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Childhood-onset systemic lupus erythematosus (i.e., cSLE), also termed juvenile-onset systemic lupus erythematosus, juvenile systemic lupus erythematosus, and pediatric systemic lupus erythematosus, is a form of the chronic inflammatory and autoimmune disease, systemic lupus erythematosus (i.e., SLE), that develops in individuals up to 18 years old. [1]
Lupus, formally called systemic lupus erythematosus (SLE), is an autoimmune disease in which the body's immune system mistakenly attacks healthy tissue in many parts of the body. [1] Symptoms vary among people and may be mild to severe. [1]
Neonatal lupus erythematosus is an autoimmune disease in an infant born to a mother with anti-Ro/SSA and with or without anti-La/SSB antibodies. [1] [2] The disease most commonly presents with a diffuse/periorbital rash resembling subacute cutaneous lupus erythematosus and can have systemic abnormalities such as complete heart block or hepatosplenomegaly. [3]
Lupus erythematosus is a collection of autoimmune diseases in which the human immune system becomes hyperactive and attacks healthy tissues. [1] Symptoms of these diseases can affect many different body systems, including joints, skin, kidneys, blood cells, heart, and lungs. The most common and most severe form is systemic lupus erythematosus.
In addition, onset of neuropsychiatric symptoms may happen prior to the diagnosis of lupus. [7] Due to the lack of uniform diagnostic standards, statistics about NPSLE vary widely. [8] Tests which aid in diagnosis include MRI, electrophysiological studies, psychiatric evaluation, and autoantibody tests. [9]
Lupus nephritis is an inflammation of the kidneys caused by systemic lupus erythematosus (SLE) and childhood-onset systemic lupus erythematosus which is a more severe form of SLE that develops in children up to 18 years old; both are autoimmune diseases. [3] [4] It is a type of glomerulonephritis in which the glomeruli become inflamed.
In 1999 a study noted, "In recent years there has been growing concern regarding the diagnosis of incomplete forms of the autoimmune diseases" [26] and the first classification criteria were proposed in that year. [1] Historically the condition was sometimes called undifferentiated connective tissue syndrome, latent lupus or incomplete lupus. [1]
In addition, MAS has been described in association with systemic lupus erythematosus (SLE), Kawasaki disease, and adult-onset Still's disease. It is thought to be closely related and pathophysiologically very similar to reactive (secondary) hemophagocytic lymphohistiocytosis (HLH). [1]