Abstract Sjogren's syndrome (SS) is a complex autoimmune disease characterized by lymphocytic infiltration of salivary and lacrimal glands, resulting in sicca symptoms. Additionally, SS presents with neurological manifestations that significantly impact the nervous system. This review aims to provide a comprehensive overview of the neurological aspects of SSj, covering both the peripheral and central nervous system involvement, while emphasizing diagnosis, treatment, and prognosis. Sjogrens Sjogren s (SS glands symptoms Additionally SSj involvement diagnosis treatment prognosis

Resumo A síndrome de Sjogren (SS) é uma doença autoimune complexa caracterizada pela infiltração linfocítica das glândulas salivares e lacrimais, resultando em sintomas sicca. Além disso, a SS apresenta manifestações neurológicas que afetam significativamente o sistema nervoso. Esta revisão tem como objetivo fornecer uma visão abrangente dos aspectos neurológicos da SSj, abordando tanto o envolvimento do sistema nervoso periférico quanto do central, com ênfase no diagnóstico, tratamento e prognóstico. (SS lacrimais sicca disso SSj central diagnóstico prognóstico

Abstract Background Few studies have assessed elderly patients with Takayasu’s arteritis (TAK). Objectives To evaluate the progression of TAK in different age groups and its possible effects on drug treatment and disease activity. Methods This cross-sectional and retrospective cohort study included 66 TAK patients. Patients were interviewed and data of the 12 preceding months were collected from electronic medical records. The patients were divided into four quartiles according to current age and compared for clinical and laboratory data, treatment, comorbidities, disease status, and functional status. Statistical significance was set at p<0.05. Results The groups were Q1(22-36 years, n=16), Q2(37-42 years, n=18), Q3(43-49 years, n=17), and Q4(51-66 years, n=15). The frequency of patients with disease activity, fatigue, comorbidities and vascular impairments, and the TAK disease extent index were also comparable between the groups. With age, disease duration was longer (p=0.001), fewer patients used prednisone (Q1:43.8%, Q2:33.3%, Q3:11.8%, and Q4:6.7%; p=0.049) and immunosuppressive drugs [Q1:100.0%, Q2:66.7%, Q3:58.8%, and Q4:46.7%; Q1 versus Q3 (p=0.043), and Q1 versus Q4 (p=0.005) in post-hoc analyses], and patients had greater functional status impairment (Q2 versus Q3, p=0.003). In addition, the levels of disease damage, new TAK symptoms, and complications in the preceding 12 months were not different between the groups. Conclusions Older patients with TAK require minimal drug treatment, and have greater impairment of functional status, which may be attributed to aging-related factors. Takayasus Takayasu s TAK. . (TAK) activity crosssectional cross sectional 6 1 records p005 p 0 05 p<0.05 Q12236 Q 22 36 Q1(22-3 years n=16, n16 n n=16 , 16 n=16) Q23742 Q2 37 42 Q2(37-4 n=18, n18 n=18 18 n=18) Q34349 43 49 Q3(43-4 n=17, n17 n=17 17 n=17) Q45166 51 Q4(51-6 n=15. n15 n=15 15 n=15) fatigue impairments p=0.001, p0001 p=0.001 001 (p=0.001) Q143.8%, Q1438 43.8%, 8 (Q1:43.8% Q233.3%, Q2333 33.3%, 33 3 Q2:33.3% Q311.8%, Q3118 11.8%, 11 Q3:11.8% Q46.7% Q467 6.7% 7 Q4:6.7% p=0.049 p0049 049 Q1100.0%, Q11000 100.0%, 100 [Q1:100.0% Q266.7%, Q2667 66.7%, Q2:66.7% Q358.8%, Q3588 58.8%, 58 Q3:58.8% Q446.7% Q4467 46.7% 46 Q4:46.7% p=0.043, p0043 p=0.043 043 (p=0.043) p=0.005 p0005 005 (p=0.005 posthoc post hoc analyses, analyses analyses] (Q p=0.003. p0003 p=0.003 003 p=0.003) addition damage symptoms agingrelated aging related factors (TAK p00 p<0.0 Q1223 2 Q1(22- n1 n=1 Q2374 4 Q2(37- Q3434 Q3(43- Q4516 5 Q4(51- p000 p=0.00 00 (p=0.001 Q143 Q143.8% 438 43.8% (Q1:43.8 Q233 Q233.3% 333 33.3% Q2:33.3 Q311 Q311.8% 118 11.8% Q3:11.8 Q46 Q46.7 67 6.7 Q4:6.7 p=0.04 p004 04 Q1100 Q1100.0% 1000 100.0% 10 [Q1:100.0 Q266 Q266.7% 667 66.7% Q2:66.7 Q358 Q358.8% 588 58.8% Q3:58.8 Q446 Q446.7 467 46.7 Q4:46.7 (p=0.043 (p=0.00 p0 p<0. Q122 Q1(22 n= Q237 Q2(37 Q343 Q3(43 Q451 Q4(51 p=0.0 Q14 Q143.8 43.8 (Q1:43. Q23 Q233.3 33.3 Q2:33. Q31 Q311.8 11.8 Q3:11. Q46. 6. Q4:6. Q110 Q1100.0 100.0 [Q1:100. Q26 Q266.7 66.7 Q2:66. Q35 Q358.8 58.8 Q3:58. Q44 Q446. 46. Q4:46. (p=0.04 (p=0.0 p<0 Q12 Q1(2 Q2(3 Q34 Q3(4 Q45 Q4(5 p=0. Q143. 43. (Q1:43 Q233. 33. Q2:33 Q311. 11. Q3:11 Q4:6 Q11 Q1100. 100. [Q1:100 Q266. 66. Q2:66 Q358. 58. Q3:58 Q4:46 (p=0. p< Q1( Q2( Q3( Q4( p=0 (Q1:4 Q2:3 Q3:1 Q4: [Q1:10 Q2:6 Q3:5 Q4:4 (p=0 p= (Q1: Q2: Q3: [Q1:1 (p= (Q1 [Q1: (p [Q1 [Q

