Glomerular Disease (Clinical) Flashcards
What is glomerulonephritis?
Acute inflammation of the kidney, typically caused by an immune response
What are glomerular diseases?
Immune-mediated disorders that affect the glomeruli
What are glomerular diseases classified by?
Based on kidney biopsy findings
What are the 2 different classifications of glomerular diseases?
- Proliferative
- IgA nephropathy
- Post infectious glomerulonephritis
- Crescentic glomerulonephritis
- Anti-GBM disease
- Non-proliferative
- Minimal change disease
- Focal and segmental glomerulosclerosis
- Membranous nephropathy
What are examples of proliferative glomerular diseases?
- IgA nephropathy
- Post infectious glomerulonephritis
- Crescentic glomerulonephritis
- Anti-GBM disease
What are some examples of non-proliferative glomerular diseases?
- Minimal change disease
- Focal and segmental glomerulosclerosis
- Membranous nephropathy
What are the 2 kinds of glomerulonephritis syndromes?
Nephritic syndrome
Nephrotic syndrome
What are some features of glomerulonephritis?
- Haematuria (non visible or visible)
- Proteinuria (low grade or nephrotic)
- Hypertension
- Renal impairment
What is observed if glomerulonephritis is in a nephritic state?
- Active urine sediment
- Haematuria
- Dysmorphic RBCs
- Cellular casts
- Hypertension
- Renal impairment
What is observed if glomerulonephritis is in a nephrotic state?
- Body passes too much protein in urine
- Oedema
- Nephrotic range proteinuria
- >3.5g/day or 350mg/mmol creatinine
- Hypoalbuminaemia
- Serum albumin <35g/L
What is the range of proteinuria required to be considered a nephrotic syndrome?
- >3.5g/day or 350mg/mmol creatinine
What is the difference between nephritic and nephrotic syndromes?
Difference between nephritic and nephrotic is that nephrotic syndrome involves the loss of a lot of protein whereas nephritic syndrome involves the loss of a lot of blood
What is proliferative glomerulonephritis defined as?
Excessive numnbers of cells in glomeruli, these include infiltrating leucocytes
What is non-proliferative glomerulonephritis defined as?
Glomeruli look normal or have areas of scarring
They have normal numbers of cells
What are the pathological descriptions of glomerulonephritis depending on how much of the glomeruli is affected?
- Diffuse
- >50% of glomeruli affected
- Focal
- <50% of glomeruli affected
- Global
- All of the glomerulus affected
- Segmental
- Part of the glomerulus affected
What is the term used to describe >50% of the glomeruli is affected?
Diffuse
What is the term used to describe <50% of the glomeruli is affected?
Focal
What is the term used to describe all of the glomeruli is affected?
Global
What is the term used to describe part of the glomeruli is affected?
Segmental
Are non-proliferative diseases normally nephrotic or nephritic?
Nephrotic
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Are proliferative diseases normally nephrotic or nephritic?
Nephritic
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What is the commonest cause of glomerulonephritis?
IgA nephropathy
What is IgA nephropathy characterised by?
IgA deposition in th emesangium and mesangial proliferation
Who does IgA nephropathy most commonly occur in?
Most commonly occurs in 2nd and 3rd decade of life with males more commonly affected
Does IgA nephropathy affect more men or woman?
Men
What is the presentation of IgA nephropathy?
- Microscopic haematuria
- Microscopic haematuria and proteinuria
- Nephrotic syndrome
- IgA crescentic glomerulonephritis
What investigations are done for IgA nephropathy?
- Urine dip
- Blood pressure
- Ultrasound scan
- Renal biopsy
- eGFR
What is the treatment for IgA nephropathy?
- Dialysis
- Renal transplant
What is post infectious glomerulonephritis most commonly caused by?
Most commonly caused by Lancefield group A streptococci infection after infection typically of throat or skin
Is there a genetic predisposition to post infectious glomerulonephritis?
Yes
HLA-DR, -DP
What is the presentation of post infectious glomerulonephritis?
- Malaise
- Dark urine
- Puffy face
- No rashes or oedema
- Sore throat previously
What investigations are done for post infectious glomerulonephritis?
- Bloods
- Urine dip
What is the treatment of post infectious glomerulonephritis?
- Antibiotics for infection
- Loop diuretics such as frusemide for oedema
- Anti-hypertensives such as vasodilator drugs
What are the different kinds of crescentic glomeronephritis?
Anti-neutrophil cytoplasmic antobody (ANCA) associated
Anti-glomerular basement membrane (GBM)
Others (IgA vasculitis, post infection glomerulonephritis, lupus nephritis)
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What is lupus nephritis caused by?
What is the presentation of crescentic glomerulonephritis?
