The central nervous system in systemic lupus erythematosus. Part 1. Clinical syndromes: a literature investigation

To establish the central nervous system (CNS) manifestations of systemic lupus erythematosus (SLE) as described in the literature and to compare the results with two previously published classifications.

Using PUBMED, a systematic search was performed for publications from 1980 onwards on CNS syndromes of patients with SLE. A distinction was made between CNS syndromes induced by SLE and the CNS autoimmune diseases associated with SLE. Criteria were defined for inclusion of CNS syndromes or diseases as SLE-induced or SLE-associated.

The literature search yielded names of 30 syndromes and two diseases, but only 16 syndromes and one disease fulfilled the set of predefined criteria. Two syndromes-depression and anxiety-were predominantly psychological in origin in most patients; other syndromes were biological.

Strengths and weaknesses of two classifications of CNS syndromes are evaluated. The older of the two is long and has not been accepted fully. Brevity is an advantage of the American College of Rheumatology (ACR) nomenclature system. A disadvantage of this system is the concealment of differences in health risks by the pooling of items. Furthermore, the items of the system do not all belong to the same dimension: one is pathological and the others are clinical. To remedy these drawbacks, we suggest the rephrasing and subdivision of items and that the predominantly psychopathological syndromes should be dealt with separately in epidemiological studies.

SLE may induce 16 different clinical syndromes of the CNS and is occasionally associated with one other CNS autoimmune disease. A modification of the ACR nomenclature system is proposed.