Resumo Fundamentos Poucos estudos avaliaram pacientes idosos com Arterite de Takayasu (AT). Objetivo Avaliar o progresso de AT em diferentes grupos etários em seus possíveis efeitos sobre o tratamento medicamentoso e atividade da doença. Métodos este estudo transversal, retrospectivo, do tipo coorte incluiu 66 pacientes com AT. Os pacientes foram entrevistados, e dados dos 12 meses anteriores foram coletados dos prontuários médicos eletrônicos. Os pacientes foram divididos em quatro quartis de acordo com idade atual, e comparados quanto aos dados clínicos e laboratoriais, tratamento, comorbidades, status da doença, e status funcional. Um p<0,05 foi estabelecido como estatisticamente significativo. Resultados Os grupos foram definidos como Q1(22-36 anos, n=16), Q2(37-42 anos, n=18), Q3(43-49 anos, n=17), e Q4(51-66 anos, n=15). A frequência de pacientes com atividade da doença, fadiga, comorbidades e comprometimentos vasculares, e o índice de extensão da doença (DEI. Tak) foram comparáveis entre os grupos. Pacientes com idade mais avançada apresentaram maior duração da doença (p=0,001) e maior comprometimento do status funcional (Q2 versus Q3, p=0,003); menos pacientes usaram prednisona (Q1:43,8%; Q2:33,3%; Q3:11,8%; e Q4:6,7%; p=0,049) e agentes imunossupressores [Q1:100,0%; Q2:66,7%; Q3:58,8% e Q4:46,7%; Q1 versus Q3 (p=0,043) e Q1 versus Q4 (p=0,005) nas análises post-hoc]. Além disso, os níveis de danos da doença, sintomas de uma nova ocorrência de AT, e complicações nos 12 meses precedentes não foram diferentes entre os grupos. Conclusão Pacientes com AT e idade mais avançada requerem mínima intervenção medicamentosa e apresentam maior comprometimento no status funcional, o que pode ser atribuído a fatores relacionados ao envelhecimento. . (AT) transversal retrospectivo 6 entrevistados 1 eletrônicos atual laboratoriais p005 p 0 05 p<0,0 significativo Q12236 Q 22 36 Q1(22-3 anos n=16, n16 n n=16 , 16 n=16) Q23742 Q2 37 42 Q2(37-4 n=18, n18 n=18 18 n=18) Q34349 43 49 Q3(43-4 n=17, n17 n=17 17 n=17) Q45166 51 Q4(51-6 n=15. n15 n=15 15 n=15) fadiga vasculares DEI. DEI (DEI Tak p=0,001 p0001 001 (p=0,001 (Q p=0,003 p0003 003 p=0,003) Q143,8% Q1438 43,8% 8 (Q1:43,8% Q233,3% Q2333 33,3% 33 3 Q2:33,3% Q311,8% Q3118 11,8% 11 Q3:11,8% Q46,7% Q467 6,7% 7 Q4:6,7% p=0,049 p0049 049 Q1100,0% Q11000 100,0% 100 [Q1:100,0% Q266,7% Q2667 66,7% Q2:66,7% Q358,8% Q3588 58,8% 58 Q3:58,8 Q446,7% Q4467 46,7% 46 Q4:46,7% p=0,043 p0043 043 (p=0,043 p=0,005 p0005 005 (p=0,005 posthoc. posthoc post hoc post-hoc] disso envelhecimento (AT p00 p<0, Q1223 2 Q1(22- n1 n=1 Q2374 4 Q2(37- Q3434 Q3(43- Q4516 5 Q4(51- p=0,00 p000 00 (p=0,00 Q143 Q143,8 438 43,8 (Q1:43,8 Q233 Q233,3 333 33,3 Q2:33,3 Q311 Q311,8 118 11,8 Q3:11,8 Q46 Q46,7 67 6,7 Q4:6,7 p=0,04 p004 04 Q1100 Q1100,0 1000 100,0 10 [Q1:100,0 Q266 Q266,7 667 66,7 Q2:66,7 Q358 Q358,8 588 58,8 Q3:58, Q446 Q446,7 467 46,7 Q4:46,7 (p=0,04 post-hoc p0 p<0 Q122 Q1(22 n= Q237 Q2(37 Q343 Q3(43 Q451 Q4(51 p=0,0 (p=0,0 Q14 Q143, 43, (Q1:43, Q23 Q233, 33, Q2:33, Q31 Q311, 11, Q3:11, Q46, 6, Q4:6, Q110 Q1100, 100, [Q1:100, Q26 Q266, 66, Q2:66, Q35 Q358, 58, Q3:58 Q44 Q446, 46, Q4:46, p< Q12 Q1(2 Q2(3 Q34 Q3(4 Q45 Q4(5 p=0, (p=0, (Q1:43 Q2:33 Q3:11 Q4:6 Q11 [Q1:100 Q2:66 Q3:5 Q4:46 Q1( Q2( Q3( Q4( p=0 (p=0 (Q1:4 Q2:3 Q3:1 Q4: [Q1:10 Q2:6 Q3: Q4:4 p= (p= (Q1: Q2: [Q1:1 (p (Q1 [Q1: [Q1 [Q

