Cimetidine for erythropoietic protoporphyria.
Cimetidine
Erythropoietic protoporphyria
Hepatology
Photosensitivity
Protoporphyrin IX
Questionnaire
Journal
Photodiagnosis and photodynamic therapy
ISSN: 1873-1597
Titre abrégé: Photodiagnosis Photodyn Ther
Pays: Netherlands
ID NLM: 101226123
Informations de publication
Date de publication:
Jun 2022
Jun 2022
Historique:
received:
04
02
2022
revised:
18
02
2022
accepted:
28
02
2022
pubmed:
5
3
2022
medline:
9
6
2022
entrez:
4
3
2022
Statut:
ppublish
Résumé
Erythropoietic protoporphyria (EPP) is caused by deficiency of the enzyme converting protoporphyrin IX (PpIX) into heme resulting in accumulation of PpIX; leading to photosensitivity and liver toxicity. Cimetidine might inhibit δ-aminolevulinic acid synthase influencing the heme biosynthesis. We present cases with EPP treated with cimetidine at our department, and a literature review. Systematic searches were performed to identify literature describing EPP patients treated with cimetidine. On that ground we treated EPP patients with cimetidine through spring and summer in 2020 and 2021 at our department. Their erythrocyte PpIX level and standard blood and liver parameters were collected before and during 4 months of treatment. Using a questionnaire, patients were asked about change in photosensitivity, side effects, and whether they would like to resume treatment in the spring of 2022. Literature searches identified 9 patients treated with cimetidine. Four were outpatients reporting decreased photosensitivity. At our department 18 outpatients started treatment. Fifteen used oral cimetidine daily for 4 months or more providing a significant decrease in erythrocyte PpIX with a median of 20% (range: -18% to 53%) after 4 months. Eleven of the 15 patients reported a decrease in photosensitivity during treatment, 3 patients were unsure, and 1 patient experienced unchanged photosensitivity. Only mild side effects were reported. Fourteen patients requested to resume treatment in the spring of 2022. These cases suggest that cimetidine can lower erythrocyte PpIX in patients with EPP.
Sections du résumé
BACKGROUND
BACKGROUND
Erythropoietic protoporphyria (EPP) is caused by deficiency of the enzyme converting protoporphyrin IX (PpIX) into heme resulting in accumulation of PpIX; leading to photosensitivity and liver toxicity. Cimetidine might inhibit δ-aminolevulinic acid synthase influencing the heme biosynthesis. We present cases with EPP treated with cimetidine at our department, and a literature review.
METHODS
METHODS
Systematic searches were performed to identify literature describing EPP patients treated with cimetidine. On that ground we treated EPP patients with cimetidine through spring and summer in 2020 and 2021 at our department. Their erythrocyte PpIX level and standard blood and liver parameters were collected before and during 4 months of treatment. Using a questionnaire, patients were asked about change in photosensitivity, side effects, and whether they would like to resume treatment in the spring of 2022.
RESULTS
RESULTS
Literature searches identified 9 patients treated with cimetidine. Four were outpatients reporting decreased photosensitivity. At our department 18 outpatients started treatment. Fifteen used oral cimetidine daily for 4 months or more providing a significant decrease in erythrocyte PpIX with a median of 20% (range: -18% to 53%) after 4 months. Eleven of the 15 patients reported a decrease in photosensitivity during treatment, 3 patients were unsure, and 1 patient experienced unchanged photosensitivity. Only mild side effects were reported. Fourteen patients requested to resume treatment in the spring of 2022.
CONCLUSIONS
CONCLUSIONS
These cases suggest that cimetidine can lower erythrocyte PpIX in patients with EPP.
Identifiants
pubmed: 35245673
pii: S1572-1000(22)00082-5
doi: 10.1016/j.pdpdt.2022.102793
pii:
doi:
Substances chimiques
Protoporphyrins
0
Heme
42VZT0U6YR
Cimetidine
80061L1WGD
Ferrochelatase
EC 4.99.1.1
Types de publication
Journal Article
Review
Langues
eng
Sous-ensembles de citation
IM
Pagination
102793Informations de copyright
Copyright © 2022. Published by Elsevier B.V.