Minimal change disease classification

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Clinical Classification

The clinical classification of minimal change disease is based on the underlying etiology of the disease.

Primary

In primary (idiopathic) cases, the underlying cause is not known.

Secondary

Secondary forms of minimal change disease are associated with certain environmental exposures, such as allergies (bee sting), malignancies (lymphomas and leukemias), medications (NSAID, penicillamine, ampicillin), and other toxins (gold, mercury)

Pathological Classification

Minimal change disease currently has no classification system. Early observations noted that a small number of patients with minimal change disease have focal tip lesions. Based on the proposed Columbia classification by D’Agati and colleagues in 2004, minimal change disease was considered an entity within the spectrum of the focal segmental glomerulonephritis (FSGS) and may have a clinical course similar to those with “tip lesion” subtype of FSGS.

Pathological Classification of Focal Segmental Glomerulosclerosis

Variant	Location of Lesion	Distribution of Lesion	Characteristic Features
Not Otherwise Specified (NOS)	Anywhere	Segmental	Capillary lumen abolished by the segmental increase in matrix.
Perihilar Variant	Perihilar	Segmental	Presence of one or more glomeruli containing hyalinosis in the perihilar regions with or without sclerosis. Within each glomerulus, the segmental lesions must contain > 50% perihilar hyalinosis and/or sclerosis.
Cellular Variant	Anywhere	Segmental	Presence of one or more glomerulus with segmental hypercellularity of the capillary endothelium that blocks the capillary lumen, with or without foam cells and/or karryohexis.
Tip Variant	At tip domain	Segmental	One or more segmental lesions, that include tip domains. Lesions must have adhesions/confluence of podocytes with parietal or tubular cells. Tip domains are defined as 25% of tuft adjacent to the origin of the proximal tubule. Sclerosing lesions shuld be <25% of tuft, while cellular lesions should be < 50% of tuft. No perihilar sclerosis should be observed.
Collapsing Variant	Anywhere	Segmental or global	One or more glomeruli with collapse with evidence of podocyte hypertrophy and hyperplasia.

^{Adapted from D’Agati VD, Fogo AB, Bruijn JA, and Jennette JC. Pathological classification of focal segmental glomerulosclerosis. A working proposal. Am J of Kidney Dis. 2004; 43(2):368-382.}

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