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- 저자 : ( Suguru Uemura ) (검색결과 1 건)
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A case of autoimmune enteropathy with CTLA4 haploinsufficiency
( Haruka Miyazaki ),( Namiko Hoshi ),( Michitaka Kohashi ),( Eri Tokunaga ),( Yuna Ku ),( Haruka Takenaka ),( Makoto Ooi ),( Nobuyuki Yamamoto ),( Suguru Uemura ),( Noriyuki Nishimura ),( Kazumoto Iij 대한장연구학회 2022 Intestinal Research Vol.20 No.1
Autoimmune enteropathy (AIE) is a rare disease, characterized by intractable diarrhea, villous atrophy of the small intestine, and the presence of circulating anti-enterocyte autoantibodies. Immune dysregulation, polyendocrinopathy, enteropathy, X-linked (IPEX) syndrome, and mutations in FOXP3, which is a master gene of regulatory T cells (Tregs), are major causes of AIE. Recent studies have demonstrated that mutations in other Treg-associated genes, such as CD25 and CTLA4, show an IPEX-like phenotype. We present the case of a 13-year-old girl with CTLA4 haploinsufficiency, suffering from recurrent immune thrombocytopenic purpura and intractable diarrhea. We detected an autoantibody to the AIE-related 75 kDa antigen (AIE-75), a hallmark of the IPEX syndrome, in her serum. She responded well to a medium dose of prednisolone and a controlled dose of 6-mercaptopurine (6-MP), even after the cessation of prednisolone administration. Serum levels of the soluble interleukin-2 receptor and immunoglobulin G (IgG) were useful in monitoring disease activity during 6-MP therapy. In conclusion, autoim mune-mediated mechanisms, similar to the IPEX syndrome, may be involved in the development of enteropathy in CTLA4 haploinsufficiency. Treatment with 6-MP and monitoring of disease activity using serum levels of soluble interleukin-2 recep tor and IgG is suggested for such cases. (Intest Res 2022;20:144-149)
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