OBJECTIVE: To analyze the prevalence of myositis-specific and myositis-associated autoantibodies and their clinical correlations in a large series of patients with dermatomyositis/polymyositis. METHOD: This cross-sectional study enrolled 127 dermatomyositis cases and 95 polymyositis cases. The disease-related autoantibody profiles were determined using a commercially available blood testing kit. RESULTS: The prevalence of myositis-specific autoantibodies in all 222 patients was 34.4%, whereas myositis-associated autoantibodies were found in 41.4% of the patients. The most frequently found autoantibody was anti-Ro-52 (36.9%), followed by anti-Jo-1 (18.9%), anti-Mi-2 (8.1%), anti-Ku (4.1%), anti-SRP (3.2%), anti-PL-7 (3.2%), anti-PL-12 (2.7%), anti-PM/Scl75 (2.7%), and anti-PM/Scl100 (2.7%). The distributions of these autoantibodies were comparable between polymyositis and dermatomyositis, except for a higher prevalence of anti-Jo-1 in polymyositis. Anti-Mi-2 was more prevalent in dermatomyositis. Notably, in the multivariate analysis, anti-Mi-2 and anti-Ro-52 were associated with photosensitivity and pulmonary disorders, respectively, in dermatomyositis. Anti-Jo-1 was significantly correlated with pulmonary disorders in polymyositis. Moreover, anti-Ro-52 was associated with anti-Jo-1 in both diseases. No significant correlation was observed between the remaining autoantibodies and the clinical and/or laboratory findings. CONCLUSIONS: Our data are consistent with those from other published studies involving other populations, although certain findings warrant consideration. Anti-Ro-52 and anti-Jo-1 were strongly associated with one another. Anti-Ro-52 was correlated with pulmonary disorders in dermatomyositis, whereas anti-Jo-1 was correlated with pulmonary alterations in polymyositis.

OBJETIVO: A recente descrição de anticorpos antilipoproteína lipase (anti-LPL) associados à dislipoproteinemia levounos a analisar sua presença e possível associação com achados clínicos e laboratoriais em pacientes com doença de Behçet. PACIENTES E MÉTODOS: Trinta e oito pacientes consecutivos com doença de Behçet [critérios do Grupo de Estudos Internacional em Doença de Behçet (International Study Group on Behçet's Disease - ISGBD)] foram testados para a presença de anticorpos anti-LPL através da técnica de ELISA. Foi realizada avaliação clínica e laboratorial, incluindo perfil lipídico de jejum, pesquisa de autoanticorpos e marcadores inflamatórios (PCR, VHS) na inclusão dos pacientes. Os critérios de exclusão foram quaisquer condição que afetassem o perfil lipídico. RESULTADOS: A média de idade foi de 42 ± 9 anos, sendo 68% do sexo feminino e 68% da cor branca. O tempo médio de duração da doença foi de 9,8 ± 7,5 anos. Vinte e nove por cento dos pacientes apresentaram história de trombose. Os níveis de PCR estavam elevados em 31% dos pacientes e VHS aumentada foi detectada em 31%, com níveis médios de 5,95 ± 10,3 mcg/mL e 14,5 ± 13,2 mm/1ªhora, respectivamente. Cerca de 47% dos pacientes estavam tomando prednisona, com dose média de 7,6 ± 10,8 mg/dia. Quanto aos níveis de risco cardiovascular da NCEP/ATPIII, colesterol elevado foi verificado em 26%, triglicerídeos em 18%, HDL baixo em 15% e elevado LDL em 25% dos pacientes com Behçet. Os níveis médios de CT foram 198 ± 48 mg/dL, de triglicerídeos 121 ± 61 mg/dL, HDL 52.4 ± 14.7 mg/dL e LDL 119 ± 35 mg/dL. Anticorpos anti-LPL to subtipo IgG foram detectados em 0/30 pacientes com Behçet. CONCLUSÃO: Esses dados aqui apresentados corroboram uma ausência de ligação entre inflamação, resposta imunológica e dislipoproteinemia nos pacientes com doença de Behçet e sugerem que outros mecanismos estão associados à dislipidemia vista nestes pacientes.

