Efficacy of oral tofacitinib in refractory chronic spontaneous urticaria and urticarial vasculitis

The treatment of chronic urticaria (CU) is difficult, currently, antihistamines (AH) are the mainstay of treatment, however, up to 40% of patients do not respond to even high (four-fold) daily doses of AH. Tofacitinib is, a small-molecule that blocks JAK1/3 and inhibits intracellular signaling of multiple key cytokines involved in the inflammatory cascade and its beneficial effects were reported in patients with mast cell activation disease but there is no report in patients with urticaria. Here, we present four cases of refractory CU and one case of urticarial vasculitis (UV) that were managed with tofacitinib. Despite the long-term unresponsiveness of various treatments in our patients, the addition of tofacitinib significantly improved the urticarial activity and ultimately led to tapering and discontinuation of cyclosporine or AH. In conclusion, tofacitinib appears to downregulate inflammatory phenomena associated with mast cells and might be a new therapeutic option for patients with refractory CU or UV.