Table 2

Summary of studies linking acute porphyria with HT and renal impairment

ReferenceStudy typePopulationSubjectsComments
Beattie and Goldberg28 Retrospective cohort (20-year period)UK38 AIP patients with previous attacksDiastolic BP>100 mm Hg in 50%; 1 death due to early onset CRF and malignant HT; 3 deaths due to complications of HT
Laiwah et al 29 Retrospective
case series
Scotland6 AIP patients with previous attacks identified with chronic renal failure and sustained HTObserved vs expected CRF prevalence 6 vs 65 in AIP cases (in remission but with previous acute attacks) compared with 52 per million in general population
Church et al 30 Prospective and retrospective case series (within single kindred)UK26 AIP and 26 non-AIP subjectsOverall in AIP subjects, 62% had HT, 50% had renal impairment; 5 deaths directly related to HT (of whom 3 had renal failure)
Kauppinen and Mustajoki6 Retrospective cohortFinland268 patients in disease register, 206 in follow-up group. No data on 62 (23%)HT and renal failure prevalence higher than general population in some age groups:
HT: 12% (n=5) vs 3.3% in women aged 30–44 years (p=0.03)
56% (n=5) vs 19% in men aged 55–64 years (p=0.03)
Renal failure: 9 subjects (5.7%) overall. 7% (n=3) vs 0.07% of women aged 30–44 years (p=0.0002) and 9.5% (n=2) vs 0.37% of women aged 45–54 years (p=0.01). 4 deaths due to renal failure
Andersson et al 31 (1) Case–control study; (2) retrospective mortality study (12-year period); (3) case seriesSweden(1) 50 AIP (25 overt; 25 latent) with 200 matched controls;
(2) 2122 deceased subjects (33 with AIP);
(3) 8 AIP patients with severe recurrent attacks
(1) HT in 56% of patients with overt AIP, 33% of controls (p=0.041) and 16% with latent AIP (p=0.004). No difference in renal disease (AIP vs controls)
(2) HT registered in 13/19 (68%) with overt AIP and 3/14 (21%) with latent AIP (p=0.008; OR 7.9). Death from MI or stroke was not more common than in non-AIP patients
3/33 (9.1%) AIP patients died from uraemia vs 22/2089 without AIP (1%) (p=0.006; OR 9.4)
(3) 3/8 severe AIP cases had renal impairment (1 due to SLE)
Jeans et al 10 Retrospective cohort study of cumulative mortality over a 50-year periodUSA168 AIP patients;
no data on 32 (19%)
72/136 HT;
53/136 ‘azotemia BUN>25 mg/dl)’;
SIR not calculated. 1 death due to renal failure
Andersson et al 32 Population-basedSweden286/386 eligible patients with AIP screened for low CrCl34/286 (12%) had CrCl <65 ml/min per 1.73 m2;
AIP was sole explanation in 16. 12/16 had HT
Marsden et al 37 Case seriesUK9 patients with AIP and CRFHT and NSAIDs were contributory factors in ∼50%
  • AIP, acute intermittent porphyria; BUN, blood urea nitrogen; CrCl, creatinine clearance; CRF, chronic renal failure; HT, hypertension; MI, myocardial infarction; NSAIDs, non-steroidal anti-inflammatory drugs; SIR, standardised incidence ratio; SLE, systemic lupus erythematosus.