Background Therapeutic use of female sex hormones can potentially trigger acute attacks in genetically predisposed women, however, many women with acute porphyria tolerate this treatment well. Balancing the risks and benefits of hormonal drug treatment can be challenging in acute porphyrias, and there is a need for a more comprehensive knowledge base to establish robust guidelines.

Objective Our aim of this study was to investigate the current practices among porphyria specialists regarding recommendations for choice of contraceptive methods, menopausal replacement therapy (MHT) and follow-up in acute porphyrias. Additionally, we sought to assess the clinical experiences and outcomes associated with use of hormonal drugs in Norwegian porphyria patients.

Materials and Methods We circulated an electronic survey to porphyria specialist centers within the International Porphyria Network. The survey inquired about their recommendations and follow-up in different clinical scenarios related to use of hormonal drugs in patients with active or latent acute porphyrias. From the Norwegian Porphyria Registry, data reported from 2002–2022 were extracted to investigate patient-reported drug use, tolerance, and any associated acute attacks.

Results Among 24 respondents from 22 centers across Europe, North and South America, South Africa, Taiwan and Australia, 18 would recommend non-hormonal contraceptive methods for women with active porphyria, such as the copper intrauterine device (IUD), followed by the progestin IUD, which was recommended by 5. For treatment of menopausal symptoms, 21 recommended non-hormonal drugs or local hormonal therapy only. The majority recommended to monitor porphyrin precursors during any hormonal treatment, including MHT. However, there was significant variation in the suggested frequency and duration of this monitoring. In the Norwegian Porphyria Registry, 67 out of 196 women reported use of hormonal drugs. Twenty-two of these women reported attacks likely induced by these drugs, although the majority occurred prior to 2002, which is when the registry was established.

Conclusions Porphyria specialists tend to be cautious when recommending systemic hormonal therapy and often favoring IUDs for contraception. Regular monitoring of porphyrin precursors appears to be widespread. Data from the Norwegian Porphyria Registry suggest that hormonal medications are generally well-tolerated, with minimal reports of attacks in recent years. Menopausal hormone therapy has shown good tolerability, nevertheless many specialist centers do not recommend MHT. Our data indicate that there are variations in clinical practice among Ipnet specialist centres and the availability of common guidelines on hormonal therapy in acute porphyria would be beneficial for patients.