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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References
- Elexacaftor–tezacaftor–ivacaftor for cystic fibrosis with a single Phe508del allele.N Engl J Med. 2019; 381: 1809-1819
- Serum sickness-like reaction following initiation of elexacaftor/tezacaftor/ivacaftor therapy.Pediatr Pulmonol. 2020; 55: 2846-2847
- ‘Triple therapy’ (elexacaftor, tezacaftor, ivacaftor) skin rash in patients with cystic fibrosis.Postgrad Med J. 2020; (postgradmedj-2020-139264)
- Ivacaftor drug desensitization.Pediatr Pulmonol. 2019; 54: 672-674
- Drug allergy to CFTR modulator therapy associated with lumacaftor-specific CD4(+) T lymphocytes.J. Allergy Clin Immunol. 2020;
- Lumacaftor/ivacaftor in patients aged 6-11 years with cystic fibrosis and homozygous for F508del-CFTR.Am J Respir Crit Care Med. 2017; 195: 912-920
- Immune pathomechanism and classification of drug hypersensitivity.Allergy. 2019; 74: 1457-1471
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Article info
Publication history
Published online: July 12, 2022
Accepted:
June 25,
2022
Received in revised form:
June 23,
2022
Received:
April 1,
2022
Identification
Copyright
© 2022 European Cystic Fibrosis Society. Published by Elsevier B.V. All rights reserved.
