Summary of studies linking acute porphyria with HT and renal impairment
| Reference | Study type | Population | Subjects | Comments |
|---|---|---|---|---|
| Beattie and Goldberg28 | Retrospective cohort (20-year period) | UK | 38 AIP patients with previous attacks | Diastolic 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 | Scotland | 6 AIP patients with previous attacks identified with chronic renal failure and sustained HT | Observed 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) | UK | 26 AIP and 26 non-AIP subjects | Overall 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 cohort | Finland | 268 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 series | Sweden | (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 period | USA | 168 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-based | Sweden | 286/386 eligible patients with AIP screened for low CrCl | 34/286 (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 series | UK | 9 patients with AIP and CRF | HT 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.