Primary = idiopathic (majority) Secondary - infections - drugs - associated malignancies - part of systemic disease: ANCA +ve, vasculititis SLE, GPA, HSP

Glomerulonephritis Flashcards by Ellie McMichael

what is glomerulonephritis?

immune-mediated diseases of the kidney affecting the glomeruli (characteristically by inflammation)
usually affecting both kidneys
usually occurs several weeks after an infection (secondary to tubulointerstitial damage)

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what part of the immune system is involved in glomerulonephritis?

humoral - antibody
- intrinsic/ planted antigen
- deposition of circulating immune complexes
T-cell mediated
inflammatory cells, mediators and complements

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who gets it?

Primary = idiopathic (majority)

Secondary

infections
drugs
associated malignancies
part of systemic disease: ANCA +ve, vasculititis SLE, GPA, HSP

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How are glomerulonephritis’s classified?

By aetiology - primary/secondary 
histology 
- proliferative/non-proliferative
- focal (<50%)/ diffuse (>50%)
- global (all of glomeruli)/segmental (part of glomeruli)
- cresentic - RPGN

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how does glomerulonephritis present?

haematuria - painless 
proteinuria 
impaired renal function 
hypertension 
nephrotic syndrome 
nephritic syndrome

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what is haematuria in glomerulonephritis?

Aysymptomatic microscopic haematuria

Episodes of painless macroscopic haematuria
urine microscopy
- dysmorphic RBCs (squished)
- red cell cast (=endothelial injury)

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how is it diagnosed?

Clinical presentation 
Blood test (ANA, ANCA, anti-GBM testing)
Kidney biopsy (1:400 risk of bleed)
Examination of urine 
- urinalysis - proteinuria/ haematuria
- Urine microscopy - RBC (dysmorphic), RBC casts, lipiduria 
-Urine protein - creatinine ratio/24hr

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histological findings of biopsy?

light microscopy
- hypercellularity - inflammatory cells and reactive proliferations
- sclerosis
- crescents - this is bad
electron microscopy (see where the deposits are, can look at BM)
- subepithelial
- mesangial
- subendothelial
immunofluorescence (what antibody and distribution - IgM, IgA, IgG)
- IgG - Goodpasture’s

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what is the aim of glomerulonephritis treatment?

decrease proteinuria
remission of nephrotic syndrome
longterm renal function

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what are the immunosuppressive treatments?

corticosteroids (prednisolone PO, methylpredisolone IV)
azathioprine
alkylating agents (cyclophosphamide, chlorambucil)
calcineurin inhibitors
mycophenolate mofotil
plasmapheresis
antibodies - IV immunoglobulin, monoclonal T/B antibodies
Goodpastures - cyclophophamide (azathioprine/ MMF maintenance)

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what are the non-immunosuppressive treatments?

anti-HTN (target<130/80 if proteinuria <120/75)
ACEi/ARBs 
diuretics 
statins 
anticoagulants/aspirin/antiplatelets 
dialysis 
transplant

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how to treat nephrotic patients?

Aim is sustained remission - complete proteinuria <300mg/day and partial proteinuria <3g/day
fluid restriction 
salt restriction 
diuretics 
ACEi/ARBs
anticoagulation?
IV albumin - if V deplete 
immunosuppression

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what is minimal change nephropathy?

normal renal biopsy on light microscopy

foot process fusion on electron microscopy (podocytes)

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who gets minimal change nephropathy?

commonest cause of nephrotic syndrome in children

possibly caused by IL-3

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what 3 factors are highly suggestive of minimal change neuropathy?

response to steroids/immunosuppressives
occurs with Hodgkin’s lymphoma with remission following successful treatment
patients and family have increased incidence of asthma and eczema (remission of the nephrotic syndrome following antigen avoidance)

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how is minimal change nephropathy treated?

oral steroids - 94% complete remission

if steroid resistant/dependent/multiple relapses - cyclophosphamide/CSA

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what is membranous nephropathy?

sub epithelial immune complex deposition in the basement membrane - the basement is thicker and the electron dense deposits create a ‘spike and dome’ appearance

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what is the pathogenesis in membranous nephropathy?

anti-PLA2r antibody id seen in 70%, PLA2r is an antigen on podocytes
Thickened BM - silver staining

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who gets membranous nephropathy?

2nd most common cause of nephrotic syndrome in adults
primary - idiopathic
secondary
- associated with infections (Hep B, parasites)
- connective tissue diseases (SLE)
- malignancy (carcinoma, lymphoma)
- drugs (gold, penicillin)

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what is the prognosis of membranous nephropathy?

30% develop ESRT after 10 years

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how is membranous nephropathy managed?

steroids
alkylating agents
B cell monoclonal antibody

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What is focal segmental glomerulosclerosis?

focal <50% of the tissue 
segmental part of the glomeruli 
focal segmental GS on LM 
minimal Ig/complement deposition
because segmental can be missed on biopsys

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who gets focal segmental glomerulosclerosis?

commonest cause of nephrotic syndrome in adults 
primary - idiopathic 
secondary
- HIV
- heroin 
- obesity 
- reflux

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what is the prognosis of focal segmental glomerulosclerosis?

