Table 1.
Proposed stages of progression of Fabry nephropathy in male patients
| Time of evolution | |||||
|---|---|---|---|---|---|
| (years) | Urinanalysis | Level of GFR | Pathology | Efficacy of ERT | |
| Stage I | 0–5 | Normal | <90 ml/min <140 ml/min | Gb3 deposits mainly in podocytes and capillaries +/++ | Incomplete clearance of deposits after 5 years |
| Stage II | 6–19 | Microalbuminuria (30–300 mg/day) | >140 ml/min | Gb3 deposits ++/+++ Mesangial expansion + | Possible reversibility of hyperfiltration; no data on microalbuminuria Point of no return? |
| Stage III | 20–29 | Overt proteinuria (>300 mg/day) | >60 ml/min <140 ml/min | Gb3 deposits ++/+++ Mesangial expansion ++ Glomerulosclerosis +/++ Tubular atrophy + Arterial remodelling +/++ | No significant effect on proteinuria Stabilization of GFR in the majority of patients |
| Stage IV | >30 | Overt proteinuria Proteinuria of nephrotic range Nephrotic syndrome (rare) | <60 ml/min to ESRD | Gb3 deposits ++/+++ Glomerulosclerosis ++/+++ Interstitial fibrosis ++/+++ Tubular atrophy ++/+++ Arterial remodelling ++/+++ | No stabilization but slows the rate of progression of chronic renal insufficiency |
CRI, chronic renal insufficiency; ESRD, end-stage renal disease; ERT, enzyme replacement therapy; Gb3, globotriaosylceramide; GFR, glomerular filtration rate; +, mild; ++, moderate; +++, severe.