Background
Pathophysiology
- Porphyrias encompass a group of 8 genetic disorders and may be1-6
- Inherited in an autosomal dominant (e.g., acute hepatic porphyria [AHP]) or recessive X-linked pattern (e.g., congenital erythropoietic porphyria [CEP]); or6
- Sporadic (e.g., porphyria cutanea tarda [PCT])6
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Precipitating Factors
- Certain precipitating factors can cause symptomatic disease and strongly influence the occurrence and severity of signs and symptoms of porphyria (see table).1,2
- The most frequent recognised cause of acute attacks is medications.15
- An observational study in 90 patients diagnosed with acute intermittent porphyria (AIP) reported that medications and weight-loss diets triggered acute attacks in 37% and 22% of cases, respectively.16
- Other precipitants are metabolic, emotional, or physical stresses, including those brought on by intercurrent infection or surgery.3,7,9
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This program was sponsored and funded by Alnylam Pharmaceuticals, Inc, based on an interview with Herbert L. Bonkovsky, MD, FACP, FACG, FAGA, FAASLD. Dr Bonkovsky received a fee for participation in this program. AS1-CEMEA-00184 . Date of preparation: February 2021.
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