BACKGROUND AND PURPOSE: High-resolution MR imaging is an emerging tool for evaluating intracranial artery disease. It has an advantage of defining vessel wall characteristics of intracranial vascular diseases. We investigated high-resolution MR imaging arterial wall characteristics of CNS vasculitis and reversible cerebral vasoconstriction syndrome to determine wall pattern changes during a follow-up period.
Abstract. Tumor necrosis factor ␣ (TNF␣) plays an important role in the pathogenesis of anti-neutrophil cytoplasmic antibody-associated systemic vasculitis. TNF␣ blockade is a potential therapy for these disorders. Methods: An open-label, multi-center, prospective clinical trial in two subgroups was performed. Study I examined acute disease, either first presentation or relapse (Birmingham Vasculitis Activity Score [BVAS] Ն 10; n ϭ 16); study II examined persistent disease (BVAS Ն 4; n ϭ 16). Patients received infliximab (5 mg/kg) at 0, 2, 6, and 10 wk. Concomitant therapy in study I included prednisolone and cyclophosphamide. Study II patients continued their existing treatment regimens, with prednisolone tapered according to clinical status. Results: Mean age was 52.4 yr, 53% of the patients were female, and follow-up was 16.8 mo. Twenty-eight patients (88%) achieved remission (14 per study group). BVAS decreased from 12.3 (confidence interval [CI] ϭ 10.5 to 14.0) at entry to 0.3 (CI ϭ 0.2 to 0.9) at wk 14 (P Ͻ 0.001). C-reactive protein (mg/L) decreased from 29.4 (CI ϭ 16.8 to 42.0) at entry to 7.0 (CI ϭ 3.3 to 10.9) by wk 14 (P ϭ 0.001). Mean prednisolone dose (mg/d) in study II decreased from 23.8 (CI ϭ 15.0 to 32.5) at entry to 8.8 (CI ϭ 5.9 to 11.7) at wk 14 (P ϭ 0.002). There were two deaths and seven serious infections. Relapse occurred in five patients (three in study II) after a mean of 27 wk. Conclusion: TNF␣ blockade with infliximab was effective at inducing remission in 88% of patients with antibody-associated systemic vasculitis and permitted reduction in steroid doses. Severe infections were seen in 21% of patients, and despite continued infliximab, 20% of initial responders experienced disease flares. Infliximab is a promising new therapy for vasculitis both as a component of initial therapy and in the management of refractory disease. These results need confirmation in larger randomized trials.The primary systemic necrotizing vasculitides are a group of life-threatening diseases that, untreated, have an 85% 2-yr mortality. The best defined and most studied subgroup of these diseases are the anti-neutrophil cytoplasmic antibody (ANCA)-related small vessel vasculitides (AASV), which are characterized by a pauci-immune microscopic vasculitis and focal, necrotizing glomerulonephritis (1).The introduction of steroids and cyclophosphamide results in disease remission in 80% of patients by 3 mo and in 95% by 6 mo (15). However, there is considerable morbidity related to current regimens, on which at least 25% of patients experience severe drug-related adverse effects. Furthermore, 50% of patients experience disease relapse, resulting in accumulating damage from disease scars and treatment (2,3,16). The addition of plasma exchange or pulsed methylprednisolone improves rates of renal recovery but increases the risk of side effects. There is a clear need to achieve more effective remission induction and to reduce therapy-related toxicity in AASV.Evidence suggests that tumor necrosis factor ␣ (TNF␣) plays...
LAMS seem to have excellent efficacy and safety in the management of PFCs. They may be preferred over plastic stents as they are associated with better clinical success and lesser adverse events.
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