Acute intermittent porphyria

Patients with AIP should be evaluated at least yearly.

• Urinary porphobilinogen (PBG) should be followed and may have prognostic value.

• Hypertension should be controlled, and renal and hepatic function followed.

• Chronic, mild increases in alanine aminotransferase are common, but the risk of cirrhosis is not known to be increased.

• Liver imaging is recommended at 6-month intervals after age 50 years in patients who have a history of symptoms, especially if urinary PBG remains elevated, for early detection of hepatocellular carcinoma.