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Diagnosis: Cellular Variant of Focal Segmental Glomerulosclerosis

The Columbia classification of focal segmental glomerulosclerosis (FSGS) describes 5 variants, of which the cellular variant is the rarest. This variant is more common in children than in adults, and typically presents with nephrotic-range proteinuria. The cellular variant can be diagnosed after excluding collapsing and tip variants, when there is endocapillary hypercellularity involving at least 25% of the tuft of at least one glomerulus, without immune complexes, and typically with extensive foot process effacement (Fogo AB, Lusco MA, Najafian B, Alpers CE. AJKD Atlas of Renal Pathology: Cellular Variant of Focal Segmental Glomerulosclerosis. Am J Kidney Dis. 2015;66(2):e7. [PubMed link]).

In our experience, this variable is difficult to diagnose, since from the histological point of view it is usually similar to a focal endocapillary proliferative glomerulonephritis, however, immunofluorescence is essential to rule out immune complexes or complement deposits (although there may be nonspecific entrapment, in segmental lesions, for IgM and/or C3), in addition, the clinical context is also essential: usually a frank nephrotic syndrome. Also in our experience, although with very few cases, we have seen that the long-term prognosis does not differ much from that of FSGS not otherwise specified (NOS).

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