Pathophysiology & triggers
MECHANISM: cholesterol crystals shower from a disrupted atherosclerotic plaque (usually thoracoabdominal aorta) and embolise to small arteries — kidneys, skin, GI tract, retina, CNS. The crystals lodge in arterioles, trigger inflammation and downstream ischaemia.
Classic Triggers
- Cardiac catheterisation / PCI (commonest)
- Aortic surgery, EVAR / TEVAR
- Carotid surgery
- Thrombolysis (especially streptokinase, tPA)
- Initiation of warfarin or heparin (haemorrhage into plaque)
- Spontaneous (in heavily atheromatous aortas)
Risk Factors
- Age > 60
- Male
- Smoker
- Established atherosclerosis (CAD, PAD, AAA)
- Diabetes, hypertension, dyslipidaemia
- Pre-existing CKD
Clinical features & diagnosis
TIME COURSE: 1–4 weeks after trigger (rarely > 8 weeks). Often described as 'stepwise' AKI.
Renal
- Sub-acute progressive AKI
- Hypertension (often accelerated)
- Mild proteinuria, bland sediment
Skin
- Livedo reticularis (especially flanks, lower limbs, abdomen)
- Blue/purple toes (peripheral pulses preserved — key distinguishing feature from acute limb ischaemia)
- Digital gangrene
- Painful nodules in lower limbs
Eye
- Hollenhorst plaques (bright orange-yellow cholesterol crystals at retinal arteriolar branch points) on fundoscopy
- Amaurosis fugax
Gi
- Abdominal pain, GI bleeding, ischaemic colitis, pancreatitis
Cns
- Confusion, TIA, focal deficits
Systemic
- Fever, weight loss, malaise (mimics vasculitis)
Bloods
- Creatinine rising
- Eosinophilia (transient, 60–80% of cases)
- ESR/CRP raised
- Low complement (especially C3) in 30%
- Often misdiagnosed as ANCA vasculitis — check ANCA, anti-GBM (negative)
Imaging
- Renal USS — usually normal
- Aortic CT/MRA may show extensive plaque
Diagnosis
- Often clinical (compatible history + features)
- Tissue diagnosis: SKIN BIOPSY from livedo or nodule — biconvex cholesterol clefts in arterioles (easiest)
- Kidney biopsy: cholesterol clefts in interlobular arteries (definitive but invasive)
- Muscle, GI tract biopsy where appropriate
Management & prognosis
Prevention
- Minimise unnecessary aortic instrumentation in high-risk patients
- Radial access for coronary angiography preferred where feasible
- Use lowest contrast volume, minimise catheter exchanges
ACUTE MANAGEMENT (supportive):
- Stop offending agent if possible (e.g. thrombolytic completed; review anticoagulation indication)
- High-intensity statin (atorvastatin 80 mg) — observational benefit; stabilises plaque
- BP control — ACEI/ARB unless contraindicated
- Manage CKD complications (acidosis, hyperkalaemia, anaemia, MBD)
- Treat heart failure (diuretics, RAS inhibition)
- Nutritional support (catabolic illness)
- Wound care, pain control for skin and toe ischaemia
- Vascular surgery review for limb-threatening ischaemia
Dialysis
- Indicated for standard AKI complications (refractory hyperkalaemia, fluid overload, uraemia, severe acidosis)
- 30–40% will need dialysis at some point
- Recovery may be slow over weeks–months
Controversial Therapies
- CORTICOSTEROIDS: no consistent benefit; reserved for selected refractory cases with marked systemic inflammation
- ICLOPROST / PROSTACYCLIN ANALOGUES: case series for digital ischaemia
- LDL APHERESIS: anecdotal use in refractory cases
Anticoagulation
- Weigh original indication vs ongoing crystal showers
- If anticoagulation precipitated the event, consider stopping when safe
- DOACs vs warfarin — no clear data showing one is safer in this context
Prognosis
- Mortality at 1 year: 25–40% (often cardiovascular)
- 20–40% recover useful kidney function
- 30–40% require dialysis; some come off, some are long-term
- Recurrent embolic showers can occur
- Long-term cardiovascular risk reduction (statin, antiplatelet, BP, glucose, smoking) is essential






