Table 3.

Histological Findings in FD Nephropathy.

Vacuolations in podocytes, mesangial, endothelial, and epithelial cells of the distal tubules; loops of Henle; and collecting ducts. Involvement of proximal tubular cells is uncommon.

Vascular lesions include deposits in smooth muscle cells and endothelial cells, sometimes associated with hyaline deposits in arteries and arterioles.

Segmental or global glomerulosclerosis, interstitial fibrosis, tubular atrophy, and thickening of the vascular walls.

Immunofluorescence microscopy: deposits characteristic of immunocomplexes are not found, but nonspecific findings such as IgM or C3 may be identified in areas of sclerosis.

Electronic microscopy: GL3 deposits are observed inside lysosomes of different cell types and can take forms known as “myelin figure,” “onion skin,” or “zebra bodies.”

Effacement of foot processes.

Note. FD = Fabry disease.