This trial is registered with ClinicalTrials.gov, number NCT00323960.FINDINGS: Between May 31, 2006, and Nov 12, 2010, 47 patients were randomly assigned prednisone alone, 46 were allocated prednisone plus ciclosporin, and 46 were randomised prednisone plus methotrexate. Median duration of follow-up was 35·5 months. At month 6, 24 (51%) of 47 patients assigned prednisone, 32 (70%) of 46 allocated prednisone plus ciclosporin, and 33 (72%) of 46 administered prednisone plus methotrexate achieved a juvenile dermatomyositis PRINTO 20 improvement (p=0·0228). Median time to clinical remission was 41·9 months in patients assigned prednisone plus methotrexate but was not observable in the other two treatment groups (2·45 fold [95% CI 1·2-5·0] increase with prednisone plus methotrexate; p=0·012). Median time to treatment failure was 16·7 months in patients allocated prednisone, 53·3 months in those assigned prednisone plus ciclosporin, but was not observable in patients randomised to prednisone plus methotrexate (1·95 fold [95% CI 1·20-3·15] increase with prednisone; p=0·009). Median time to prednisone discontinuation was 35·8 months with prednisone alone compared with 29·4-29·7 months in the combination groups (p=0·002). A significantly greater proportion of patients assigned prednisone plus ciclosporin had adverse events, affecting the skin and subcutaneous tissues, gastrointestinal system, and general disorders. Infections and infestations were significantly increased in patients assigned prednisone plus ciclosporin and prednisone plus methotrexate. No patients died during the study.INTERPRETATION: Combined treatment with prednisone and either ciclosporin or methotrexate was more effective than prednisone alone. The safety profile and steroid-sparing effect favoured the combination of prednisone plus methotrexate.FUNDING: Italian Agency of Drug Evaluation, Istituto Giannina Gaslini (Genoa, Italy), Myositis Association (USA).

OBJETIVO: O objetivo deste estudo foi comparar a resposta dilatadora dependente e independente do endotélio em pacientes portadores de esclerose sistêmica limitada (ESL) com aquela de indivíduos sadios de mesmo gênero, idade e cor. MÉTODOS: Vinte mulheres adultas, não obesas, não tabagistas, não diabéticas, não dislipidêmicas, não hipertensas, que preencheram os critérios para esclerose sistêmica (ES) segundo o American College of Rheumatology, foram submetidas ao exame de Doppler de artéria braquial do membro superior direito. Foi analisada a resposta dilatadora, dependente do endotélio, após isquemia induzida com esfigmomanômetro por cinco minutos no braço direito, e a resposta dilatadora, independente do endotélio, após administração de 300 mcg de nitroglicerina (NTG) sublingual. Esses resultados foram comparados com a resposta obtida em indivíduos sadios. RESULTADOS: O diâmetro longitudinal da artéria braquial (DAB) foi significativamente menor na fase basal 1 nos pacientes com ESL (3,57 ± 0,52 mm e 3,93 ± 0,39 mm, respectivamente no grupo paciente (P) e grupo-controle (C), P = 0,005). Não foi encontrada diferença estatisticamente significativa entre a velocidade das hemácias (VH) após isquemia/hiperemia reativa (HR) e após NTG (110,2 ± 43,86 cm/s vs. 102,0 ± 25,89 cm/s e 63,80 ± 17,69 cm/s vs. 65,4 ± 12,90 cm/s nos grupos P e C, após HR e NTG, respectivamente). Também não foi encontrada diferença significativa entre o DAB após HR e após NTG (3,77 ± 0,59 mm vs. 4,14 ± 0,49 mm e 4,44 ± 0,64 mm vs. 4,70 ± 0,58 mm nos grupos P e C, após HR e NTG, respectivamente). CONCLUSÃO: Embora o grupo de pacientes com ESL tenha apresentado menor DAB basal, a resposta dilatadora dependente e independente do endotélio se manteve preservada em ambos os grupos. OBJECTIVES: The aim of this study was to compare the brachial artery endothelium-dependent and endothelium-independent dilating responses in patients with limited systemic sclerosis (LSSc) with those of healthy subjects of the same gender, age and color. METHODS: Twenty adult, non-obese, non-smoker, non-diabetic, non-dyslipidemic, and non-hypertensive women, who fulfilled the American College of Rheumatology criteria for the diagnosis of SSc, were submitted to right brachial artery Doppler ultrasound. The vasodilating responses were analyzed as follows: the endothelium-dependent dilating response, after a 5-minute ischemia in the right arm; and the endothelium-independent dilating response, after administering 300 mcg of nitroglycerin (NTG) sublingually. The results were compared with the response obtained in healthy subjects. RESULTS: Brachial artery longitudinal diameter was significantly low at baseline 1: 3.57 ± 0.52 mm and 3.93 ± 0.39 mm for the LSSc group and the control group, respectively, P = 0.005. The vascular reactivity after the ischemia/reactive hyperemia and the NTG showed no significant difference between the groups (8.60 ± 5.45 mm vs. 9.26 ± 5.91 mm and 25.01 ± 12.55 mm vs. 19.59 ± 7.94 mm for the LSSc and control groups, respectively). Also, no statistically significant difference was found between red blood cell velocity (RBCV) after reactive hyperemia and NTG (110.2 ± 43.86 cm/s vs. 102.0 ± 25.89 cm/s and 63.80 ± 17.69 cm/s vs. 65.4 ± 12.90 cm/s in the LSSc and control groups, respectively). CONCLUSION: Although the LSSc group showed lower brachial artery diameter, the endotheliumdependent and the endothelium-independent dilating responses were preserved in both groups.

