Neuropsychiatric Abnormalities in Systemic Lupus Erythematosus

Abstract

SUMMARY We are impressed that the neurological and psychiatric abnormalities occurring in SLE are, in many respects, the most important manifestation of the entire systemic disease. Yet, paradoxically, it seems that they are also the least understood. With certainty, CNS dysfunction in lupus is common, presents a spectrum of manifestations, and, most significantly, is an important cause of morbidity and mortality. The remainder of our knowledge regarding natural disease, course, pathology and therapy is incomplete and consequently confusing. The explanations for the deficiencies are twofold. First, any individual rheumatic disease unit sees only a limited number of patients with a specific kind of CNS dysfunction. This has resulted in a tendency to combine patients manifesting neuropsychiatric signs and symptoms under the general heading ‘CNS lupus’; a group which is not homogeneous and therefore would not be expected to yield consistent observations. The second major obstacle has been the failure of routine diagnostic procedures to provide an objective assessment of abnormalities within the central nervous system-thus the difficulty in establishing diagnoses and monitoring therapy. In order to expand and refine our current knowledge of lupus neuropsyhiatric disease, we would propose the following: 1. Careful evaluation of neurological and psychiatric function should be a routine procedure in the examination of all patients with SLE. Current reporting often reflects only catastrophic events-and underestimates the magnitude of the problem. 2. Publications should include explicit descriptions of the specific forms of neuropsychiatric abnormalities manifested by the patients being studied. Terms such as vasculitis, cerebritis, encephalitis and psychoses are confusing and should be avoided. Classification by clinical presentation is a satisfactory method of organisation and may identify groups with different natural disease courses and therapeutic requirements. 3. Primary, idiopathic lupus CNS disease must be separated from those secondary aetiologies producing identical clinical abnormalities. Recognition of these secondary entities is essential; first, because they require different therapeutic approaches, and second because their inclusion will further obscure attempts at classification of idiopathic neuropsychiatric manifestations of SLE. 4. The efficacyof treatments currently used for the neuropsychiatric abnormalities of lupus need evaluation by randomised trials. Multi-clinic coperation will be necessary to obtain sufficiently large, homogeneous patient populations.