Department of Dermatology, Copenhagen University Hospital - Bispebjerg and Frederiksberg, Denmark.
Department of Dermatology, Copenhagen University Hospital - Bispebjerg and Frederiksberg, Denmark; Department of Pharmacy, University of Copenhagen, Denmark.
Photodiagnosis Photodyn Ther. 2022 Jun;38:102793. doi: 10.1016/j.pdpdt.2022.102793. Epub 2022 Mar 2.
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.
红细胞生成性原卟啉症（EPP）是由于将原卟啉 IX（PpIX）转化为血红素的酶缺乏导致 PpIX 积累引起的；导致光敏性和肝毒性。西咪替丁可能通过抑制 δ-氨基酮戊酸合酶影响血红素生物合成。我们在本科室报告了用西咪替丁治疗 EPP 的病例，并进行了文献复习。
系统搜索了描述用西咪替丁治疗 EPP 患者的文献。在此基础上，我们在 2020 年和 2021 年春夏季在本科室用西咪替丁治疗 EPP 患者。收集他们治疗前和治疗 4 个月期间的红细胞 PpIX 水平和标准血液及肝脏参数。通过问卷调查，询问患者对光敏性变化、副作用的看法，以及是否愿意在 2022 年春季恢复治疗。
文献检索确定了 9 例用西咪替丁治疗的患者。4 例为门诊患者，报告光敏性降低。在本科室，18 例门诊患者开始治疗。15 例患者每天口服西咪替丁治疗 4 个月或更长时间，红细胞 PpIX 显著下降，中位数为 20%（范围：-18%至 53%），治疗 4 个月后。15 例患者中的 11 例报告治疗期间光敏性下降，11 例患者不确定，1 例患者光敏性无变化。仅报告轻微副作用。14 例患者要求在 2022 年春季恢复治疗。
这些病例表明西咪替丁可降低 EPP 患者的红细胞 PpIX。
