Nephritic Syndrome Flashcards
IgA nephropathy (Berger’s disease)
most common cause of nephrotic syndrome
abnormal IgA immunoglobulin (antibody) deposits in the kidneys
IgA= breast milk, tears, saliva, GI tract, resp tract, GU tract.
the hinge region of IgA is made up of amino acids. they are glycosylated with galactose and are identified and degraded if too much accumulates. in IgA there is abnormally glycosylated so they are galactose deficient, meaning they are not identified nor degraded by eh body. they then accumulate in the body.
body targets these with IgG (autoantibodies) IgG binds to IgA= immune complexes are foms. this activates alternative complement pathway = immune response= glomerular injury,.
immune complex deposition.
RBC into urine= haematuria
IgA nephropathy clinical features.
presents in childhood
gross/microscopic haematuria
develops during infection of the mucosal lining (resp tract/GI tract) because IgA1 production increases so more for IgG to bind to.
light microscopy: mesangial proliferation.
electron: immune complex in the mesangium
immunofluorescence: involve IgA. scarring of kidneys
(in HSP this is the same but also involves skin, connective tissue, GI and joints)
Mx: BP, corticosteroids,
Crescentic glomerulonephritis
- rapidly progressive
nephritic syndrome
crescent-shaped cell proliferation
idiopathic
type I: anti-GBM antibodies (target the basement membrane) associated with Goodpasture’s syndrome
type II: immune complex-mediated. e.g. post streptococcal, IgA nephropathy, lupus
type III: Pauci-immune (no anti GBM, no immune complex) ANCA in blood
Crescentic glomerulonephritis
- rapidly progressive
nephritic syndrome
crescent-shaped cell proliferation
idiopathic
type I: anti-GBM antibodies (target the basement membrane) associated with Goodpasture’s syndrome
type II: immune complex-mediated. e.g. post streptococcal, IgA nephropathy, lupus
type III: Pauci-immune (no anti GBM, no immune complex) ANCA in blood
cANCA
pANCA
GBM breaks- RBC into the urine, inflammatory mediators and plasma proteins all pass through the bowman space.
low GFR<50%
acute renal failure
light microscopy: crescent shape glomeruli
immunofluorescent:
type I: linear (anti-GBM)
type II: granular
type III: negative on immunofluorescent (pauci immune)
tx: anticoagulant to reduce fibrin, plasmapheresis, immunosuppressant
Consider immunosuppression Cyclophosphamide / steroids Rituximab \+/- Plasma exchange dialysis/kidney transplant
Post streptococcal glomerulonephritis
glomeruli become inflamed after an infection with streptococcal bacteria
*group a beta haemolytic streptocci
streptococcus has an antigen ‘group A’ and produces an enzyme streptolycin which lyse/destroy red blood cells= beta haemolysis (alpha haemolysis= damaged)
this initiates a type III hypersensitivity. IgA and IgM get trapped in the glomerulus (GBM). initiates an inflammatory reaction (C3, oxidants, proteases) larger molecules filter through like RBC and protein.
cola coloured urine
ologuria
more fluid retained in body (peripheral oedema, periorbital oedema)
lumpy bumpy appernace of the glomeruli
typicaly affects children 6 weeks after infection (E.g. impetigo) (1-2 weeks after pharyngitis)
- light microscopy= enlarged and hyper cellular
- electron micorscopy= supepithelial deposits
- immunoflurocence= tarry sky.
1/4 adults can progress into rapidly progressive glomerulonephritis
Goodpasture’s syndrome
autoimmune disease that effects the lungs and kidneys.
inflammation and bleeding in lungs (haemoptysis) and haematuria. no raised ESR
anti GBM test
autoantibodies (IgG) against the alpha-3-chain on the BM. activates the complement system.
risk factors: infection, smoking, oxidative stress, hydrocarbon based solvents.
diagnosis: biopsy of kidney shows inflammation of basement membrane. curvature of glomeruli (crescents) (crescent moon)
immunofluorescence- IgG deposits are seen as linear formation along the BM
tx: aggressive tx with corticosteroids, immunosuppressive, plasmapheresis. plasmapheresis, cyclophosphamide.
Nephritic syndrome
group of symptoms (not a clinical diagnosis)
damage to glomerulus- RBC leak and stick together (dysmorphic acanthocytes) and proteinuria <2.5g. WBC, oligouria, RAAS = HTN
criteria
- haematuria (blood in urine) can be microscopic (not visible) or macroscopic (visile)
- oligouria (signficantly reduced urine output)
- proteinuria (protein in urine <3g/24hr) (anything more is classed as nephROtic) (pROtein)
- fluid retention
- autoimmune
- hereditary
- infectious
types of nephritic syndrome
- Goodpastures (anti-GBM antibody)
- IgA nephropathy (Berger’s)
- Rapidly Progressive Glomerulonephritis
- Post streptococcal glomerulonephritis
ANCA associated vasculitis
also known as Wegners: granulomatosis with polyangitis
ENT (blocked nose, regular epistaxis)
lung (haemoptysis, SOB)
kidney (haematuria, flank/general abdominal pain)
treatment: immunosupressants, cyclophosphamide, prednisolone, rituximab
no immune deposits on immunofluorescence
no immune deposition on immunofluorescence
azathioprine at least 12 months after stable remission
Immune complex glomerulonephritis
immune deposits in the glomerulus which activates a classic complement pathway resulting inflammation
three main types:
1. IgA nephropathy (mesangial and glomerular capillary)
- Post-Streptoccoal glomerulonephritis (sub epithelial humps) (anti streptococcal antibodies in the blood)
- Lupus nephritis (ANCA full house immunofluorescence staining) IgE/IgM/C3/C1Q
nephritic and nephrotic pictures
diagnosed on kidney biopsy with renal
*mesangial expansion
Alport syndrome
hereditary nephritis
abnormality of type IV collagen
associated with deafness
splitting of the basement membrane.
pylonephritis
infection of the upper tract system- kidney and ureter
fever, chills, flank pain, costovertebral angle tenderness.
elderly- confusion, delerium, urinary retention / incontinence
+ve nitrite and leukocytes
glucose (chronic DM) and ketone
Churg syndrome
Eosinophilic granulomatosis with polyangitis
asthma like symptoms
tissue eosinophilia
granulomatosis inflammation
vasculitis
tx: corticosteroids, methotrexate
microscopic polyangiitis: necrotising vasculitis effecting the small and medium vessels
