Complications
Hypertension is common during acute attacks, but the risk of long-term hypertension also may be increased. This should be treated to avoid complications, including renal damage.[34]
Some patients with AIP develop chronic renal failure. The mechanism is unclear, but chronic hypertension may contribute. Limited biopsy studies have shown an interstitial nephropathy.[35] A common variant of PEPT2, which transports delta-aminolevulinic acid, may predispose.[36] Plasma porphyrins may increase in AIP patients with renal insufficiency and rarely cause blistering photosensitivity that resembles porphyria cutanea tarda.[37] Some patients with AIP and renal disease have successfully undergone renal transplantation, or combined liver and renal transplantation.[38][39]
The risk of liver cancer is clearly increased in AIP and other acute porphyrias, and is usually not associated with an increase in serum alpha-fetoprotein.[40] Screening by imaging is recommended 6-month intervals after 50 years of age in patients who have a history of symptoms, especially if urinary porphobilinogen remains elevated.[41]
Use of this content is subject to our disclaimer