Major Features of the Two Most Common Hepatic Porphyrias

Major Features of the Two Most Common Hepatic Porphyrias

Porphyria

Presenting Symptoms

Exacerbating Factors

Most Important Screening Tests*

Treatment

Acute intermittent porphyria

Neurovisceral (intermittent, acute)

Alcohol ingestion

Fasting/crash dieting

Gastric sleeve or bypass surgery

Infections

Medications (mostly cytochrome P-450 inducers)

Organic solvents

Physical or emotional stress/intercurrent illness

Progesterone/progestogens

Urinary PBG and creatinine†

Glucose

Heme

GivosiranGivosiran

Porphyria cutanea tarda

Blistering skin lesions (chronic)

Alcohol ingestion

Estrogens

Halogenated hydrocarbons

Hepatitis C virus

HIV

Iron

Smoking

Urinary or plasma porphyrins

Direct-acting antivirals to cure hepatitis C virus infection

Low-dose chloroquine or hydroxychloroquineLow-dose chloroquine or hydroxychloroquine

Phlebotomy

* In symptomatic phase.

† Creatinine is obtained to normalize urinary PBG levels since urinary dilution varies.

PBG = porphobilinogen.

* In symptomatic phase.

† Creatinine is obtained to normalize urinary PBG levels since urinary dilution varies.

PBG = porphobilinogen.