OBJECTIVE: The recent description of anti-lipoprotein lipase antibodies (anti-LPL) associated with dislipoproteinemia led us to analyze its presence and possible association with clinical and laboratorial findings in patients with Behçet's disease. PATIENTS AND METHODS: Thirty-eight consecutive patients with Behçet's disease [International Study Group for Behçet's Disease (ISGBD) criteria] were tested for the presence of anti-LPL antibodies by ELISA. Patients underwent clinical and laboratorial evaluation, including fasting lipid profile, determination of autoantibodies, and inflammatory markers (CRP, ESR) before inclusion in the study. Exclusion criteria were as follows: any conditions that affected the lipid profile. RESULTS: Patients had a mean age of 42 ± 9 years, 68% were females, and 68% were Caucasian. Mean disease duration was 9.8 ± 7.5 years. Twenty-nine percent of the patients had a history of thrombosis. C-reactive protein levels were elevated in 31% of the patients, and ESR was increased in 31% of the patients, with mean levels of 5.95 ± 10.3 mcg/mL and 14.5 ± 13.2 mm/1st hour, respectively. Approximately 47% of the patients were taking prednisone, with a mean dose of 7.6 ± 10.8 mg/day. As for NCEP/ATPIII cardiovascular risk levels, cholesterol levels were elevated in 26% of the patients, triglycerides in 18%, low HDL in 15%, and elevated LDL in 25% of the patients with Behçet's disease. Mean total cholesterol levels were 198 ± 48 mg/dL, triglycerides 121 ± 61 mg/dL, HDL 52.4 ± 14.7 mg/dL, and LDL 119 ± 35 mf/dL. IgG anti-LPL antibodies were detected in 0/30 patients with Behçet's disease. CONCLUSION: The data presented here indicates the lack of correlation among inflammation, immune response, and dislipoproteinemia in patients with Behçet's disease and suggests that other mechanisms are associated with the dyslipedemia seen in those patients.

INTRODUÇÃO: O Lúpus Eritematoso Sistêmico (LES) se caracteriza por períodos de exacerbação e remissão clínica que frequentemente são acompanhados por alterações nos níveis séricos de anticorpos específicos, como o anti-dsDNA, que está presente em 40% dos casos, associado principalmente à atividade renal. Recentemente houve a descrição de duas subpopulações de anticorpos antilipoproteína lipase (anti-LPL) no LES: uma com e a outra sem atividade anti-dsDNA. A possível relação desse último grupo de anticorpos com a atividade inflamatória de doença ainda não foi analisada no LES. OBJETIVOS: Avaliar longitudinalmente a associação dos níveis séricos dos anticorpos anti-LPL com atividade do LES em pacientes com anti-dsDNA persistentemente negativo. PACIENTES E MÉTODOS: Cinco pacientes com LES com anti-dsDNA persistentemente negativo mensurado por ELISA e por imunofluorescência indireta em crithidia luciliae e altos títulos de anti-LPL por ELISA (> 5 desvios-padrão (DP) da média de 20 controles normais) foram selecionados e acompanhados longitudinalmente durante um período mínimo de dois anos. RESULTADOS: Caso 1: Homem, 24 anos com LES desde 2001 apresentou hemorragia alveolar, proteinúria, hipertensão arterial sistêmica, eritema malar, aftas, artrite, FAN+, com SLEDAI (systemic lupus erythematosus disease activity index) = 16 e anti-LPL = 144UA. Tratado com pulso de metilprednisolona e prednisona com melhora clínica e SLEDAI = 0 e redução do anti-LPL (109UA). Nova atividade com acometimento renal em abril de 2002, SLEDAI = 10 e aumento de anti-LPL (150UA). Iniciada pulsoterapia de ciclofosfamida e metilprednisolona com boa resposta, SLEDAI = 0 e diminuição de anti-LPL (77UA) até a sua total negativação acompanhando a remissão do quadro no ano de 2003. Caso 2: Mulher, 32 anos, com LES desde 1997. Em setembro de 2001 iniciou vasculite cutânea, febre e rash, SLEDAI = 10, anti-LPL = 80UA. Em janeiro de 2002, teve atividade renal e HAS, SLEDAI = 8 e anti-LPL = 25UA, mas com a introdução de CE e ciclofosfamida evoluiu com melhora importante. Em 2003, assintomática, apresentava SLEDAI = 2 e anti-LPL = 12UA. Caso 3: Homem, 39 anos, com LES desde 1997. Estável em uso de cloroquina, sem atividade durante 3 anos, SLEDAI = 0 em todas as ocasiões e sem variação dos títulos de anti-LPL no período do estudo: 85UA (2001),100UA (2002) e 86UA (2003). Caso 4: Mulher, 58 anos com LES desde 1996. Remissão do LES desde agosto de 2001 e com SLEDAI = 0 no período do estudo. Títulos de anti-LPL sem flutuações significativas: 71UA (2001), 42UA (2002) e 61UA (2003). Caso 5: Mulher, 55 anos com LES desde 1989. Estável desde 2000 em uso de cloroquina, SLEDAI = 0, e títulos de anti-LPL também sem variações: 71UA (1999), 92UA (2001), 71UA (2002) e 90UA (2003). CONCLUSÃO: A avaliação longitudinal dos pacientes selecionados mostrou-se estar associada à flutuação dos títulos de anti-LPL com o SLEDAI, sugerindo ser essa variação um novo marcador de atividade do LES, na ausência de anticorpos anti-dsDNA.

INTRODUCTION: Systemic lupus erythematosus (SLE) is characterized by periods of clinical flares and remission that are followed by alterations of sera specific autoantibodies such as anti-dsDNA, present in 40% of the cases and strongly associated with renal involvement. Recently, there was a description of two subpopulations of anti-lipoprotein lipase antibodies (anti-LPL) in SLE: with and without anti-dsDNA activity. A possible relationship between these antibodies with inflammatory activity of SLE was not analyzed. OBJECTIVES: To evaluate longitudinally the association between anti-LPL with lupus activity in patients persistently negatives for anti-dsDNA antibodies. PATIENTS AND METHODS: Five SLE patients with persistently negative anti-dsDNA measured by ELISA and indirect immunofluorescence using crithidia luciliae and high titers of anti-LPL by ELISA (> 5 SD) were selected and followed for at least 2 years. RESULTS: Case 1: A 24-year-old male with SLE since 2001, presented with alveolar hemorrhage, proteinuria, systemic hypertension, malar rash, oral ulcers, polyarthritis, positive ANA, SLEDAI=16 and anti-LPL=144U. He was treated with intravenous (IV) methylprednisolone followed by prednisone and had an excellent response. SLEDAI=0, anti-LPL decreased to 109U. New renal flare in April 2002, SLEDAI=10 and a new increment of anti-LPL (150U). IV Cyclophosphamide and methylprednisolone were started and he achieved remission, SLEDAI=0 and a decrease of anti-LPL (77U) until become negative in 2003. Case 2: A 32-year-old female had SLE since 1997. In September 2001 began cutaneous vasculitis, fever and rash, SLEDAI=10, anti-LPL=80U. In January 2002, she had renal involvement and systemic hypertension, SLEDAI=8 and anti-LPL= 25U. She received corticosteroid and cyclophosphamide and improved. In 2003, she was asymptomatic, SLEDAI=2 and anti-LPL=12U. Case 3: A 39-year-old male has SLE since 1997. He was stable, under chloroquine use, without disease activity for 3 years, SLEDAI=0 in all period studied and no fluctuation of anti-LPL titers: 85U (2001),100U (2002) e 86U (2003). Case 4: A 58-year-old female had SLE since 1996. She was in remission since August 2001 with a SLEDAI=0 during all this study. Anti-LPL titers did not significantly change: 71U (2001), 42U (2002) e 61U (2003). Case 5: A 55-year-old female had SLE since 1989. She was stable since 2000 using chloroquine, SLEDAI=0, and anti-LPL titers without variations: 71U (1999), 92U (2001), 71U (2002) e 90U (2003). CONCLUSION: The longitudinal evaluation of the selected patients showed a positive correlation between the fluctuations of the titers of anti-LPL with the SLEDAI score. These findings suggest that this variation may be a new marker for lupus activity in patients without anti-dsDNA antibodies.

