Table 4.

Conventional Therapy for the Podocytopathies^*

			Reactive Forms
	Idiopathic Forms	Genetic Forms	Others	Medication Associated
MCN	First: daily prednisone Second: CSA or tacrolimus Third: mycophenolate mofetil	PLCE1 mutation: glucorticoids†	Treat underlying disease	Stop medication Consider glucorticoids
FSGS	Nephrotic proteinuria     First: daily prednisone or al-         ternate day prednisone         (fewer data)     Second: CSA/tacrolimus     Third: mycophenolate mofetil Subnephrotic proteinuria     As above or ACEI or ARB	NPHS2 mutation     Avoid glucorticoids     Cautious trial CSA,† or ACEI† or         ARB† Other mutations     ACEI† or ARB† or possibly CSA†     CoQ10† in CoQ2 nephropathy         and Leigh syndrome	Postadaptive FSGS     ACEI or ARB†     Obesity-associated:         weight loss Treat underlying disease	Stop medication Consider glucorcorti-     coids
DMS	ACEI/ARB†	ACEI† or ARB†
CG	First†: daily prednisone or daily     prednisone + CSA Second†: CSA or tacrolimus Third†: mycophenolate mofetil     or sirolimus	CoQ10† in CoQ2 nephropathy	HIV-1 associated     HAART therapy—ACEI         or ARB† Parvovirus B19-associated     IVIG only if immunosup-         pressed†	Stop medication Consider glucorti-     coids†

^*Therapies without symbols are supported by randomized controlled trials (cyclosporine for focal segmental glomerulosclerosis [FSGS] is the only therapy with this level of support) or by nonrandomized controlled trials, uncontrolled trials, or observational studies (all other therapies). MCN indicates minimal change nephropathy; CSA, cyclosporine; ACEI, angiotensin-converting enzyme inhibitor; ARB, angiotensin receptor blocker; DMS, diffuse mesangial sclerosis; CG, collapsing glomerulopathy; HAART, highly active antiretroviral therapy; IVIG, intravenous immunoglobulin; and HIV, human immunodeficiency virus.

^†Therapies that are supported by opinion from one or more expert clinicians.