Table 1.
Summary of case presentations.
| Case 1 | Case 2 | |
|---|---|---|
| Initial BP (mmHg) | 200/120 | 208/131 |
| Presenting symptoms | Erythromelalgia, occipital headaches, Vertigo, one episode of dyspnoea + eye floaters + syncope (1-2 minutes) | Acrocyanosis, Vasculitic skin rashes, nonhealing toe ulceration |
| Past medical history | Spondylolisthesis | Migraines with aura |
| Family history | Father: Vasovagal syncope, myocardial infarction | Unremarkable |
| Social history | Ex-smoker | 20 units of alcohol per week |
| Investigations and results | ||
| Fundus examination | Papilloedema with nasal margin blurring | Arteriovenous nipping with nasal margin blurring |
| Urine dipstick | Unremarkable | Unremarkable |
| FBC | WBC—15.8 × 109/L; RBC—6.20 × 109/L; Hct -0.534 L/L PLT −953 × 109/L; Neutrophil count −12.10 × 109/L |
PLT -1096 x109/L |
| Liver function tests | Not indicated | Total bilirubin—38 μmol/L; ALT—42 U/L; Gamma GT—94 U/L |
| Electrocardiogram | Sinus rhythm 70 bpm, left axis deviation, biphasic T waves in leads V5 and V6 | Unremarkable |
| Echocardiogram | Moderate global left ventricular hypertrophy with 1.4 cm wall thickness | Mild aortic dilatation |
| Creatinine | 146 μM | 88 μM |
| Renin | 107 mU/L | 80 mU/L |
| Autoimmune screen | Negative | Negative |
| Vasculitic skin rash biopsy | N/A | Unremarkable |
| Contrast CT abdomen | N/A | Enlarged left adrenal gland, lower abdominal lymphadenopathy |
| Inert gas rebreathing studies (clinical research facility) | N/A | Peripheral vascular resistance -1840 dynes/s/cm5 |
| Interventions | Renal artery angioplasty, aspirin 75 mg, intermittent venesection, interferon-alpha 2A | Aspirin 75 mg, long-acting nifedipine |
| Outcomes | Normalisation of BP and renal parameters, minor side effects from interferon therapy (decreased libido and reported thinning of hair), now managed with single dose of long-acting nifedipine | Normalisation of BP, no adverse outcomes reported, toe ulceration now healed |