OBJECTIVES: To investigate the prevalence of dyslipoproteinemia in a homogeneous cohort of polyarticular juvenile idiopathic arthritis patients. METHODS: Based on the National Cholesterol Education Program, fasting lipoprotein levels and risk levels for coronary artery disease were determined in 28 patients with polyarticular juvenile idiopathic arthritis. The exclusion criteria included diabetes, thyroid dysfunction, smoking, proteinuria, lipid-lowering drugs, and hormone/diuretic therapy. Disease activity, disease duration, and therapy with corticosteroids and/or chloroquine were defined at the time of lipid measurements. RESULTS: Dyslipoproteinemia was identified in 20 of the 28 (71%) patients with polyarticular juvenile idiopathic arthritis. The primary lipoprotein risk factor was decreased high-density lipoprotein cholesterol (57%), followed by elevated levels of low-density lipoprotein cholesterol (18%), triglycerides (14%), and total cholesterol (7%). The male patients had decreased high-density lipoprotein cholesterol levels than the female patients (p<0.05). The incidence of decreased high-density lipoprotein cholesterol levels did not seem to be affected by disease activity or therapy because the incidence was similar in patients with active or inactive disease, with or without corticosteroid use and with or without chloroquine use. In addition, the frequency of decreased high-density lipoprotein cholesterol levels was similar in patients with short (≤5 years) vs. long (>5 years) disease duration. CONCLUSIONS: Dyslipoproteinemia is highly prevalent in patients with polyarticular juvenile idiopathic arthritis and is primarily related to decreased high-density lipoprotein cholesterol levels; therefore, early intervention is essential.

O quadro clínico de trombose generalizada de múltiplos órgãos acompanhada de insuficiência ou falência dos mesmos, em associação com anticorpos antifosfolípides, é chamado de "síndrome antifosfolípide catastrófica" (síndrome de Asherson). Esta é uma variante da síndrome antifosfolípide, que pode ocorrer em pacientes sem este diagnóstico ou mesmo durante o curso da síndrome primária e/ou secundária, mesmo com terapêutica anticoagulante. Os pacientes apresentam, principalmente, oclusões de pequenos vasos e as manifestações clínicas dependem dos órgãos acometidos. Fatores desencadeantes podem ser observados em metade dos casos e, apesar dos tratamentos, a sua mortalidade ainda é alta. Esta revisão apresentará a evolução de nossos conhecimentos sobre esta condição e como nós a compreendemos na atualidade.

The clinical picture of widespread multi-organ thrombosis and consequent organ failure in association with antiphospholipid antibodies is referred to as the "catastrophic antiphospholipid syndrome" (Asherson's Syndrome). This is an unusual variant of the antiphospholipid syndrome which may occurs in patients without a previous diagnosis or during the course of primary or secondary antiphospholipid syndrome, even in those with anticoagulant therapy. Patients present with small vessel occlusions and clinical manifestations depend on which organs are affected. Triggering factors have been identified in half of the cases and despite therapy its mortality remains high. This review will present the state of our knowledge on this condition, as we understand it at the present time.

OBJETIVO: Caracterizar as células do infiltrado inflamatório, o padrão de expressão das moléculas de adesão (ICAM-1 e VCAM-1), complexo de ataque à membrana (C5b-9) e antígenos de histocompatibilidade maior (MHC) em biópsias musculares de patientes com doença mista do tecido conectivo (DMTC). MÉTODO: Foram estudados14 pacientes com DMTC e comparadas com 8 pacientes com polimiosite (PM), 5 com dermatomiosite (DM) e 4 com distrofias. As células inflamatórias foram caracterizadas como CD4+, CD8+, células T de memória (CD45RO+) e virgens, células "natural killer" e macrófagos. As expressões de MHC-I e -II, ICAM-1, VCAM-1 e C5b-9 foram caracterizadas em fibras musculares e vasos. RESULTADOS:A análise morfológica demonstrou um padrão tipo PM. O estudo imuno-histoquímico revelou diminuição do número de capilares, predomínio de células CD4+ e B nas regiões perivasculares e predomínio de CD8+ e CD45RO+ nas regiões endomisiais. A expressão de MHC-I nos vasos e nas fibras degeneradas, MHC-II nos vasos e fibras perifasciculares e expressão de ICAM-1 / VCAM-1 no endotélio indicaram uma associação de processos vascular e imune-celular mediando a lesão muscular. CONCLUSÃO: Os achados revelaram duplo mecanismo na DMTC, imune-celular como na PM e vascular como na DM.

OBJECTIVE: To characterize the inflammatory cells, the expression pattern of adhesion molecules (ICAM-1 and VCAM-1), membrane attack complex (C5b-9), and major histocompatibility complex (MHC) antigens in muscle biopsy of mixed connective tissue disease (MCTD). METHOD: We studied 14 patients with MCTD, and compared to 8 polimyositis (PM) patients, 5 dermatomyositis (DM) and 4 dystrophies. Inflammatory cells were examined for CD4+, CD8+, memory and naïve T cells, natural killer cells, and macrophages. Expression of MHC-I and -II, ICAM-1, VCAM-1 and C5b -9 were characterized on muscle fibers and vessels. RESULTS: Morphological analysis displayed a pattern of PM. Immunohistochemical study revealed a decreased number of capillaries, predominance of CD4+ and B cells in perivascular regions and predominance of CD8+ and CD45RO+ in endomysial regions. The expression of MHC-I on vessels and on degenerated muscle fibers, MHC-II expression on vessels and perifascicular muscle fibers, and the expression of ICAM-1 / VCAM-1 on endothelial cells indicated both vascular and cellular-immune mediated processes causing the muscular lesion. CONCLUSION:Our findings revealed a mixed mechanism in MCTD, both vascular involvement as DM, and cell-mediated like PM.