Acute intermittent porphyria: general aspects with focus on pain

Despite medical advances, the diagnosis and management of acute intermittent porphyria continues to be challenging. Acute pain is one of the most important clinical manifestations in acute intermittent porphyria, but management and pain assessment have been poorly studied in these patients. The lack of information and evidence based recommendations regarding these topics in the medical literature is certainly surprising. Furthermore, pain management is discussed based on extrapolating concepts adopted for other pain syndromes. An important arsenal of medications, including reportedly safe opioid and non-opioid analgesics, is available for use in this type of patient. In addition to conducting an extensive review of the current literature, the present article aims to show the general aspects of a disease that generates a challenge for the clinician, emphasizing what is related to pain as a cardinal symptom and to create awareness about the need for studies supporting the development of guidelines, based on evidence obtained from analgesic management in patients presenting with this disorder. A systematic assessment directed at understanding the physiopathological processes underlying acute pain combined with a stepwise approach to pain management with safe opioid and non-opioid drugs constitutes the fundamental basis for a successful pain management program in patients with acute intermittent porphyria.