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Immunol."],"abstract":"<jats:p>Blau syndrome is a systemic autoinflammatory granulomatous disease caused by mutations in the nucleotide-binding oligomerization domain 2 (<jats:italic>NOD2<\/jats:italic>) gene. NOD2 is an intracellular pathogen recognition receptor. Upon binding to muramyl dipeptide (MDP), NOD2 activates the NF-\u03baB pathway, leading to the upregulation of proinflammatory cytokines. Clinical manifestations of Blau syndrome appear in patients before the age of four. Skin manifestations resolve spontaneously in some cases; however, joint and eye manifestations are progressive, and lead to serious complications, such as joint contracture and blindness. Currently, there is no specific curative treatment for the disease. Administration of high-dose oral steroids can improve clinical manifestations; however, treatments is difficult to maintain due to the severity of the side effects, especially in children. While several new therapies have been reported, including JAK inhibitors, anti-IL-6 and anti-IL-1 therapies, anti-TNF therapy plays a central role in the treatment of Blau syndrome. We recently performed an <jats:italic>ex vivo<\/jats:italic> study, using peripheral blood and induced pluripotent stem cells from patients. This study demonstrated that abnormal cytokine expression in macrophages from untreated patients requires IFN\u03b3 stimulation, and that anti-TNF treatment corrects the abnormalities associated with Blau syndrome, even in the presence of IFN\u03b3. Therefore, although the molecular mechanisms by which the genetic mutations in <jats:italic>NOD2<\/jats:italic> lead to granuloma formation remain unclear, it is possible that prior exposure to TNF\u03b1 combined with IFN\u03b3 stimulation may provide the impetus for the clinical manifestations of Blau syndrome.<\/jats:p>","DOI":"10.3389\/fimmu.2022.895765","type":"journal-article","created":{"date-parts":[[2022,6,1]],"date-time":"2022-06-01T05:32:11Z","timestamp":1654061531000},"update-policy":"https:\/\/doi.org\/10.3389\/crossmark-policy","source":"Crossref","is-referenced-by-count":25,"title":["Potential Benefits of TNF Targeting Therapy in Blau Syndrome, a NOD2-Associated Systemic Autoinflammatory 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