Brain hypoperfusion in a girl with systemic lupus erythematosus

Abstract

We describe an adolescent girl with systemic lupus erythematosus, presenting with severe cardiovascular autonomic dysfunction and incapacitating orthostatic hypotension to a degree not previously reported. Further evaluation of cerebral blood flow velocity, using transcranial Doppler testing, demonstrated an abnormal hypercapnic cerebrovascular response. Both the orthostatic hypotension and the abnormal cerebrovascular hypercapnic response improved with intensive medical treatment of her systemic lupus erythematosus. Additional studies are necessary to elucidate the pathogenesis of these cerebrovascular and autonomic abnormalities, especially considering the potential consequences they may exert on cerebral perfusion, which may be subtle, underrecognized, and capable of affecting cognition.