Background: Acute intermittent porphyria (AIP) is an autosomal dominant hepatic porphyria characterized by a partial deficiency of hydroxymethylbilane synthase involved in heme biosynthesis. It is difficult for all patients to achieve complete control of AIP episodes.
Method: We report on a 20-year-old female woman who suffered from recurrent abdominal pain and was diagnosed as "acute intermittent porphyria". She failed to respond to conventional symptomatic treatment and subsequently was treated with gonadotropin-releasing hormone analogues (GnRH) combined with estrogen for one year.
Result: The case did not experience acute attacks and obtained long-term clinical remission to date.
Conclusions: GnRH combined with estrogen, one of the treatment options for menstrual-associated AIP, might induce long-term remission.