Abstract Background Giant cell arteritis (GCA) is the most common primary systemic vasculitis in people 50 years of age and over, and it is considered a medical emergency due to the potential risk of permanent visual loss. Color Doppler ultrasound (CDU) of the temporal arteries is a rapid, noninvasive method to diagnose GCA. This study aims to determine the diagnostic accuracy of the halo sign in temporal arteries by CDU in people with suspected GCA. Methods The systematic literature review included the search for publications in the following electronic databases: PubMed, Embase, CENTRAL, LILACS, WHO ICTRP, ClinicalTrials.gov, gray literature up to December 2022, and no date or language restrictions were applied. We analyzed studies including patients over 50 years of age with suspected GCA evaluating CDU of temporal arteries as a diagnostic tool against clinical diagnosis as a standard reference. Paper titles and abstracts were selected by two investigators independently for all available records. The quality of the studies was assessed using the Quality of Diagnostic Accuracy Studies tool (QUADAS-2) and the R software (version 4.2.1) was used for data analysis. The protocol of this review is registered with PROSPERO (CRD42016033079). Results Twenty-two studies including 2893 participants with suspected GCA who underwent temporal artery CDU were evaluated. The primary analysis results showed a sensitivity of 0.76 [95% confidence interval (95 CI) 0.69–0.81] and specificity of 0.93 (95 CI 0.89–0.95) when the halo sign was compared to clinical diagnosis. The sensitivity value of 0.84 (95 CI 0.72–0.92) and specificity of 0.95 (95 CI 0.88–0.98) were found in five studies involving 1037 participants that analyzed the halo sign and temporal artery compression sign. A sensitivity of 0.86 (95 CI 0.78–0.91) and specificity of 0.95 (95 CI 0.89–0.98) were found in four studies with 603 participants where the halo sign was evaluated CDU on temporal and axillary arteries. Conclusion The detection of the halo sign by CDU of temporal arteries has good accuracy for the diagnosis of cranial GCA. The compression sign in temporal arteries and the addition of axillary arteries assessment improves the diagnostic performance of CDU for GCA. Trial registration PROSPERO CRD42016046860. (GCA 5 loss (CDU rapid databases PubMed Embase CENTRAL LILACS ICTRP ClinicalTrialsgov ClinicalTrials gov ClinicalTrials.gov 2022 applied reference records QUADAS2 QUADAS 2 (QUADAS-2 version 4.2.1 421 4 1 CRD42016033079. CRD42016033079 CRD . (CRD42016033079) Twentytwo Twenty 289 076 0 76 0.7 95% 95 [95 (9 0.69–0.81 069081 69 81 093 93 0.9 0.89–0.95 089095 89 084 84 0.8 0.72–0.92 072092 72 92 095 0.88–0.98 088098 88 98 103 086 86 0.78–0.91 078091 78 91 0.89–0.98 089098 60 CRD42016046860 202 (QUADAS- 4.2. 42 CRD4201603307 (CRD42016033079 28 07 7 0. 9 [9 ( 0.69–0.8 06908 6 8 09 0.89–0.9 08909 08 0.72–0.9 07209 0.88–0.9 08809 10 0.78–0.9 07809 CRD4201604686 20 (QUADAS 4.2 CRD420160330 (CRD4201603307 [ 0.69–0. 0690 0.89–0. 0890 0.72–0. 0720 0.88–0. 0880 0.78–0. 0780 CRD420160468 4. CRD42016033 (CRD420160330 0.69–0 069 0.89–0 089 0.72–0 072 0.88–0 088 0.78–0 078 CRD42016046 CRD4201603 (CRD42016033 0.69– 06 0.89– 0.72– 0.88– 0.78– CRD4201604 CRD420160 (CRD4201603 0.69 0.89 0.72 0.88 0.78 CRD42016 (CRD420160 0.6 CRD4201 (CRD42016 CRD420 (CRD4201 CRD42 (CRD420 CRD4 (CRD42 (CRD4 (CRD

