glomerulonephritis Flashcards
definition of glomerulonephritis
immunologically mediated inflammation of renal glomeruli.
what does glomerulonephritis encompass
are caused by pathology in the glomerulus
present with proteinuria, haematuria, or both
are diagnosed on a renal biopsy
cause CKD
can progress to kidney failure - except minimal change disease
spectrum of glomerulonephritis
glomerulonephritides present on a spectrum from nephrosis (proteinuria due to podocyte pathology) to nephritis (haematuria due to inflamm damage)
if glomerulonephritis causes scarring, proteinuria can happen - therefore proteinuria can complete the longer term clinical picture of any glomerulonephritis - even those that are classically nephritic
aetiology of glomerulonephritis
deposition of antigen–antibody immune complexes in the glomeruli that lead to inflammation and activation of complement and coagulation cascades.
immune complexes either form within glomerulus or are deposited in there by circ
ag unknown - occaisionally associated with:
infection
bacterial - Streptococcus viridans, group Ab-haemolytic streptococci, staphylococci, gonococci, Salmonella, syphilis.
viral - Hepatitis B/C, HIV, measles, mumps, EBV, VZV, coxsackie.
protazoal - plasmodium malariae, schistosomiasis, filariasis
inflammatory/systemic disease
- SLE
- systemic vasculitis
- cyroglobinaemia (Hepatitis B/C, HIV, measles, mumps, EBV, VZV, coxsackie.)
drugs - gold, penicillamine
tumours - Classified based on the site of nephron pathology and its distribution.
post-streptococcal glomerulonephritis
after a throat (2wk) or skin (3-6wk) infection
Streptococcal antigen deposits in the glomerulus leading to immune complex formation and infl ammation
minimal change glomerulonephritis
25% of adult nephrotic syndrome
idiopathic/drugs - NSAIDS, Lithium
paraneoplastic - haematological malignancy - usually hodgkin’s lymphoma
doesnt cause renal failure
normal appearance on light microscopy
electron microscopy = loss of epi foot processes
membranous glomerulonephritis
25% of adult nephrotic syndrome
Thickening of GBM from immune complex deposition. Associated with Goodpasture’s syndrome
primary - idiopathic
secondary
- malignancy - lung, breast, GI, prostate, haematological
- infection - hep B/C, strep, malaria, schistosomiasis
- immunological disease - SLE, RA, sarcoidosis, Sjogren’s
- drugs - gold, penicillamine
membraneoproliferative glomerulonephritis (MPGN)
10% of adult nephrotic syndrome (higher in low- and middle-income countries due to infection).
thickening of GBM, mesangial cell proliferation and interposition
Type 1: Subendothelial immune complex deposits and reduplication of GBM.
- immune complexes deposit in skin and activate complement
- underlying cause - infection, cryoglobulinaemia, monoclonal gammopathy, autoimmunity
Type 2: Dense intramembranous deposits (stain only for C3).
- genetic or acquired defect in alternative complement pathway eg C3 nephritic factor
- progressive kidney dysfunction is uncommon
focal segmental glomerulonephrosis
commonest GN seen on biopsy
primary - idiopathic
secondary - HIV, heroin, lithium, lymphoma, any cause of reduced kidney mass/nephrons, kidney scarring
all pts at risk of CKD and kidney failure
focal segmental proliferative glomerulonephritis
Mesangial and endothelial cell proliferation.
Focal refers to involvement of some of the glomeruli,
segmental refers to involvement of parts of individual glomeruli.
Diffuse proliferative glomerulonephritis
Mesangial and endothelial cell proliferation.
affects all glomeruli.
IgA nephropathy
Mesangial cell proliferation and mesangial IgA and C3 deposits.
Henoch–Schönlein purpura (HSP)
small vessel vasculitis and systemic variant of IgA nephropathy with IgA deposition in skin/joints/gut in addition to kidney
Crescentric glomerulonephritis:
Crescent formation by macrophages and epithelial cells,
which fills up Bowman’s space.
Focal segmental necrotizing glomerulonephritis:
Peripheral capillary loop necrosis (e.g. inWegener’s granulomatosis, microscopic polyarteritis and other vasculitides).
Often evolves into crescentric glomerulonephritis.