Systemic lupus erythematosus

Eleni I Kampylafka MD ( Dr. Kampylafka of the University of Athens  has no relevant financial relationships to disclose.)
Harry Alexopoulos DPhil (Dr. Alexopoulos of the National and Kapodistrian University of Athens has no relevant financial relationships to disclose.)
Marinos C Dalakas MD (Dr. Dalakas of the University of Athens Medical School and Thomas Jefferson University received honorariums from Baxter, Hoffmann LaRoche, Novartis, and Therapath for consulting, serving on advisory committees, and speaking engagements.)
Raymond P Roos MD, editor. (Dr. Roos of the University of Chicago owns stock in Amgen, Best Doctors, Express Scripts, Ionis, and Merck.)
Originally released May 14, 1996; last updated March 13, 2017; expires March 13, 2020

This article includes discussion of systemic lupus erythematosus, SLE, lupus, NPSLE, neuropsychiatric lupus, antiphospholipid antibody syndrome, discoid lupus, drug-induced lupus, mixed connective tissue disease, Sjögren's syndrome, and subacute cutaneous lupus erythematosus. The foregoing terms may include synonyms, similar disorders, variations in usage, and abbreviations.

Overview

Systemic lupus erythematosus is one of the best-studied autoimmune diseases that may affect all systems, particularly the central and peripheral nervous systems. Therapies have improved, resulting in better quality of life and lower mortality rates. In this article, the authors review the diagnostic, pathogenetic, and therapeutic issues of systemic lupus erythematosus, with an emphasis on neurologic and neuropsychiatric lupus. The most recent guidelines for diagnosis and management are presented.

Key points

 

• The differential diagnosis of NPSLE from CNS infections, metabolic disturbances, primary neuropsychiatric disorders, and drug adverse reactions is important.

 

• Neuroimaging studies and novel laboratory tests for autoantibodies may aid the diagnosis of neuropsychiatric systemic lupus erythematosus and provide clues for the underlying pathophysiology.

 

• The prevalence of cognitive dysfunction in systemic lupus erythematosus has not been accurately determined due to lack of uniform criteria used in its definition and diagnostic tests.

 

• The distinction between major and minor or nonspecific events in neurologic manifestations has a prognostic value because accrual of major neuropsychiatric events and CNS lesions is associated with increased mortality and morbidity.

Historical note and terminology

Even though the term lupus was first used by Rogerius in the 13th century (Blotzer 1983), the systemic nature of the disease was established much later by Osler in 1904 (Smith and Cyr 1988). A number of laboratory findings have revolutionized the understanding and diagnosis of the disease. The false-positive test for syphilis was first described in lupus by Hauck and Reinhart in 1909. Hargraves and colleagues reported the lupus erythematosus cell in 1948, and Friou developed the fluorescent antinuclear antibody test in 1957 (Hargraves et al 1948; Friou 1957). These discoveries established lupus as an autoimmune disease. Since then, defects in immune complex clearance, B-cell tolerance, and T-cell function have been demonstrated (Sullivan 2000). The discovery of various autoantibodies and the recognition that the antiphospholipid syndrome (Hughson et al 1993) can coexist with systemic lupus erythematosus have been important milestones in understanding the disease process. Other landmark observations have included the recognition of drug-induced lupus in 1945 (Alarcon-Segovia 1969) and the establishment of a mouse animal model in 1958.

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