Abstract

Abstract Trisomy 21 (T21) is strongly associated with autoimmune disease, including psoriasis. Little is known about biologic outcomes for severe psoriasis in T21. T21-associated immune dysregulation has implications for efficacy, safety and drug survival of biologic medications. The aim was to review the outcomes of patients with T21 and severe psoriasis treated with biologic agents or Janus kinase inhibitors (JAKi) in our region. Patients were identified via databases of the regional dermatology departments in the province of Munster, Ireland. Information on demographics, comorbidities, previous and current therapies and response to therapies was retrospectively collated. Adequate treatment response was defined as a physician global assessment score of zero (indicating clear skin), or one (indicating almost clear skin). Twenty-one patients were identified (mean age 24.7 years). Most (76%) did not have a first-degree relative with psoriasis. One-third (33%) of patients had arthritis, but all children (n = 6) had arthritis. Ninety per cent (n = 18/20) of trials of tumour necrosis factor (TNF)-α inhibitors failed. Almost two-thirds (n = 7/11) of patients achieved adequate response with ustekinumab. All three patients treated with tofacitinib achieved adequate response following at least three biologic failures. The mean number of biologic/JAKi therapies received was 2.1, with an overall biologic/JAKi survival rate of 36%. Overall, 81% (n = 17/21) of patients required a switch from their index biologic treatment due to failure. The four patients who remained on their index biologic all received ustekinumab. In patients with T21 and severe psoriasis, failure of TNF-α inhibition is common, and ustekinumab therapy should be considered first line. There is an emerging role for JAKi.

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