Clinical Course and Cytokine Profile of Systemic Juvenile Idiopathic Arthritis in a Patient with Trisomy 21.

Abstract

Trisomy 21 (Down syndrome) is sometimes complicated by congenital heart disease; however, comorbid type I diabetes mellitus and diseases involving autoantibodies, such as Hashimoto disease and Graves disease, are not uncommon. Autoinflammatory diseases such as Kawasaki disease and systemic juvenile idiopathic arthritis are rare. We report a rare case of trisomy 21 with systemic juvenile idiopathic arthritis that responded well to the initial course of methylprednisolone pulse therapy but flared up and was complicated by macrophage activation syndrome (MAS). Subsequent methylprednisolone pulse therapy and cyclosporine resolved this condition. Cytokines were analyzed at several time points during the clinical course and revealed that interleukin-18, interleukin-6, and chemokine ligand 9 levels were elevated at MAS onset in the present patient, even though clinical symptoms had abated. Thus, early analysis of cytokine profiles should be performed to assess MAS risk and determine treatment intensity, even in T21 patients.

• Authors: Yujiro Tanabe, Haruka Ota, Shuya Kaneko, Kanae Tsuno, Makoto Watanabe, Shingo Yamanishi, Hidehiko Narazaki, Ryuji Fukazawa, Masaki Shimizu, Yasuhiko Itoh
• Journal: Journal of Nippon Medical School = Nippon Ika Daigaku zasshi (J Nippon Med Sch) Vol. 90 Issue 5 Pg. 419-424 (Nov 07 2023) ISSN: 1347-3409 [Electronic] Japan
• PMID: 36273908 (Publication Type: Case Reports, Journal Article)

Chemical References

• Cytokines
• Methylprednisolone

Topics

• Humans
• Arthritis, Juvenile (complications, diagnosis, drug therapy)
• Down Syndrome (complications)
• Cytokines
• Macrophage Activation Syndrome (diagnosis, etiology, drug therapy)
• Methylprednisolone
• Disease Progression