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.
- SourceAvailable from: onlinelibrary.wiley.com[Show abstract] [Hide abstract]
ABSTRACT: Abstract Mycophenolate mofetil (MMF) is efficacious in the management of transplantations and lupus nephritis. It is also useful in the treatment of autoimmune conditions as its mechanisms of action target the T- and B-lymphocytes, leading to suppression of the cell-mediated immune responses and antibody formation. It has been used successfully in immune-mediated conditions like myasthenia gravis, autoimmune hepatitis and immune cytopenias. However, its optimal use in non-renal manifestations (haematological, neuropsychiatric, myocardial, pulmonary, curaneous) in lupus patients are unclear and predominantly based on anecdotal reports. There are yet to be randomised controlled trials to guide the optimal doses and durations of MMF in such situations. MMF is relatively well tolerated and safe to use, though there are reports of serious adverse effects like urticaria, myopathy, Epstein-Barr virus-associated B-cell lymphoma, cytomegalovirus infections and disseminated varicella zoster. Another area of concern for patients is the increased cost of using long-term MMF. A practical approach for rheumatologists would be to use a dosing regimen guided by data from lupus nephritis trials and using MMF mainly in those lupus patients with non-renal manifestations failing conventional therapy.APLAR Journal of Rheumatology 01/2006; 9(4):408-412.
- [Show abstract] [Hide abstract]
ABSTRACT: Mofetil micofenolato (MMF), un agente inmunosupresor eficaz en el tratamiento de trasplante de órganos sólidos, ha sido utilizado exitosamente en la terapia de inducción y de mantenimiento de la nefritis lúpica proliferativa y membranosa. La evidencia sobre su uso en manifestaciones lúpicas no renales es limitada y se basa principalmente en series de casos y estudios abiertos. Según la evidencia, MMF puede ser eficaz en manifestaciones hematológicas y cutáneas refractarias; sin embargo, su eficacia en estas manifestaciones debe ser confirmada con estudios controlados.Revista Colombiana de Reumatología. 10/2008; 15(4):307-320.
- [Show abstract] [Hide abstract]
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