skip to main content

Acute Intermittent Porphyria CKD — ESENeph MCQ

Instant feedback + full explanation. One question, done properly.

HardChronic Kidney DiseaseAcute Intermittent Porphyria CKDESENeph

A 35-year-old woman presents with recurrent episodes of acute intermittent porphyria (AIP) and developing CKD (eGFR 45 mL/min/1.73m2). What is the mechanism of kidney injury in AIP?

Educational content. Not a substitute for clinical judgement or local policy.

Reveal the answer and explanation

Correct answer: D — Chronic tubulointerstitial nephropathy from porphyrin precursor accumulation (aminolevulinic acid and porphobilinogen) causing oxidative tubular injury; also proximal tubular dysfunction and hypertension

Acute intermittent porphyria causes CKD through chronic accumulation of porphyrin precursors (delta-aminolevulinic acid, porphobilinogen) which are directly nephrotoxic to proximal tubular cells via oxidative stress. Chronic hypertension (from autonomic neuropathy) also contributes. CKD develops in up to 30-60% of long-term AIP patients. Givosiran (RNA interference therapy targeting aminolevulinic acid synthase 1) reduces porphyrin precursors and may slow CKD progression.

Reference: NICE HST16 2021 – Givosiran for AIP; Pallet et al 2015 – Porphyria Nephropathy