Lupus Cerebritis
The Common Vein Copyright 2010
Definition
Systemic Lupus Erythematosus is an autoimmune disease, affecting multisystems, in which there is an active inflammation and reaction against most tissues of the body. It is caused mostly caused by antibodies against the nucleus of the cells (ANA’s), although there can be other antibodies involved. It can result in the development of arthritis, skin rashes, oral ulcers, renal impairment, bone marrow suppression, vasculitidis, and neurologic damage. It is a highly variable disease, so it can range from 1 to several systems affected.
Structurally, it is characterized by active inflammation and lymphocytic infiltrates in different tissues, which will show many signs of necrosis, fibrosis and destruction.
As mentioned, many systems can be affected, and the CNS is damaged in 50% of patients with SLE. When that happens, patients functionally have, on top of their SLE symptoms secondary to damage to other organs, seizures and psychosis. The cause for the CNS symptomatology is due to damage to the endothelium and occlusion of small vessels due to intimal proliferation.
Clinically, in Lupus Cerebritis (the term used to describe affection of the CNS by SLE), patients present with acute confusional states, psychosis, depression or mania. They can also have focal symptoms, seizures, tremor, chorea or ataxia.
Diagnosis is made by meeting the criteria for SLE proposed by the American College of Rheumatology. In terms of cerebral lupus, coagulopathy, infection and emboli should be excluded.
Radiologically, lupus cerebritis will show changes consistent with long-standing white matter disease, and as such, have many similar findings as seen, for example in chronic multiple sclerosis. T1 weighted images demonstrate hypointense periventricular lesions (loss of white matter due to axonal loss)
Treatment requires immune suppression with corticosteroids and may require more potent immune suppression with cyclophosphamide or other immunosupressive agents.