TOPIC DISCUSSION: Pure Thin Basement Membrane vs " Hereditary Nephritis with Thin Basement like features"

Classically, Hereditary Nephritis or Alports Syndrome shows the basket weaving appearing or thin and think alternating basement membrane.  Early in the course in some males and almost all females, the only finding on kidney biopsy might be thin basement membrane. The pathologist needs to carefully look for any subtle changes in lamination and change in thickness in other areas as it makes it almost impossible in that stage to differentiate from pure Thin Basement Membrane disease.  When its not possible to differentiate its called " Hereditary Nephritis with thin basement membrane phenotype".  Those cases have to be clinically followed closely in case they develop renal insufficiency.

Ref:
ASN Nephsap July 2011, Pathology