Abstract Background: Modifiable cardiovascular risk factors (MCRFs), such as those related to aerobic capacity, muscle strength, physical activity, and body composition, have been poorly studied in Takayasu arteritis (TAK). Therefore, the aim of the study was to investigate MCRFs and their relationships with disease status and comorbidities among patients with TAK. Methods: A multicenter cross-sectional study was conducted between 2019 and 2020, in which 20 adult women with TAK were compared with 16 healthy controls matched by gender, age, and body mass index. The following parameters were analyzed: aerobic capacity by cardiopulmonary test; muscle function by timed-stands test, timed up-and-go test, and handgrip test; muscle strength by one-repetition maximum test and handgrip test; body composition by densitometry; physical activity and metabolic equivalent by IPAQ, quality of life by HAQ and SF-36; disease activity by ITAS2010 and NIH score; and presence of comorbidities. Results: Patients with TAK had a mean age of 41.5 (38.0-46.3) years, disease duration of 16.0 (9.5-20.0) years, and a mean BMI of 27.7±4.5 kg/m2. Three out of the 20 patients with TAK had active disease. Regarding comorbidities, 16 patients had systemic arterial hypertension, 11 had dyslipidemia, and two had type 2 diabetes mellitus, while the control group had no comorbidities. TAK had a significant reduction in aerobic capacity (absolute and relative VO2 peak), muscle strength in the lower limbs, increased visceral adipose tissue, waist-to-hip ratio, reduced walking capacity, decreased weekly metabolic equivalent, and quality of life (P< 0.05) as compared to controls. However, there were no correlations between these MCRFs parameters and disease activity. Conclusions: TAK show impairment in MCRFs; therefore, strategies able to improve MCRF should be considered in this disease.

Abstract Background: Endothelial progenitor cells (EPCs) are responsible for endothelial damage repair. Takayasu's arteritis (TA) is a chronic inflammatory disease that affects large vessels. The aim of the study was to evaluate the number of EPCs and the levels of vascular endothelial growth factor (VEGF) and the relationship of these variables in patients with TA. Methods: Thirty women with TA and 30 healthy controls were included. EPCs were assessed by flow cytometry and cell culture and VEGF quantification was performed by commercial ELISA kits. Results: Ages of patients and controls were similar. The number of EPCs in patients and controls (median (interquartile range) were 0.0073% (0.0081%) vs. 0.0062% (0.0089%), p = 0.779 by flow cytometry and 27.0 (42.3) colony forming units (CFUs) vs. 27.0 (20.5) CFUs, p = 0.473 by cells culture, respectively. VEGF levels in patients and controls was 274.5 (395.5) pg/ml vs. 243.5 (255.3) pg/ml, p = 0.460. There was no difference in the number of EPCs and VEGF level between patients with active and inactive disease. There was a tendency of the number of angioblast-like EPCs in patients taking anti-TNFs to be higher; and in patients using methotrexate to be lower. Conclusion: No significant difference was found in the quantification of EPCs and VEGF levels in TA patients compared to controls, and no difference was observed between patients with active and inactive disease.

Resumo O objetivo destas recomendações é orientar o tratamento apropriado de indução em pacientes com vasculite associada a anticorpos anticitoplasma de neutrófilos (VAA) ativa. As recomendações propostas pelo Comitê de Vasculopatias da Sociedade Brasileira de Reumatologia para a terapia de indução para vasculites associadas aos anticorpos anticitoplasma de neutrófilos (VAA), inclusive granulomatose com poliangiite, poliangiite microscópica e vasculite limitada ao rim, foram baseadas em uma revisão sistemática da literatura e na opinião de especialistas. A revisão da literatura foi feita com as bases de dados Medline (PubMed), Embase e Cochrane para consultar artigos até outubro de 2016. As diretrizes Prisma (Preferred Reporting Items for Systematic Reviews and Meta-Analyses - Principais itens para reportar revisões sistemáticas e metanálises) foram usadas para a revisão sistemática e os artigos foram avaliados de acordo com os níveis de evidência Oxford. Dezesseis recomendações foram feitas em relação a diferentes aspectos da terapia de indução para VAA. O objetivo dessas recomendações é servir como um guia para decisões terapêuticas por profissionais da saúde no tratamento de pacientes com VAA que apresentem a doença ativa.

Abstract The purpose of these recommendations is to guide the appropriate induction treatment of antineutrophil cytoplasmic antibody-associated vasculitis (AAV) patients with active disease. The recommendations proposed by the Vasculopathies Committee of the Brazilian Society Rheumatology for induction therapy of AAV, including granulomatosis with polyangiitis, microscopic polyangiitis and renal-limited vasculitis, were based on systematic literature review and expert opinion. Literature review was performed using Medline (PubMed), EMBASE and Cochrane database to retrieve articles until October 2016. PRISMA guidelines were used for the systematic review and articles were assessed according to the Oxford levels of evidence. Sixteen recommendations were made regarding different aspects of induction therapy for AAV. The purpose of these recommendations is to serve as a guide for therapeutic decisions by health care professionals in the management of AAV patients presenting active disease.

RESUMO Objetivo: Avaliar os dados de seguimento em longo prazo em relação à eficácia e toxicidade do tratamento com leflunomida em pacientes com arterite de Takayasu (AT) previamente recrutados no estudo aberto original dos efeitos de curto prazo da leflunomida na AT. Métodos: Fez-se um estudo longitudinal aberto de longo prazo com pacientes que preencheram os critérios para AT da American College of Rheumatology de 1990 e que participaram de um estudo anterior que avaliou a eficácia em curto prazo da leflunomida na AT. Obtiveram-se informações completas do seguimento de 12 dos 15 pacientes incluídos no estudo original. A atividade da doença foi avaliada pelos critérios de Kerr e pelo Indian Takayasu Activity Score 2010 (ITAS2010). Resultados: O tempo médio de seguimento foi de 43,0 ± 7,6 meses. Cinco (41,6%) pacientes com AT permaneceram em tratamento com leflunomida, enquanto sete (58,3%) tiveram de mudar para outro tratamento em razão da falha em prevenir recidivas em seis pacientes e toxicidade em um paciente. Não foram encontradas diferenças significativas entre os pacientes que continuaram o tratamento com leflunomida e aqueles que mudaram para outro agente em relação à idade no início do estudo, tempo desde o diagnóstico, dose diária de prednisona no início do estudo, ITAS2010 inicial, valor médio ou máximo de VHS e PCR, e dose de prednisona cumulativa no fim do estudo. Entre os dois pacientes com AT que mudaram de leflunomida para outro agente, dois tiveram uma recidiva clínica e precisaram mudar de tratamento. Conclusão: A leflunomida levou à remissão sustentada em aproximadamente metade dos pacientes por um período médio de 12 meses e foi bem tolerada pelos pacientes com AT.