50% develop ESRF after 10 years

what is the management of focal segmental glomerulosclerosis?

prolonged steroids | remission in 60%

how does it present?

massive proteinuria haematuria hypertension renal impairment

What is membranoproliferative nephropathy?

big lobulated hypercellular glomeruli with thick membranes - tram tracks (duplication of BM)

who gets membranoproliferative nephropathy?

idiopathic type 2 - infection, lupus, malignancy adults and children

how does membranoproliferative nephropathy present?

nephritic/nephrotic syndrome - can be either

what is the prognosis of membranoproliferative nephropathy?

is dependent on the severity

what is IgA nephropathy?

mesiangial cell proliferation and expansion on LM and IgA deposits in the mesangium on immunofluorescence - follows a resp/GI infection by a couple of days

who gets IgA nephropathy?

``` commonest glomerulonephritis associated with HSP - arthritis - colitis - purpuric rash ```

how does IgA nephropathy present?

asymptomatic microhaematuria +/- non-nephrotic proteinuria macroscopic haematuria after a couple of days resp/GI infection (haematuria a couple of WEEKS after an infection is post-infective nephropathy) AKI/CKD

what is the prognosis of IgA nephropathy?

25% develop ESRF after 10 yrs

how is IgA nephropathy managed?

BP control ACEi/ARB fish oil

what is rapidly progressive glomerulonephritis?

a treatable cause of AKI | it has a systemic association

how does rapidly progressive glomerulonephritis present?

treatable cause of acute renal failure rapid deterioration in renal function over days/weeks active urinary sediment - RBC, RBC and granular cast part of systemic disease glomerular crest on biopsy

who gets rapidly progressive glomerulonephritis?

``` ANCA+ve - GPA (cANCA, PR3) - MPA (pANCA, MPO) - wegners granulomatosis - Churg-Strauss syndromes ANCA -ve - Goodpasture's (antiGBM, IgG) - HSP/IgA, - SLE ```

how is rapidly progressive glomerulonephritis managed?

systemic and renal management is needed immunosuppression - steroids: PO prednisolone, IV methylprednisolone - cytotoxics: cyclophosphamide, mycophenolate, azathioprine supportive treatment (dialysis) plasmapheresis

what is diabetic nephropathy?

kidneys initially enlarge and there is hyperfiltration (GFR >150mL/min)

pathology in diabetic nephropathy?

diffuse and nodular glomerulosclerosis Kimmel Stiel Wilson lesion - nodules thickening of glomerular basement membrane and mesangial expansion also microvascular disease - arterial sclerosis

how is diabetic nephropathy managed?

lifestyle advice | ACEi/ARB regardless of BP

linear IgG glomerularnephritis?

Goodpasture's Syndrome

difference between glomerulonephritis and glomerulopathy?

if there is predominant inflammation on histology then glomerular disease can be glomerulonephritis if inflammation is absent - an awful lot of overlap and they are sometimes used interchangeably but this isn't correct

what cells are more involved in nephrotic syndrome?

pOdocytes - proteinuria - oedema - hyperlipidaemia

what cells are more involved in nephritic?

endothelial and mesangial cells - haematuria - hypertension

What is nephritis?

Generic term that means inflammatory of kidneys, not a diagnosis or syndrome with criteria

What is nephritic syndrome/acute nephritis syndrome?

Haematuria Oliguria Proteinuria Fluid retention

What is oliguria?

Significantly reduced urine output

What is nephrotic syndrome?

Refers to group of symptoms without specifying underlying cause -Not a disease, a way of explaining symptoms Criteria: - Peripheral oedema - Proteinuria - Serum albumin - Hypercholesterolaemia

Types of glomerular nephritis?

- Minimal change disease - Focal segmental glomerulosclerosis - Membranous glomerulonephritis - IgA nephropathy - Post-streptococcal glomerulonephritis - Mesangiocapillary glomerulonephritis - Rapidly progressive glomerulonephritis - Good pasture syndrome

What is IgA nephropathy AKA?

Mesangioproliferative glomerulonephritis

What are most types of glomerulonephritis treated with?

Immunosuppression (roids) | BP control with ACEis/ARBs

Presentation of nephrotic syndrome?

Usually oedema Possible frothy urine Predisposes patients to thrombosis, HT, and high cholesterol

Most common cause of primary glomerulonephritis?

IgA nephropathy

Most common type of glomerulonephritis overall?

Membranous glomerulonephritis

what are the nephrotic syndrome triad?

proteinuria >3g/day hypoabuminaemia <30g oedema (hypercholesterolaemia)

what syndrome is often seen with normal renal function?

``` nephrotic syndrome complications - infection - RV thrombosis - PE ```

what are the signs and symptoms of nephritic syndrome?

``` AKI oliguria oedema/fluid retention - not to the degree of nephrotic hypertension active urinary sediment - RBC - granular cast - proteinuria ```