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A case of Elexacaftor-Tezacaftor-Ivacaftor induced rash resolving without interruption of treatment

      Highlights

      • Elexacaftor-Tezacaftor-Ivacaftor is a new triple therapy combination that is a CFTR modulator and has demonstrated marked improvement in lung function in persons with cystic fibrosis.
      • Triple therapy is known to cause a rash as an adverse event, and this has been demonstrated in clinical trials with a predisposition for females.
      • Previous case reports have shown desensitization, steroid therapy, and complete cessation of the drug as methods of dealing with this adverse event.
      • The immunological basis for this cutaneous adverse reaction is unclear.
      • This case reports a patient continuing the drug despite the rash with final resolution of the rash with no residual scarring or discoloration.

      Abstract

      The triple combination of Elexacaftor-Tezacaftor-Ivacaftor (ELX-TEZ-IVA) has been shown to markedly improve lung function in persons with cystic fibrosis (pwCF). An important adverse effect of the drug is rash, which was reported in clinical trials and highlighted in case reports. Our report demonstrates a similar adverse event with the drug in one of our patients with spontaneous resolution of the rash not necessitating cessation of treatment or desensitization to the drug as were done in other cases. We highlight through our report the heterogeneity of the clinical presentation of the rash when on triple therapy and the need for further studies to understand the immunological mechanism of this adverse event.
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