A síndrome do lúpus neonatal (SLN) é uma doença auto-imune associada à presença de auto-anticorpos na circulação materno-fetal contra complexos ribonucléicos, SSA/Ro e SSB/La, e se caracteriza principalmente por bloqueio cardíaco congênito isolado (BCCI) e/ou manifestações cutâneas e hematológicas. A despeito da sua raridade, a SLN é a principal causa de BCCI, sendo responsável pela importante mortalidade (20% a 30%) e morbidade desses pacientes. A denominação de lúpus neonatal se baseia na semelhança das lesões cutâneas associadas à SLN nos neonatos com aquelas observadas em pacientes com lúpus eritematoso sistêmico (SLE). Por outro lado, o termo "isolado", para designar o BCC na SLN, é utilizado para especificar a ausência de malformações cardíacas congênitas e a ausência de infecções que causam alterações na condução átrio-ventricular (BAV). A SLN constitui-se num clássico modelo de auto-imunidade adquirida, no qual os anticorpos IgG maternos atravessam a barreira placentária e na circulação fetal podem exercer um papel importante na patogênese da síndrome. A presença quase universal dos anticorpos anti-Ro/SSA e anti-SSB/La no soro materno e fetal os inclui como marcadores para a SLN. Ao contrário da lesão cardíaca que compromete irreversivelmente a condução átrio-ventricular, os acometimentos cutâneos e/ou hematológicos são transitórios e podem regredir após o desaparecimento dos anticorpos maternos da circulação do lactente. Clinicamente, a SLN representa um desafio para profissionais reumatologistas, obstetras, neonatalogistas, dermatologistas e cardiologistas pediátricos que têm como meta identificar o risco gestacional de desenvolvimento da doença fetal, diagnosticar a síndrome precocemente e definir uma estratégia terapêutica adequada quando "in utero" ou pós-natal.

Neonatal Lupus Syndrome (NLS) is an autoimmune disease associated to the presence of autoantibodies against ribonucleoproteins SSA/Ro and/or SSB/La in the maternal-fetal circulation and is characterized by isolated congenital heart block (ICHB) and/or cutaneous and hematological manifestations. Despite the rarity, NLS is the main cause of ICHB, which is responsible for the significant mortality (20%-30%) and morbidity of these patients. The neonatal lupus designation comes from the observation of cutaneous lesions similarities between NLS and systemic lupus erythematosus. The isolated term to define the CHB in NLS is employed to exclude cardiac abnormalities secondary to congenital structural malformations or infections. NLS is considered a model of passively acquired autoimmunity in which maternal autoantibodies cross the placenta and, once in fetal circulation, may play a role in the pathogenesis of the syndrome. The almost universal presence of anti-SSA/Ro and/or anti-SSB/La antibodies in the maternal and fetal circulation validates them as serological markers of NLS. Contrasting with the fetal cardiac tissue lesion, which is an irreversible process, cutaneous and hematological involvements are transitory manifestations and disappear after the clearance of maternal autoantibodies from the infant's circulation. It is important to notice that NLS represents a multidisciplinary challenge involving rheumatologists, obstetricians, neonatologists, dermatologists and pediatric cardiologists to identify the pregnancy at risk for NLS development and to introduce appropriate in utero or postnatal therapeutic strategies.