To evaluate the prevalence of genetic defects in clinically suspected autoinflammatory syndromes (AIS) in a Brazilian multicenter study. The study included 102 patients with a clinical diagnosis of Cryopyrin Associated Periodic Syndromes (CAPS), TNF Receptor Associated Periodic Syndrome (TRAPS), Familial Mediterranean Fever (FMF), Mevalonate Kinase Deficiency (MKD) and Pediatric Granulomatous Arthritis (PGA). One of the five AIS-related genes (NLRP3, TNFRSF1A, MEFV, MVK and NOD2) was evaluated in each patient by direct DNA sequencing, based on the most probable clinical suspect. Clinical diagnoses of the 102 patients were: CAPS (n = 28), TRAPS (n = 31), FMF (n = 17), MKD (n = 17) and PGA (n = 9). Of them, 27/102 (26 %) had a confirmed genetic diagnosis: 6/28 (21 %) CAPS patients, 7/31 (23 %) TRAPS, 3/17 (18 %) FMF, 3/17 (18 %) MKD and 8/9 (89 %) PGA. We have found that approximately one third of the Brazilian patients with a clinical suspicion of AIS have a confirmed genetic diagnosis.

Objective To validate and promulgate a core set of outcome measures for the evaluation of response to treatment in patients with juvenile systemic lupus erythematosus (SLE). Methods In 2001, a preliminary consensus-derived core set of measures for evaluating the response to therapy in juvenile SLE was established. In the present study, the core set was validated through an evidence-based, large-scale data collection process that led to the enrollment of 557 patients from 39 different countries. Consecutive patients with active disease were assessed at baseline and after 6 months. The validation procedures included assessment of feasibility, responsiveness, discriminant and construct ability, agreement in the evaluation of response to therapy between physicians and parents, redundancy, internal consistency, and ability to predict a therapeutic response. Results The following clinical measures were found to be feasible and to have good construct validity, discriminative ability, and internal consistency; furthermore, they were not redundant, proved responsive to clinically important changes in disease activity, and were associated strongly with treatment outcome and thus were included in the final core set: 1) physician's global assessment of disease activity, 2) global disease activity measure, 3) 24-hour proteinuria, 4) parent's global assessment of the patient's overall well-being, and 5) health-related quality of life assessment. Conclusion The members of PRINTO propose a core set of criteria for the evaluation of response to therapy that is scientifically and clinically relevant and statistically validated. The core set will help standardize the conduct and reporting of clinical trials and assist practitioners in deciding whether a patient with juvenile SLE has responded adequately to therapy.

Osteochondrosis or osteochondritis are alterations characterized by failure in sub-chondral ossification and affects the immature skeleton of children and teenagers. It may affects any epiphysis, apophysis or short bone with similar radiological and anatomopathological characteristics. It has no defined etiology, although there are some risk factors as constitutional predisposing, trauma and ischemia. Femoral osteochondrosis is usually unilateral and can be misdiagnosed as juvenile idiopathic arthritis (JIA). The authors report two patients with bilateral femoral condyle osteochondrosis that were treated as JIA.

Background Cross-sectional study, in which were evaluated the bone mineral density of 53 patients from the Rheumatology ambulatory, diagnosed with JSLE in their childhood or teenage years, according to American College of Rheumatology criteria. Aim Evaluate the bone mass in a group of patients diagnosed with juvenile systemic lupus erythematosus (JSLE), which carry high risks of developing low bone mass, as well as verifying alterations on calcium metabolism. Method