ABSTRACT Objective: To evaluate the extended follow-up data on efficacy and toxicity of leflunomide therapy in Takayasu arteritis (TA) patients previously enrolled in the original open-label study of short-term effects of leflunomide in TA. Methods: An open-label long-term longitudinal study was performed in TA patients who fulfilled the 1990 American College of Rheumatology criteria for TA and had participated in a previous study that evaluated short-term efficacy of leflunomide in TA. Complete follow-up information could be retrieved from 12 out of 15 patients enrolled in the original study. Disease activity was evaluated by Kerr's criteria and by the Indian Takayasu Activity Score 2010 (ITAS2010). Results: The mean follow up time was 43.0 ± 7.6 months and 5 (41.6%) TA patients remained on leflunomide therapy while 7 (58.3%) TA patients had to change to another therapy due to failure to prevent relapses in 6 patients and toxicity in one patient. No significant differences were found between patients who remained on leflunomide therapy and those who changed to another agent regarding age at study entry, time since diagnosis, prednisone daily dose at study entry, baseline ITAS2010, mean or maximum ESR and CRP, and cumulative prednisone dose at study end. Among TA patients who had changed leflunomide to another agent, two had an additional clinical relapse and needed to change therapy. Conclusion: Leflunomide led to sustained remission in approximately half of patients at a mean time of 12 months and was well tolerated by TA patients.

A síndrome do anticorpo antifosfolipídeo (SAF) é uma doença sistêmica autoimune caracterizada por trombose arterial e venosa, morbidade gestacional e presença de níveis séricos de anticorpos antifosfolipídeos elevados e persistentemente positivos. O tratamento da SAF ainda é sujeito a controvérsias, já que qualquer decisão terapêutica potencialmente irá confrontar-se com o risco de uma cobertura antitrombótica insuficiente ou com o risco excessivo associado à anticoagulação e seus principais efeitos adversos. Esta diretriz foi elaborada a partir de nove questões clínicas relevantes e relacionadas ao tratamento da SAF pela Comissão de Vasculopatias da Sociedade Brasileira de Reumatologia. O objetivo deste trabalho foi criar uma diretriz que incluísse as questões mais relevantes e controversas no tratamento da SAF, com base na melhor evidência científica disponível. As questões foram estruturadas por meio do P.I.C.O. (paciente, intervenção ou indicador, comparação e outcome/desfecho), o que possibilitou a geração de estratégias de busca da evidência nas principais bases primárias de informação científica (MEDLINE/Pubmed, Embase, Lilacs/Scielo, Cochrane Library, Premedline via OVID). Também realizou-se busca manual da evidência e de teses (BDTD e IBICT). A evidência recuperada foi selecionada a partir da avaliação crítica, utilizando instrumentos (escores) discriminatórios de acordo com a categoria da questão terapêutica (JADAD para ensaios clínicos randomizados e New Castle Ottawa Scale para estudos não randomizados). Após definir os estudos potenciais para sustento das recomendações, eles foram selecionados pela força da evidência e pelo grau de recomendação, segundo a classificação de Oxford.

The antiphospholipid syndrome (APS) is a systemic autoimmune disease characterized by arterial and venous thrombosis, gestational morbidity and presence of elevated and persistently positive serum titers of antiphospholipid antibodies. The treatment of APS is still controversial, because any therapeutic decision potentially faces the risk of an insufficient or excessive antithrombotic coverage associated with anticoagulation and its major adverse effects. This guideline was elaborated from nine relevant clinical questions related to the treatment of APS by the Committee of Vasculopathies of the Brazilian Society of Rheumatology. Thus, this study aimed at establishing a guideline that included the most relevant and controversial questions in APS treatment, based on the best scientific evidence available. The questions were structured by use of the PICO (patient, intervention or indicator, comparison and outcome) process, enabling the generation of search strategies for evidence in the major primary scientific databases (MEDLINE/PubMed, Embase, Lilacs, Scielo, Cochrane Library, Premedline via OVID). A manual search for evidence and theses was also conducted (BDTD and IBICT). The evidence retrieved was selected based on critical assessment by using discriminatory instruments (scores) according to the category of the therapeutic question (JADAD scale for randomized clinical trials and Newcastle-Ottawa scale for non-randomized studies). After defining the potential studies to support the recommendations, they were selected according to level of evidence and grade of recommendation, according to the Oxford classification.

