Cerebral vasculitis in a patient with hereditary complete C4 deficiency and systemic lupus erythematosus.
ABSTRACT We describe the case of a female patient with hereditary complete C4 deficiency and systemic lupus erythematosus. She had suffered from lupus nephritis in early childhood. At the age of 23 years she developed severe lupus with skin disease and life-threatening cerebral vasculitis. Her cerebral disease was unresponsive to high-dose steroids, intravenous immunoglobulin, fresh frozen plasma and plasma exchange. Improvement was achieved with immunoadsorption in combination with mycophenolate mofetil. The patient made a complete recovery and is maintained in complete remission on mycophenolate and low-dose steroids.
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ABSTRACT: Clinicians are increasingly using mycophenolate mofetil (MMF) for the treatment of systemic lupus erythematosus (SLE). This review will discuss the key studies that have contributed to our understanding of the efficacy and safety of MMF in the treatment of SLE. The Aspreva Lupus Management Study (ALMS) firmly established that MMF is equivalent to intravenous pulse cyclophosphamide (IVC) for the induction treatment of lupus nephritis. In addition, MMF was shown to be superior to azathioprine in decreasing the incidence of treatment failure during maintenance therapy. A posthoc analysis of the induction phase of ALMS suggested that MMF also improved nonrenal manifestations of SLE. In contrast to the ALMS maintenance results, a European trial concluded that MMF and azathioprine were equivalent in the ability to prevent renal flare after induction treatment with low-dose IVC. Favorable efficacy and safety results of several clinical trials conducted over the past 10 years have led to the adoption of MMF for the treatment of lupus nephritis and nonrenal lupus. Future research will be important to more fully understand the best dosing regimen of MMF for induction versus maintenance treatment, total duration of treatment, and the utility of therapeutic monitoring of MMF levels.Current opinion in rheumatology 06/2011; 23(5):454-8. · 4.60 Impact Factor
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ABSTRACT: Immunoadsorption (IAS) is used as a rescue therapy in severely ill SLE patients who are refractory to conventional therapies. This extracorporeal method aims at the rapid and extensive removal of pathogenic immunocomplexes (ICs) and (auto-)antibodies (Abs). Although past data have shown short- to mid-term efficacy and biocompatibility of IAS in (renal) SLE, it is still an experimental and rather expensive procedure - and evidence from randomized controlled trials (RCTs) is lacking. Nevertheless, IAS is successfully used in life-threatening situations because of its fast mode of action and its acceptable safety profile.Lupus 01/2011; 20(2):115-9. · 2.78 Impact Factor
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ABSTRACT: Systemic Lupus Erythematosus (SLE) is a clinically diverse, chronic autoimmune disease with inflammation in several organ systems. Its pathogenesis is complex, but includes many factors that can be influenced by glucocorticoids (GC). Indeed, GC constitute the corner-stone in SLE-treatment. However, guidelines for GC- treatment of the different disease manifestations are lacking and not every patient responds (sufficiently). The focus of this systematic review is to evaluate the differential glucocorticoid treatment of various SLE manifestations. In addition, some relevant mechanisms of glucocorticoid action as well as resistance are discussed.Autoimmunity reviews 12/2012; · 6.37 Impact Factor