Elexacaftor, tezacaftor and ivacaftor: a case of severe rash and approach to desensitisation

BMJ Case Rep. 2022 Mar 2;15(3):e247042. doi: 10.1136/bcr-2021-247042.

Abstract

We present a case of severe rash following induction of elexacaftor, tezacaftor and ivacaftor (ELX/TEZ/IVA) in a young adult male cystic fibrosis patient. While rash is a commonly reported side effect which resolves in 1-2 weeks with minimal intervention, our patient had presented with fever and widespread rash prompting medication cessation. After a washout period, reintroduction with 1/2 tablet of ELX/TEZ/IVA produced a similar systemic response within 24 hours. Repeat attempt, this time with 1/8 tablet and increasing in increments of an eighth daily, was successful and has allowed our patient to experience the transformative benefits of ELX/TEZ/IVA including improved pulmonary function and reduced episodes of infective exacerbation. This case illustrates one of the most common side effects of ELX/TEZ/IVA triple therapy, and our experience of desensitisation to ELX/TEZ/IVA in a challenging case of rash.

Keywords: cystic fibrosis; drugs: respiratory system; unwanted effects / adverse reactions.

Publication types

  • Case Reports

MeSH terms

  • Aminophenols
  • Benzodioxoles
  • Chloride Channel Agonists* / adverse effects
  • Cystic Fibrosis Transmembrane Conductance Regulator / genetics
  • Drug Combinations
  • Exanthema* / chemically induced
  • Humans
  • Indoles
  • Male
  • Pyrazoles
  • Pyridines
  • Pyrrolidines
  • Quinolones
  • Young Adult

Substances

  • Aminophenols
  • Benzodioxoles
  • Chloride Channel Agonists
  • Drug Combinations
  • Indoles
  • Pyrazoles
  • Pyridines
  • Pyrrolidines
  • Quinolones
  • tezacaftor, ivacaftor drug combination
  • Cystic Fibrosis Transmembrane Conductance Regulator
  • elexacaftor