OBJECTIVE: To describe demographic features, disease manifestations and therapy in patients with giant cell arteritis from referral centers in Brazil. METHODS: A retrospective cohort study was performed on 45 giant cell arteritis patients from three university hospitals in Brazil. Diagnoses were based on the American College of Rheumatology classification criteria for giant cell arteritis or temporal artery biopsy findings. RESULTS: Most patients were Caucasian, and females were slightly more predominant. The frequencies of disease manifestations were as follows: temporal headache in 82.2%, neuro-ophthalmologic manifestations in 68.9%, jaw claudication in 48.9%, systemic symptoms in 44.4%, polymyalgia rheumatica in 35.6% and extra-cranial vessel involvement in 17.8% of cases. Aortic aneurysms were observed in 6.6% of patients. A comparison between patients with biopsy-proven giant cell arteritis and those without temporal artery biopsies did not yield significant differences in disease manifestations. All patients were treated with oral prednisone, and intravenous methylprednisolone was administered to nearly half of the patients. Methotrexate was the most commonly used immunosuppressive agent, and low-dose aspirin was prescribed to the majority of patients. Relapses occurred in 28.9% of patients, and aspirin had a protective effect against relapses. Females had higher prevalences of polymyalgia rheumatica, systemic manifestations and jaw claudication, while permanent visual loss was more prevalent in men. CONCLUSIONS: Most of the clinical features of Brazilian giant cell arteritis patients were similar to those found in other studies, except for the high prevalence of neuro-ophthalmic manifestations and permanent blindness in the Brazilian patients. Aspirin had a protective effect on relapses.

OBJETIVO: Avaliar a associação entre a presença de anticorpos antinucleossomo (anti-NCS) e a síndrome antifosfolipídica primária (SAFP) e o posterior desenvolvimento de lúpus eritematoso sistêmico (LES). MATERIAIS E MÉTODOS: Trinta e seis mulheres com o diagnóstico de SAFP foram avaliadas prospectivamente para manifestações de doenças reumáticas autoimunes e para a presença de anticorpos antifosfolípides, anticorpos antinucleares e anti-NCS/cromatina. RESULTADOS: Após um período médio de seguimento de 45,7 meses, anticorpos anti-NCS/cromatina foram detectados em apenas uma paciente (2,8%), que desenvolveu manifestações de LES tais como poliartrite, linfopenia, neurite óptica, lesões compatíveis com esclerose múltipla em substância branca cerebral e perfil de autoanticorpos altamente sugestivo de LES. CONCLUSÃO: A frequência de anticorpos anti-NCS/cromatina é baixa em pacientes com SAFP, e sua presença pode associar-se ao desenvolvimento de manifestações de LES.

OBJECTIVE: To study the association of anti-nucleosome (anti-NCS) antibodies in primary antiphospholipid syndrome (APS) and the development of systemic lupus erythematosus (SLE) during follow-up. MATERIALS AND METHODS: Thirty-six women with primary APS were evaluated prospectively for clinical features of systemic autoimmune diseases and for the presence of antiphospholipid antibodies, antinuclear antibodies and anti-NCS/chromatin antibodies. RESULTS: After a mean follow-up period of 45.7 months, anti-NCS/chromatin antibodies were detected in only one patient (2.8%), who developed features of SLE including polyarthritis, lymphopenia, optic neuritis, multiple sclerosis-like lesions, and an autoantibody profile suggestive of SLE. CONCLUSION: The frequency of anti-NCS/chromatin antibodies in primary APS patients is very low, and they may be associated with the development of SLE manifestations.

O sistema imunológico é constituído por uma intrincada rede de órgãos, células e moléculas e tem por finalidade manter a homeostase do organismo, combatendo as agressões em geral. A imunidade inata atua em conjunto com a imunidade adaptativa e caracteriza-se pela rápida resposta à agressão, independentemente de estímulo prévio, sendo a primeira linha de defesa do organismo. Seus mecanismos compreendem barreiras físicas, químicas e biológicas, componentes celulares e moléculas solúveis. A primeira defesa do organismo frente a um dano tecidual envolve diversas etapas intimamente integradas e constituídas pelos diferentes componentes desse sistema. A presente revisão tem como objetivo resgatar os fundamentos dessa resposta, que apresenta elevada complexidade e é constituída por diversos componentes articulados que convergem para a elaboração da resposta imune adaptativa. Destacamos algumas etapas: reconhecimento molecular dos agentes agressores; ativação de vias bioquímicas intracelulares que resultam em modificações vasculares e teciduais; produção de uma miríade de mediadores com efeitos locais e sistêmicos no âmbito da ativação e proliferação celulares; síntese de novos produtos envolvidos na quimioatração e migração de células especializadas na destruição e remoção do agente agressor; e finalmente a recuperação tecidual com o restabelecimento funcional do tecido ou órgão

The immune system consists of an intricate network of organs, cells, and molecules responsible for maintaining the body's homeostasis and responding to aggression in general. Innate immunity operates in conjunction with adaptive immunity and is characterized by rapid response to aggression, regardless of previous stimulus, being the organism first line of defense. Its mechanisms include physical, chemical and biological barriers, cellular components, as well as soluble molecules. The organism first line of defense against tissue damage involves several steps closely integrated and constituted by different components of this system. The aim of this review is to restore the foundations of this response, which has high complexity and consists of several components that converge to articulate the development of adaptive immune response. We selected some of the following steps to review: perception and molecular recognition of aggressive agents; activation of intracellular pathways, which result in vascular and tissue changes; production of a myriad of mediators with local and systemic effects on cell activation and proliferation, synthesis of new products involved in the chemoattraction and migration of cells specialized in destruction and removal of offending agent; and finally, tissue recovery with restoration of functional tissue or organ

