Advances in research of Blau syndrome of children
LI Cai-feng
Abstract：Blau syndrome (BS)is a monogenic disease resulting from mutations in the NOD2.It is characterized by the triad of granulomatous polyarthritis,dermatitis and uveitis with an onset age before five years.Takayasu's arteritis,granuloma in liver and spleen and kidney are often observed in Blau syndrome.Diagnosis is made on the basis of pathological biopsy of skin or synovium.The mutation of NOD2 can also confirm the diagnosis.The treatment for Blau syndrome includes glucocorticoid,nonsteroidal anti-inflammatory drug and immunosuppressor.Early treatment with TNF-α inhibitor for patients with iridocyclitis and Takayasu's arteritis can improve the prognosis.
Keywords：Blau syndromeNOD2 genegranulomatous arthritis of childhood
Publication Date：2018-01-01
Online Publishing Date：2025-08-15(First online date of this platform, not the publication date of the document)
Pages：4( 26-29 )