Definition
HIV-associated nephropathy (HIVAN) is a collapsing variant of focal segmental glomerulosclerosis occurring in advanced or untreated HIV. It is strongly linked to APOL1 risk alleles and predominantly affects people of African ancestry.
Clinical presentation
- Heavy (nephrotic-range) proteinuria
- Rapid decline in renal function, often to end-stage disease
- Large, echogenic kidneys on ultrasound (rather than the small kidneys of most CKD)
- Frequently occurs with a low CD4 count / high viral load
Investigations
- Urinary protein quantification; HIV viral load and CD4 count
- Ultrasound: enlarged, bright kidneys
- Renal biopsy: collapsing FSGS with tubular microcysts
Management
- Antiretroviral therapy — the mainstay; viral suppression improves outcomes
- ACE inhibitors/ARBs to reduce proteinuria and blood pressure
- Corticosteroids in selected cases
- Renal replacement therapy/transplantation for end-stage disease
MRCP-specific traps
- Large kidneys + nephrotic syndrome in an HIV-positive patient suggests HIVAN.
- The single most important treatment is antiretroviral therapy, not immunosuppression.
Summary
HIVAN is a collapsing FSGS of advanced HIV, with heavy proteinuria, rapid decline and large echogenic kidneys, linked to APOL1. Treat primarily with antiretroviral therapy, plus RAS blockade and sometimes steroids.
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