Large vessel vasculitis

Takayasu arteritis is a chronic granulomatous disease of the aorta and its major branches that usually affects women during the second and third decades of life, but it has been reported in young children. This review details the clinical, pathological and radiological features, differential diagnoses and management of the condition, focusing chiefly on the disease in children. The recent definition of Takayasu arteritis is discussed. The condition should be considered in patients with unexplained arterial hypertension or unexplained inflammatory syndromes without signs of localization. Since the disease may be life-threatening and progressive, early recognition is necessary to initiate appropriate therapy. Patients with persistent ischaemic symptoms including hypertension might benefit from revascularization procedures

Corpos de inclusão citoplasmática: estudo em diversas doenças e revisão da literatura Inclusion cytoplasmic bodies: a study in several diseases and a literature review

Estudamos 16 casos entre 1400 biópsias musculares que apresentavam vacúolos marginados, cujo aspecto histológico sugeria corpos de inclusão citoplasmáticos. Procuramos correlacionar os dados clínicos, laboratoriais e histopatológicos, a fim de determinar a especificidade dos corpos de inclusão citoplasmáticos para determinadas doenças. A creatinaquinase mostrou-se elevada em 10 casos. A eletromiografia foi anormal em todos os casos. A histoquímica muscular em 5 casos revelou uma miopatia, em 7 padrão misto, em dois desinervação e em 2 casos miopatia inflamatória. A microscopia eletrônica demonstrou a presença de filamentos em 8 casos (nucleares, dispersos no citoplasma ou na região subsarcolemal). Os pacientes foram classificados conforme a história clínica, hereditariedade, dados laboratoriais, eletrofisiológicos, histoquímicos e microscopia eletrônica Encontramos miosite com corpos de inclusão citoplasmática (4 casos), atrofia muscular espinhal juvenil (6 casos), miopatias distais (3 casos), distrofia de cinturas pélvica e escapular (2 casos) e polineuropatia periférica (1 caso). Apresentamos revisão sobre a patogenia, formação e possível etiologia dos vacúolos marginados e sua relação com as diversas entidades em que foram detectados, sugerindo que não são específicos para uma única doença.<br>Among 1400 muscle biopsies, we studied 16 cases with rimmed vacuoles, whose histology suggests cytoplasm inclusion bodies. We tried to correlate the clinical, laboratory and histopatological data in order to verify the specificity of cytoplasm inclusion bodies to certain diseases. The creatinekinase was increased in 10 cases. In all cases electromyography was abnormal. Muscle histochemistry revealed myopathy in 5 cases, mixed pattern in 7, denervation in 2 and in 2 cases, inflammatory myopathy. Electron microscopy showed the presence of filaments in 8 cases (nuclear, disseminated in cytoplasm or in the subsarcolemmal region). The patients were classified according to history, heredity, laboratory, electrophysiologic, histochemistry data and electron microscopy: in myositis with inclusion cytoplasmic bodies (4 cases), juvenile spinal muscular atrophy (6 cases), distal myopathies (3 cases), limb-girdle dystrophy (2 cases) and peripheral neuropathy (1 case). We present a revision on the pathogenesis and possible etiology of rimmed vacuoles and their relationship with several diseases

Inhibition of joint damage and improved clinical outcomes with rituximab plus methotrexate in early active rheumatoid arthritis: the IMAGE trial.

Rituximab is an effective treatment in patients with established rheumatoid arthritis (RA). The objective of the IMAGE study was to determine the efficacy of rituximab in the prevention of joint damage and its safety in combination with methotrexate (MTX) in patients initiating treatment with MTX

Inhibition of joint damage and improved clinical outcomes with rituximab plus methotrexate in early active rheumatoid arthritis: the IMAGE trial.

Rituximab is an effective treatment in patients with established rheumatoid arthritis (RA). The objective of the IMAGE study was to determine the efficacy of rituximab in the prevention of joint damage and its safety in combination with methotrexate (MTX) in patients initiating treatment with MTX.In this double-blind randomised controlled phase III study, 755 MTX-naïve patients with active RA were randomly assigned to MTX alone, rituximab 2×500 mg + MTX or rituximab 2×1000 mg + MTX. The primary end point at week 52 was the change in joint damage measured using a Genant-modified Sharp score.249, 249 and 250 patients were randomly assigned to MTX alone, rituximab 2×500 mg + MTX or rituximab 2×1000 mg + MTX, respectively. At week 52, treatment with rituximab 2×1000 mg + MTX compared with MTX alone was associated with a reduction in progression of joint damage (mean change in total modified Sharp score 0.359 vs 1.079; p=0.0004) and an improvement in clinical outcomes (ACR50 65\% vs 42\%; p<0.0001); rituximab 2×500 mg + MTX improved clinical outcomes (ACR50 59\% vs 42\%; p<0.0001) compared with MTX alone but did not significantly reduce the progression of joint damage. Safety outcomes were similar between treatment groups.Treatment with rituximab 2×1000 mg in combination with MTX is an effective therapy for the treatment of patients with MTX-naïve RA. ClinicalTrials.gov identifier NCT00299104