Table 2.

Taxonomy of the podocytopathies

PodocytopathyIdiopathic FormsGenetic FormsReactive Forms
Minimal-change nephropathyIdiopathic MCNNonsyndromicReactive MCN
        steroid-sensitive        NPHS2        Hodgkin disease
        steroid-resistantSyndromic        Immunogenic stimuli
        DYSF (limb-girdle muscular dystrophy 2B)        Medication-associated: nonsteroidal anti-inflammatory agents, gold, penicillamine, lithium, IFN-α and -β, pamidronate
Focal segmental glomerulosclerosisIdiopathic FSGSNonsyndromicPostadaptive FSGS
        NPHS1 + NPHS2 NPHS2 ACTN4 CD2AP TRPC6 WT1 mtDNA tRNALeu PLCE1
  •         Reduced nephron mass: renal dysplasia, surgical renal mass reduction, reflux nephropathy, chronic interstitial nephritis

  •         Initially normal nephron mass: obesity, increased muscle mass, sickle cell anemia, cyanotic congenital heart disease, hypertensionb

Syndromic WT1 (Frasier) mt DNA tRNALeu (MELAS)Medication-associated FSGS: cyclosporine, tacrolimus, IFN-α, lithium, pamidronate
        PAX2 (renal-coloboma syndrome with oligomeganephronia)
        LMX1B (nail-patella)COQ2 ITGB4 COL4A3, A4, A5 (Alport) GLA (Fabry)
Diffuse mesangial sclerosisIdiopathic DMSNonsyndromic NPHS1 (congenital nephrotic syndrome, Finnish type) WT1 NPHS2 PLCE1
Syndromic LAMB2 (Pierson) WT1 (Denys-Drash)
Collapsing glomerulopathyIdiopathic CGNonsyndromic COQ2Infection-associated CG viruses (HIV-1, parvovirus B19b, CMVb) other (Loa loa filiariasisb, visceral leishmaniasisb, Mycobacterium tuberculosisb)
Syndromic action myoclonus-renal failureDisease associations: Adult Still diseaseb, thrombotic microangiopathy, multiple myelomab
Medication-associated CG: IFN-α, pamidronate
  • aThe primary podocytopathies are organized into four morphologic patterns and three etiologic categories. Named syndromes are shown. ACTN4, α-actinin-4; FSGS, focal segmental glomerulosclerosis; CD2AP, CD2-associated protein; CMV, cytomegalovirus; COL4, type IV collagen; COQ2, co-enzyme Q synthetase 2; DYSF, dysferlin; FSGS, focal segmental glomerulosclerosis; GLA, α-galactosidase A; ITGB4, β4-integrin; LAMB2, laminin β2 chain; LMX1B, Lim homeobox transcription factor 1B; MELAS, mitochondrial myopathy, encephalopathy, lactic acidosis, and stroke-like episodes; NPHS1, nephrin; NPHS2, podocin; PAX2, paired homeobox protein 2; PLCE1, phospholipase Cε1; TRPC6, transient receptor potential cation channel, member 6; WT1, Wilms' tumor-1.

  • b Disease associations for which causation has not been clearly established.