Acute intermittent porphyria: analgesia can be dangerous

BMJ Case Rep. 2019 Sep 30;12(9):e231133. doi: 10.1136/bcr-2019-231133.

Abstract

Acute intermittent porphyria (AIP) is a rare condition, a metabolic disorder of the haem biosynthesis. An acute crisis of AIP can present as a combination of symptoms, such as abdominal pain, autonomic dysfunction, hyponatremia, muscle weakness and neurological symptoms in the absence of others obvious causes. We report the case of a 53-year-old woman, who was previously diagnosed with AIP 5 weeks after therapeutic suspension has developed an acute disease exacerbation. During hospitalisation, further exacerbation has occurred after analgesia with metamizole. Glucose and hemin infusions resulted in slow improvement. Physical rehabilitation was crucial to peripheral polyneuropathy recovery. Taking into account the porphyrinogenic effect described for metamizole, this drug might have triggered the second attack. Clinical history was sufficient to suspect the diagnosis and to start the treatment immediately, preventing important sequelae.

Keywords: contraindications and precautions; metabolic disorders; pain; physiotherapy (rehabilitation); safety.

Publication types

  • Case Reports

MeSH terms

  • Abdominal Pain
  • Anti-Inflammatory Agents, Non-Steroidal / administration & dosage
  • Anti-Inflammatory Agents, Non-Steroidal / adverse effects*
  • Dipyrone / administration & dosage
  • Dipyrone / adverse effects*
  • Disease Progression
  • Female
  • Glucose / administration & dosage
  • Hemin / administration & dosage
  • Humans
  • Hyponatremia
  • Infusions, Parenteral
  • Middle Aged
  • Muscle Weakness / drug therapy*
  • Muscle Weakness / physiopathology
  • Pain Management / methods*
  • Porphyria, Acute Intermittent / diagnosis
  • Porphyria, Acute Intermittent / drug therapy*
  • Porphyria, Acute Intermittent / physiopathology
  • Treatment Outcome

Substances

  • Anti-Inflammatory Agents, Non-Steroidal
  • Dipyrone
  • Hemin
  • Glucose