O sistema imunológico é constituído por uma intrincada rede de órgãos, células e moléculas, e tem por finalidade manter a homeostase do organismo, combatendo as agressões em geral. A imunidade inata atua em conjunto com a imunidade adaptativa e caracteriza-se pela rápida resposta à agressão, independentemente de estímulo prévio, sendo a primeira linha de defesa do organismo. Seus mecanismos compreendem barreiras físicas, químicas e biológicas, componentes celulares e moléculas solúveis. A primeira defesa do organismo frente a um dano tecidual envolve diversas etapas intimamente integradas e constituídas pelos diferentes componentes desse sistema. A presente revisão tem como objetivo resgatar os fundamentos dessa resposta, que apresenta elevada complexidade e é constituída por diversos componentes articulados que convergem para a elaboração da resposta imune adaptativa. Destacamos algumas etapas: reconhecimento molecular dos agentes agressores; ativação de vias bioquímicas intracelulares que resultam em modificações vasculares e teciduais; produção de uma miríade de mediadores com efeitos locais e sistêmicos no âmbito da ativação e proliferação celulares, síntese de novos produtos envolvidos na quimioatração e migração de células especializadas na destruição e remoção do agente agressor, e finalmente a recuperação tecidual com o restabelecimento funcional do tecido ou órgão.

The immune system consists of an intricate network of organs, cells, and molecules responsible for maintaining the body's homeostasis and responding to aggression in general. Innate immunity operates in conjunction with adaptive immunity and is characterized by rapid response to aggression, regardless of previous stimulus, being the organism first line of defense. Its mechanisms include physical, chemical and biological barriers, cellular components, as well as soluble molecules. The organism first line of defense against tissue damage involves several steps closely integrated and constituted by different components of this system. The aim of this review is to restore the foundations of this response, which has high complexity and consists of several components that converge to articulate the development of adaptive immune response. We selected some of the following steps to review: perception and molecular recognition of aggressive agents; activation of intracellular pathways, which result in vascular and tissue changes; production of a myriad of mediators with local and systemic effects on cell activation and proliferation, synthesis of new products involved in the chemoattraction and migration of cells specialized in destruction and removal of offending agent; and finally, tissue recovery with restoration of functional tissue or organ.

O sistema imunológico é constituído por uma intrincada rede de órgãos, células e moléculas, e tem por finalidade manter a homeostase do organismo, combatendo as agressões em geral. A imunidade inata atua em conjunto com a imunidade adaptativa e caracteriza-se pela rápida resposta à agressão, independentemente de estímulo prévio, sendo a primeira linha de defesa do organismo. Seus mecanismos compreendem barreiras físicas, químicas e biológicas, componentes celulares e moléculas solúveis. A primeira defesa do organismo frente a um dano tecidual envolve diversas etapas intimamente integradas e constituídas pelos diferentes componentes desse sistema. A presente revisão tem como objetivo resgatar os fundamentos dessa resposta, que apresenta elevada complexidade e é constituída por diversos componentes articulados que convergem para a elaboração da resposta imune adaptativa. Destacamos algumas etapas: reconhecimento molecular dos agentes agressores; ativação de vias bioquímicas intracelulares que resultam em modificações vasculares e teciduais; produção de uma miríade de mediadores com efeitos locais e sistêmicos no âmbito da ativação e proliferação celulares, síntese de novos produtos envolvidos na quimioatração e migração de células especializadas na destruição e remoção do agente agressor, e finalmente a recuperação tecidual com o restabelecimento funcional do tecido ou órgão.

The immune system consists of an intricate network of organs, cells, and molecules responsible for maintaining the body's homeostasis and responding to aggression in general. Innate immunity operates in conjunction with adaptive immunity and is characterized by rapid response to aggression, regardless of previous stimulus, being the organism first line of defense. Its mechanisms include physical, chemical and biological barriers, cellular components, as well as soluble molecules. The organism first line of defense against tissue damage involves several steps closely integrated and constituted by different components of this system. The aim of this review is to restore the foundations of this response, which has high complexity and consists of several components that converge to articulate the development of adaptive immune response. We selected some of the following steps to review: perception and molecular recognition of aggressive agents; activation of intracellular pathways, which result in vascular and tissue changes; production of a myriad of mediators with local and systemic effects on cell activation and proliferation, synthesis of new products involved in the chemoattraction and migration of cells specialized in destruction and removal of offending agent; and finally, tissue recovery with restoration of functional tissue or organ.

CONTEXTO: A síndrome do anticorpo anti-membrana basal glomerular (anti-MBG) é caracterizada pela deposição de anticorpos anti-MBG em tecidos afetados, associada à glomerulonefrite e/ou ao envolvimento pulmonar. Essa síndrome já foi descrita em associação a outras doenças autoimunes, mas até onde conhecemos, não há relatos de sua associação com dermatomiosite e psoríase. RELATO DE CASO: Um homem de 51 anos com antecedentes de dermatomiosite e psoríase vulgar apresentou quadro de polineuropatia sensitivo-motora de mãos e pés, perda de 4 kg, adinamia e febre. À admissão estava em uso crônico de ciclosporina e de anti-hipertensivos há três meses devido a hipertensão arterial leve. Exames laboratoriais mostraram anemia e leucocitose, creatinina e ureia séricas elevadas e urina com proteinúria, hematúria, leucocitúria e cilindros granulosos. A proteinúria de 24 horas foi de 2,3 g. A biópsia renal revelou uma glomerulonefrite crescêntica necrotizante com depósitos lineares de imunoglobulina G (IgG) na MBG à imunofluorescência, sugestivos de anticorpos anti-MBG. O paciente foi então tratado inicialmente com metilprednisolona e com ciclofosfamida mensalmente na forma de pulsoterapia.

