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Central nervous system lupus (CNS lupus) is one of the manifestations of the disease called systemic lupus erythematosus (SLE). It is also called neuropsychiatric lupus (NPSLE). In some SLE patients, the affectation of the nervous system is a major cause of the loss of quality of life and of death. CNS lupus often presents as cognitive dysfunction, particularly affecting memory and reasoning. Any location in the brain and spinal cord may be involved, thus the presentations of this type of lupus may vary.
The major symptom of CNS lupus is cognitive dysfunction. Affected patients often have difficulty recalling things, or are not able to think or reason out clearly. Other symptoms of CNS lupus include headaches, peripheral neuropathies, visual disturbances, and movement disorders. A few may experience autonomic neuropathy, which presents as flushing or mottling of the skin without any apparent environmental trigger. Others experience psychosis, aseptic or noninfectious meningitis, stroke, seizure, stupor, and coma.
Although the underlying pathogenesis of SLE is the production of autoantibodies against the body’s own cells, CNS lupus is caused by various mechanisms. It is proposed that the blood vessel inflammation or vasculitis due to the deposition of immune mediators in the vascular chain may disrupt the brain’s blood supply. Additionally, antiphospholipid antibodies may attack the red blood cells, leading to clot or thrombus formation within the blood vessels, and these clots also compromise the brain’s blood supply. In some lupus patients, anti-neuronal antibodies that attack the neurons are present. Inflammatory mediators such as interleukin-1, interleukin-6, and interferon-gamma may be elevated, leading to inflammation that interferes with neural function.
Hormonal dysfunction, SLE treatments, and infections due to suppression of the immune system may also contribute to CNS dysfunction. Formerly, CNS lupus was thought to affect only 25% of SLE patients, but with new diagnostic tools it has been discovered that more people are affected. It is believed that CNS lupus is actually present in many lupus patients at some point during the course of the disease.
CNS lupus treatments involve anti-inflammatory and immunosuppressive drugs. High-dose oral or intravenous corticosteroids, such as methylprednisolone and prednisone, may be given. Immunosuppressives or cytotoxic drugs, such as cyclophosphamide, azathioprine, and methotrexate, may also be given. Anticoagulation drugs, such as warfarin, may be given for patients who have antiphospholipid syndrome or significant thrombosis. When the serum antibody load is elevated to the point that medical therapy is insufficient, artificial removal of these antibodies may be performed through a process called plasmapheresis.