Korean J Pediatr.  2007 Dec;50(12):1180-1187. 10.3345/kjp.2007.50.12.1180.

Systemic lupus erythematosus

• Kim KN

• KMID: 2279448
• DOI: http://doi.org/10.3345/kjp.2007.50.12.1180

Abstract

Systemic lupus erythematosus (SLE) is an episodic, multi-system, autoimmune disease characterized by widespread inflammation of blood vessels and connective tissues and by the presence of antinuclear antibodies (ANAs), especially antibodies to native (double-stranded) DNA (dsDNA). Its clinical manifestations are extremely variable, and its natural history is unpredictable. Untreated, SLE is often progressive and has a significant fatality rate. The most widely used criteria for the classification of SLE are those of the American College of Rheumatology (ACR), which were revised in 1982 and modified in 1997. The presence of four criteria have been diagnosed as a SLE. Rashes are common at onset and during active disease. The oral mucosa is the site of ulceration with SLE. Arthralgia and arthritis affect most children and these symptoms are short in duration and can be migratory. Lupus nephritis may be more frequent and of greater severity in children than in adults. The initial manifestation of nephritis is microscopic hematuria, followed by proteinuria. The most common neuropsychiatric symptoms are depression, psychosis(hallucination and paranoia) and headache. CNS disease is a major cause of morbidity and mortality. Pericarditis is the most common cardiac manifestation. Libman-Sacks endocarditis is less common in children. The most frequently described pleuropulmonary manifestations are pleural effusions, pleuritis, pneunonitis and pulmonary hemorrhage. During the active phase ESR, CRP, gamma globulin, ferritin and anti-dsDNA are elevated. Antibodies to dsDNA occur in children with active nephritis. Antibodies to the extractable nuclear antigens (Sm, Ro/SS-A, La/SS-B) are strongly associated with SLE. Specific treatment should be individualized and based on the severity of the disease. Sepsis has replaced renal failure as the most common cause of death.

Keyword

Systemic lupus erythematosus; SLE; ANA; dsDNA; ACR criteria; Lupus nephritis; CNS lupus; Sepsis

MeSH Terms

Adult
Antibodies
Antibodies, Antinuclear
Antigens, Nuclear
Arthralgia
Arthritis
Autoimmune Diseases
Blood Vessels
Cause of Death
Central Nervous System Diseases
Child
Classification
Connective Tissue
Depression
DNA
Endocarditis
Exanthema
Ferritins
gamma-Globulins
Headache
Hematuria
Hemorrhage
Humans
Inflammation
Lupus Erythematosus, Systemic*
Lupus Nephritis
Mortality
Mouth Mucosa
Natural History
Nephritis
Pericarditis
Pleural Effusion
Pleurisy
Proteinuria
Renal Insufficiency
Rheumatology
Sepsis
Ulcer
Antibodies
Antibodies, Antinuclear
Antigens, Nuclear
DNA
Ferritins
gamma-Globulins