以Mycophenolate Mofetil及類固醇治療成功一頑固性Churg-Strauss Syndrome之病例

Decrease in Type 2 Cytokine-Producing Helper T Cells in A Refractory Case of Churg-Strauss Syndrome Successfully Treated with Mycophenolate Mofetil and Prednisolone

Churg Strauss Syndrome是一罕見且原因未明之血管炎。其T細胞激素之變化仍有所爭論，但一般認為乃屬第2型輔助T細胞激素之疾病。第2型輔助T細胞激素如介白素4負青促使B細胞分泌IgE抗體，而介白素5則會使嗜伊紅球增加，然而第1型輔助T細胞激素如干擾素-γ卻會抑制介白素4負責之IgE反應。Mycophenolate Mofetil是一種可以選擇性抑制T細胞和B細胞增殖的藥物，其對T細胞激素之影響仍未定論。我們在此報告一個15歲男生罹患Churg Strauss Syndrome，合併有反覆肺部浸潤及嚴重肋膜積水之病例，以My∞phenolate Mofetil治療後為況完全緩解，並使其血中第2型輔助T細胞激素或少及改變第1型輔助T細胞激素／第2型輔助T細胞激素之平衡。

Churg-Strauss syndrome (CSS) is a rare systemic vasculitis of unknown cause. The ratio of type 1 T helper cells/type 2 T helper cells (Th1/Th2) in CSS is unclear and it has been suggested that CSS may be a Th2-mediated disease. Interleukin-4 (IL-4) and IL-5 are responsible for enhancing B cells IgE production and eosinophilia maturation respectively. Interferon-γ( IFN-γ), which is a Th1 cytokine, acts as a suppressive factor for IL-4-mediated IgE response. Mycophenolate mofetil (MMF) is a morpholinoester of mycophenolic add (MP A) that blocks the proliferative responses of T and B lymphocytes. However, its effect on Th1/Th2 cytokine balance remains unclear. We present herein a 15-year-old boy with CSS involving the upper and lower respiratory tract, skin, and peripheral nervous system. He had recurrent pulmonary infiltration and massive pleural effusion despite treatment with high dosage of prednisolone (1 mg/kg/day). Additional treatment with MMF 750 mg/day Jed to long-term remission and decrease in the percentage of type 2 cytokine-producing Th cells in peripheral blood and serum levels of IL-5.