- Fatigue
- Weight loss
- Rash
- Bilateral basal crepitations
What is crescentic glomerulonephritis characterised by?
Characterised by the presence of extensive glomerular crescents (usually greater than 50%) as the principal histologic finding
What investigations are done for crescentic nephritis?
- Urine dipstick
- High protein and high blood
- Bloods
- High serum creatinine
- Renal biopsy
What is observed in urine dipsticks for crescentic glomerulonephritis?
High protein and high blood
What is observed in the bloods for crescentic glomerulonephritis?
High serum creatinine
What is anti-GBM disease?
Rare disease caused by circulating anti-GBM, is a type of crescentic glomerulonephritis
What does anti-GBM disease present as?
- Nephritis (anti-GMB glomerulonephritis)
- Nephritis and lung haemorrhage
In what age groups is anti-GBM disease most common?
Peaks in 3rd and 6th/7th decade of life
How is anti-GBM disease diagnosed?
Diagnosed by demonstrating anti-GBM antibodies in serum and kidney
What is the treatment for anti-GBM disease?
- Aggressive immunosuppression
- Steroids
- Plasma exchange
- Cyclophosphamide
Do proliferative glomerulonephritis’s present with nephrotic or nephritic state?
Presents with nephritic stage:
- Haematuria and variable proteinuria and hypertension with or without renal impairment
Why is early diagnosis important in proliferative glomerulonephritis’s?
Early diagnosis necessary to save nephrons:
- Clinical suspicion, immunological screen and renal biopsy
What are the pathological patterns of non-proliferative glomerulonephritis?
Focal and segmental glomerulonephritis
What is the management for non-proliferative glomerulonephritis?
- General measures
- Treat oedema
- Salt and fluid restriction and loop diuretics
- Hypertension
- RAAS blockade
- Reduce risk of thrombosis
- Heparin or warfarin
- Treat dyslipidaemia
- Statins
- Treat oedema
- Specific therapy
- Towards cause of non-proliferative glomerulonephritis
What is the commonest form of non-proliferative glomerulonephritis in children?
Minimal change disease
What is the presentation of minimal change disease?
Sudden onset of oedema
How often does relapse occur in minimal change disease?
2/3 of patients
What is the treatment for minimal change disease?
- Prednisolone
- 1mg/kg for up to 16 weeks
- Once remission achieved, slow taper over 6 months
- Initial relapse treated with further steroid course
- Subsequent relapses treated with
- Cyclophosphamide
- Cyclosporin
- Tacrolimus
- Mycophenolate mofetil
- Rituximab
What is the prognosis of minimal change diease?
- Despite relapsing behaviour, prognosis is favourable
- Risk of end stage kidney disease is low
- Steroids toxicity as multiple exposure
What is focal and segmental glomerulosclerosis (FSGS)?
Disease in which scar tissue develops on the parts of the kidneys that filter waste from the blood (glomeruli)
Is not a single disease, rather a syndrome with multiple causes
What pathological pattern does FSGS display?
Pathology reveals focal and segmental sclerosis with distinctive patterns, such as:
- Tip lesion, collapsing, cellular, perihilar and not other specified
What does FSGS stand for?
Focal and segmental glomerulosclerosis
Wnat is the prognosis of FSGS?
High change of progression to end stage kidney disease
What is the treatment for FSGS?
- General measures as previously described
- Trail of steroids
- Alternative options
- Cyclosporin
- Cyclophosphamide
- Rituximab
What are the different kinds of FSGS?
Primary or secondary
What is the commonest cause of nephrotic syndrome in adults?
Membranous nephropathy
What are some serological markers for membranous nephropathy?
- Anti-phospholipase A2 receptor (PLA2R) antibody positive in 70% of idiopathic cases
- Thrombospondin type 1 domain containing 7A
What are some secondary causes of membranous nephropathy?
- Malignancies
- SLE
- Rheumatoid arthritis
- Drugs
- NSAIDs, gold, penicillamine
What is the treatment of membranous nephropathy?
- General measures for at least 6 months
- Immunosuppression if symptomatic nephrotic syndrome, rising proteinuria or deteriorating renal function
- Cyclophosphamide and steroids for 6 months
- Tacrolimus
- Rituximab
What is the prognosis of membranous nephropathy?
- Resolves spontaneously in 1/3
- Prognosis good in treated patients whose proteinuria resolves
- About 25% are on dialysis in 10 years
- Can recur in renal transplants
Does non-proliferative glomerulonephritis present with nephrotic or nephritic syndrome?
Nephrotic syndrome
What does precise diagnoisis of the kind of glomerular disease depend on?
Renal biopsy which can influence management
What does the prognosis of glomerular disease depend on?
Type of glomerulonephritis and the response to treatment