Presenting features | Number of patients with features (%) |
---|---|
Sex | |
Female:male | 7 (64%):4 (36%) |
Ethnicity | |
Caucasian | 8 (73%) |
Asian | 2 (18%) |
Afro-Caribbean | 1 (9%) |
Systemic features | 4 (36%) |
Fever | 4 (36%) |
Weight loss >5% of body weight | 4 (36%) |
Myalgia | 1 (9%) |
Arthralgia or arthritis | 1 (9%) |
Skin involvement | 1 (9%) |
Livedo reticularis | 0 (0%) |
Purpura | 1 (9%) |
Other skin vasculitis (vasculitis different from previous, such as subcutaneous oedema, Raynaud’s phenomenon) | 1 (9%) |
Mucous membranes/eyes | 1 (9%) |
Red eye conjunctivitis | 1 (9%) |
Arterial hypertension >95% percentile for age | 8 (73%) |
Renal involvement | 1 (9%) |
Proteinuria (>0.3 g/24 hr or >20 mmol/mg creatinine) | 1 (9%) |
Haematuria (≥5 rbc/hpf or rbc casts) | 1 (9%) |
Rise in creatinine >10% or creatinine clearance (GFR) decrease >25% | 1 (9%) |
Neurological involvement | 4 (36%) |
Headache | 4 (36%) |
Organic confusion/cognitive dysfunction | 1 (9%) |
Meningitis/encephalitis | 0 (0%) |
Seizures (not hypertensive) | 2 (18%) |
Stroke | 2 (18%) |
Gastrointestinal involvement | 1 (9%) |
Abdominal pain | 1 (9%) |
Peritonitis | 0 (0%) |
Blood in the stools or bloody diarrhoea | 0 (0%) |
Bowel ischaemia/perforation | 0 (0%) |
Cardiovascular involvement | 5 (45%) |
Loss of pulses | 2 (18%) |
Bruits over accessible arteries | 5 (45%) |
Blood pressure discrepancy | 2 (18%) |
Claudication of extremities | 1 (9%) |
Ischaemic cardiac pain | 2 (18%) |
Cardiomyopathy | 3 (27%) |
Congestive cardiac failure | 2 (18%) |
Valvular heart disease | 1 (9%) |
Pericarditis | 1 (9%) |
Pulmonary involvement | 3 (27%) |
Wheeze or expiratory dyspnoea | 3 (27%) |
Pleural effusion | 3 (27%) |
Infiltrate | 2 (18%) |
Massive haemoptysis/alveolar haemorrhage | 0 (0%) |
Respiratory failure | 3 (27%) |