CONTEXT: Anti-glomerular basement membrane (anti-GBM) antibody syndrome is characterized by deposition of anti-GBM antibodies on affected tissues, associated with glomerulonephritis and/or pulmonary involvement. This syndrome has been described in association with other autoimmune disorders, but as far as we know, it has not been described in association with dermatomyositis and psoriasis. CASE REPORT: A 51-year-old man with a history of dermatomyositis and vulgar psoriasis presented with a condition of sensitive-motor polyneuropathy of the hands and feet, weight loss of 4 kg, malaise and fever. On admission, he had been making chronic use of cyclosporin and antihypertensive drugs for three months because of mild arterial hypertension. Laboratory tests showed anemia and leukocytosis, elevated serum urea and creatinine and urine presenting proteinuria, hematuria, leukocyturia and granular casts. The 24-hour proteinuria was 2.3 g. Renal biopsy showed crescentic necrotizing glomerulonephritis with linear immunoglobulin G (IgG) deposits on the glomerular basement membrane by means of direct immunofluorescence, which were suggestive of anti-GBM antibodies. The patient was then treated initially with methylprednisolone and with monthly cyclophosphamide in the form of pulse therapy.

OBJETIVO: Determinar a prevalência de anticoagulante lúpico (LAC) e dos isótipos de anticardiolipina (ACL) e suas eventuais associações clínicas. PACIENTES E MÉTODOS: Estudo retrospectivo que avaliou manifestações clínicas e laboratoriais em indivíduos que apresentaram positividade para anticorpos antifosfolipídios no Hospital Edmundo Vasconcelos entre março de 2005 e junho de 2006. RESULTADOS: Cento e seis indivíduos (média de idade 42,2 ± 14,1 anos, 84% do sexo feminino) foram incluídos no estudo. A prevalência de trombose foi de 17,9% (19/106) e de morbidade gestacional foi de 12,3% (13/106). O diagnóstico de Síndrome Antifosfolípide (SAF) foi feito em 23,6% (25/106), sendo primária em 68% (17/25) e secundária em 32% (8/25). A prevalência de ACL foi de 97,1% (103/106) e de LAC foi de 11,4% (5/44 dos exames realizados). ACL isótipos IgM, IgG e IgA foram encontrados em 100%, 23,3% e 4,9% dos 103 soros ACL positivos, respectivamente. Para o diagnóstico de SAF, a ACL IgM apresentou sensibilidade de 92% e especificidade de 1,2%, enquanto a ACL IgG teve sensibilidade de 40% e especificidade de 82,5%. A ausência de ACL IgG teve alto valor preditivo negativo (81,4%) para SAF. O LAC apresentou sensibilidade de 18,7% e especificidade de 92,8%. A curva Receiver Operating Characteristic (ROC) demonstrou maior área abaixo da curva para ACL IgG e LAC. CONCLUSÃO: Em amostra aleatória de indivíduos com anticorpos antifosfolipídios positivos, ACL IgG e LAC apresentaram maior especificidade para o diagnóstico de SAF, que se caracterizou pela maior prevalência de trombose.

OBJECTIVE: To evaluate the prevalence of lupus anticoagulant (LAC) and isotypes of anticardiolipin (ACL) antibodies and its possible clinical associations. PATIENTS AND METHODS: A retrospective study analyzed clinical and laboratorial manifestations in individuals who showed positive antiphospholipid antibodies followed-up at Hospital Edmundo Vasconcelos from March 2005 to June 2006. RESULTS: 106 participants (mean age of 42.2 ± 14.1 years at inclusion and female gender in 84% of patients) were included in the study. The prevalence of thrombosis was 17.9% (19/106 patients) and pregnancy morbidity was 12.3% (13/106 patients). The antiphospholipid syndrome (APS) was confirmed in 23.6% (25/106 patients), and it was primary in 68% (17/25 patients) and secondary in 32% (8/25 patients). The ACL antibodies were found in 97.1% (103/106) and LAC in 11.4% (5/44 of the serum samples tested). IgM, IgG and IgA ACL isotypes were respectively found in 100%, 23.3% and in 4.9% of these ACL positive sera. For APS diagnosis the sensitivity of IgM ACL was 92% and its specificity was 1.2%, while IgG ACL had a sensitivity of 40% and specificity of 82.5%. The absence of IgG ACL had a high negative predictive value for APS diagnosis (81.4%).The analysis of the Receiver Operating Characteristic (ROC) curve showed larger area under the curve for ACL IgG and LAC. CONCLUSION: In a random sample of individuals with positive antiphospholipid antibodies, IgG ACL and LAC showed a larger specificity for APS diagnosis which had been characterized by a higher prevalence of thrombosis.