Biological treatments for monogenic autoinflammatory disorders
SONG Hong-mei
LI Ji
Abstract：Autoinflammatory syndromes are conditions caused by mutations of proteins,playing a important role in the regulation of the innate immunity,which leads to an uncontrolled inflammation.The understanding of the molecular mechanisms in these disorders discloses new molecular therapeutic targets.The good response to anti IL-1 drugs in cryopyrin-associated periodic syndromes (CAPS) is the brightest example of the possibility to dampen inflammation with the blockade of a single cytokine,and IL-l blockers have been used in the treatments of systemic juvenile idiopathic arthritis (sJIA),tumor necrosis factor (TNF) receptor-associated periodic syndrome (TRAPS) and familial Mediterranean fever(FMF).Anti-TNF are needed in the control of long-term fever in TNF receptor-associated periodic syndrome,and anti-TNF agents are also useful in FMF with AA amyloidosis.Anti-TNF agents are the most common treatment for Blau syndrome,which relieve the joint symptoms.Many studies have demonstrated significant efficacy of anti IL-6 drugs in patients with sJIA.Due to their extreme rarity,there are few case reports on the response to anti IL-6 drugs in other autoinflammatory diseases.
Keywords：autoinflammatory syndromesIL-6 blockerTNF-receptor blockerIL-1 blocker
Publication Date：2017-01-01
Online Publishing Date：2025-08-15(First online date of this platform, not the publication date of the document)
Pages：6( 502-507 )