Acute Intermittent Porphyria: Current Perspectives And Case Presentation

Figures & data

Figure 1 Haem synthesis pathway.

Note: Adapted from Stein PE, Badminton MN, Rees DC. Update review of the acute porphyrias. Br J Haematol. 2017;176(4):527–538. © 2016 John Wiley & Sons Ltd.⁵

Table 1 Disease States Of AIP

AIP Subgroup	Disease Type
1.	Asymptomatic high porphyrin precursors
2.	Asymptomatic without porphyrin precursor elevations (latent disease)
3.	Symptomatic patients with less than four attacks per year
4.	Symptomatic patient with more than four episodes per year

Table 2 Pharmacologic Therapies Implicated In Precipitating Episodes

Antimicrobials	Endocrine	Cardiovascular	Antiepileptics	Anesthetics	Oncology	Pain	Miscellaneous
Sulfamethoxazole/Trimethoprim	Progestins	Nifedipine	Phenytoin, Fosphenytoin	Ethosuximide	Doxorubicin	Ketorolac	Metoclopramide
Sulfasalzine	Testosterone	Nicardipine	Carbamazepine	Etomidate	Cyclophosphamide		Modafinil
Nitrofurantoin	Megestrol	Clonidine	Oxcarbazepine	Ketamine	Ifosfamide		Bosentan
Nafcillin	Danazol	Methyldopa	Phenobarbital	Barbiturates	Vemurafenib		Primidone
Erythromycin		Nimodipine	Topiramate	Benzodiezapines	Enzalutamide		Hydroxyzine
Dapsone		Hydralazine			Flutamide		Oxybutynin
Rifampin					Busulfan
Isoniazid					Procarbazine
Pyrazinamide					Lomustine
Efavirenz
Etravirine
Nevirapine
Nelfinavir
Ritonavir
Atazanavir
Delavirdine
Indinavir
Ritonavir
Fluconazole
Griseofulvin
Itraconazole
Ketoconazole
Voriconazole

Notes: This table lists the most common drugs seen in clinical practice. A comprehensive list is available at https://porphyriafoundation.org/drug_database.

Table 3 Diagnostic Testing During Acute Episode Of AIP

Study	Urine	Serum	Fecal
PBG	↑↑↑ 50–200 mg/L	↑	–
Porphyrins	↑	↑/−	–
δ-aminolevulinate acid (ALA)	↑↑ 25–100 mg/L	↑	–

Stein PE, Badminton MNRees DC. Update review of the acute porphyrias. Br J Haematol. 2017;176(4):527–538. doi: 10.1111/bjh27982422

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