Neurologic manifestations of systemic lupus erythematosus and antiphospholipid antibody syndrome

Systemic lupus erythematosus (SLE) and the antiphospholipid antibody syndrome (APS) are autoimmune diseases that carry a high risk of thrombosis and other nervous system manifestations. APS is frequently seen in patients with SLE, but can be seen in patients without it. The nervous system is commonly affected in both children and adults with SLE and is also associated with a worse prognosis and more cumulative damage in children and adults. Neuropsychiatric lupus manifestations can occur in the absence of either serologic activity or other systemic disease manifestations. The American College of Rheumatology established case definitions for 19 central and peripheral nervous system syndromes. APS is defined as an episode of arterial or venous thrombosis leading to tissue ischemia or recurrent fetal loss in the presence of antiphospholipid antibodies of moderate to high titer or a lupus anticoagulant that is present on at least two occasions at least 12 weeks apart. APS is classified as primary if it occurs in an individual without and secondary when it occurs with SLE or some other connective tissue disease. This chapter will review the neurologic manifestations of SLE and APS, highlighting their interrelationships, as well as the clinical differences in each when the other is not present. The diagnosis, pathophysiology, and treatment of these manifestations will also be discussed.