Glomerulonephritis: Types, Associations And Causes

Glomerulonephritis: Tipe (Jenis), Hubungan dan Penyebab

	Histology	Immune deposits	Pathogenesis	Association	Clinical features
Minimal change	Normal, except on electron microscopy, where fusion of podocyte foot processes is seen (occurs in many types of proteinuria)	None	Unknown	Atopy, HLA-DR7 Drugs	Acute and often severe nephrotic syndrome Good response to corticosteroids Dominant cause of idiopathic nephrotic syndrome in childhood
Focal segmental glomerulosclerosis (FSGS)	Segmental scars in some glomeruli No acute inflammation Podocyte foot process fusion seen in primary FSGS with nephrotic syndrome	Non-specific trapping in focal scars	Unknown; in some, circulating factors increase glomerular permeability Injury to podocytes may be a common feature	Healing of previous localglomerular injury HIV infection, heroin misuse, morbid obesity	Primary FSGS presents as idiopathic nephrotic syndrome but is less responsive to treatment than minimal change; may progress to renal impairment, can recur after transplantation Secondary FSGS presents with variable proteinuria and outcome
Focal segmental (necrotising) glomerulonephritis	Segmental inflammation and/or necrosis in some glomeruli May be crescent formation	Variable according to cause, but typically negative (or 'pauci-immune')	Small-vessel vasculitis	Primary or secondary small-vessel vasculitis	Usually implies presence of systemic disease, and responds to treatment with corticosteroids and cytotoxic agents Check ANCA, ANA
Membranous nephropathy	Thickening of GBM Progressing to increased matrix deposition and glomerulosclerosis	Granular subepithelial IgG	Antibodies to a podocyte surface antigen, with complement-dependent podocyte injury (presumed from animal model)	HLA-DR3 (for idiopathic) Drugs Heavy metals Hepatitis B virus Malignancy	Usually idiopathic; common cause of adult idiopathic nephrotic syndrome One-third progress; may respond to chlorambucil/prednisolone Associated HLA class II allele varies in different populations
IgA nephropathy	Increased mesangial matrix and cells Focal segmental nephritis in acute disease	Mesangial IgA	Unknown	Usually idiopathic Liver disease	Very common disease with range of presentations, but usually including haematuria and hypertension (see text)
Mesangiocapillary glomerulonephritis (MCGN) (= membranoproliferative glomerulonephritis, MPGN)
Type I	Mesangial cells interpose between endothelium and GBM	Subendothelial	Deposition of circulating immune complexes or 'planted' antigens	Bacterial infection Hepatitis B virus Cryoglobulin-aemia (± hepatitis C virus infection)	Usually proteinuria, may be haematuria Most common pattern found in association with subacute bacterial infection No proven treatments except where cause can be treated
Type II	Mesangial cells interpose between endothelium and GBM	Intramembranous dense deposits	Associated withcomplement consumption caused by autoantibodies	C3 nephritic factor and partial lipodystrophy	Also known as dense deposit disease
Post-infection	Diffuse (uniformly in all glomeruli) proliferation of endothelial and mesangial cells Infiltration by neutrophils and macrophages May be crescent formation	Subendothelial	Immune response to streptococcal infection Cross-reactive epitopes or other explanation	Streptococcal and other infections	Now rare in developed countries Presents with severe sodium and fluid retention, hypertension, haematuria, oliguria Usually resolves spontaneously
Goodpasture's disease (anti-GBM disease)	Usually crescentic nephritis	Linear IgG along GBM	Autoimmunity to α3 chain of type IV collagen	HLA-DR15 (previously known as DR2)	Associated with lung haemorrhage but either may occur alone Treat with corticosteroids, cyclophosphamide and plasma exchange to remove circulating autoantibodies
Lupus nephritis	Almost any histological type	Always positive and often profuse Pattern varies according to type	Some anti-DNA antibodies also bind to glomerular targets	Complement deficiencies Complement consumption	Very variable presentation, sometimes as renal disease alone without systemic features Responds to cytotoxic therapy in addition to prednisolone

Referensi:
Davidson's Principles and Practice of Medicine 20th Edition (Churchill Livingstone) 2007

Tags: Lecture Notes