- Accounts for ~30% of nephrotic syndrome in adults
- 75% of cases are primary, with most of those anti-PLA2R antibody +
- 25% of cases are secondary, associated w/Hepatitis B or C, SLE, meds (e.g. NSAIDs, gold, penicillamine, other), syphilis, malignancy (e.g. lung, colon, other)
- Develops over months
- Symptoms related to volume overload
- GFR may be preserved
- Unexplained venous thrombosis
- Peripheral edema
- Sometimes periorbital and hand edema
- BP can be normal
- Nephrotic range proteinuria: > 3.5g protein in a 24h urine collection
- Spot urine protein/creat ratio: Normal < 150mg/g; 3500mg/g roughly =s 3.5g protein/24h collection
- Spot urine albumin/creat ratio: Normal < 30mg/g; moderately increased albuminuria 30-300mg/g; severely increased albuminuria > 300mg/g; 3500mg/g roughly =s 3.5g albumin/24h collection
- GFR often preserved
- Low serum albumin, hyperlipidemia
- Presence of serum anti-Phospholipase A2 Receptor (PLA2R) antibodies specific for primary disease