Glomerulonephritis Flashcards
What is glomerulonephritis?
Inflammation of the glomerulus
Causes 15% of end stage kidney disease
Classified based on kidney biopsy findings
What does diagnosis of glomerulonephritis depend on?
Kidney biopsy
What are the glomerulonephritis features?
Haematuria (visible or non-visible), proteinuria (low grade or nephrotic), hypertension and renal impairment
Describe the nephritic state
Active urine sediment - haematuria, dysmorphic RBCs, and cellular casts
Hypertension
Renal impairment
What is nephrotic syndrome?
Oedema
Nephrotic range proteinuria > 3.5g/day or 350mg/mmol creatinine
Hypoalbuminemia - serum albumin <35g/l
Dyslipidaemia
What are the 2 categories of glomerulonephritis?
Proliferative and non-proliferative
What is proliferative glomerulonephritis?
Excessive numbers of cells in glomeruli which include infiltrating leucocytes
What is non-proliferative glomerulonephritis?
Glomeruli look normal or have areas of scarring
They have normal numbers of cells
What are the pathology descriptions?
Diffuse - more then 50% of glomerulus affected
Focal - less than 50% of glomerulus affected
Global - all glomerulus affected
Segmental - part of glomerulus affected
What are the types of non-proliferative glomerulonephritis?
Minimal change disease
Membranous nephropathy
FSGS
More nephritic than nephrotic
What are the types of proliferative glomerulonephritis?
Mesangio-proliferative GN - IgA nephropathy
Membrano-proliferative GN - lupus nephropathy
Diffuse proliferative GN - post infection GN
Crescentic GN - ANCA associated GN
More nephritic than nephrotic
Describe IgA nephropathy
Commonest cause of GN world wide
Characterised by IgA deposition in the mesangium and mesangial proliferation
Common in 2nd and 3rd decades of life with more males affected
Up to 30% can progress to end stage kidney failure
What is IgA nephropathy presentation?
Microscopic haematuria
Microscopic haematuria and proteinuria
Nephrotic syndrome
IgA crescentic GN
What is the treatment for IgA nephropathy?
Start on ACE inhibitors
Kidney transplant if continued increase in creatinine and decreased eGFR
Describe post-streptococcal GN
Follows 10-21 days after infection typically of throat and skin
Most commonly with Lancefield group A streptococci
Genetic predisposition - HLA-DR and -DP
IgG deposits
What is the treatment for post-streptococcal GN?
Antibiotics for infection
Loop diuretics as frusemide for oedema
Anti-hypertensives
What is included in crescentic GN?
Anti-neutrophil cytoplasmic antibody (ANCA) associated
Anti-glomerular basement membrane
Others - IgA vasculitis, post-infection GN and SLE
What are types of ANCA associated crescentic GN?
Microscopic polyangiitis
Granulomatosis with polyangiitis
Eosinophilic granulomatosis with polyangiitis
Describe anti-GBM disease?
Rare disease caused by circulating anti-GBM
Around 10-20% of crescentic GN
Presents as nephritis and can have lung haemorrhage
Peaks in 3rd and 6/7th decade of life
How is anti-GBM diagnosed?
Demonstrating anti-GBM antibodies is serum and kidney
What is the treatment for anti-GBM?
Aggressive immunosuppression, steroids, plasma exchange and cyclophosphamide
What is a summary of proliferative GN?
Presents with nephritic state - haematuria, variable proteinuria, hypertension and possible renal impairment
Can cause rapid decline in renal function
Early diagnosis to save nephrons - immunology screen and renal biopsy
What are nephrotic syndrome management?
Treat oedema - salt and fluid restriction and loop diuretics
Hypertension treatment
Reduce risk of thrombosis - heparin or warfarin
Reduce risk of infection - pneumococcal vaccine
Treat dyslipidaemia - statins
Specific treatment therapy
Describe minimal change disease
Commonest form in children
Sudden onset of oedema - days
Remission with steroids is often
Relapse occurs in 2/3rd of patients
What is the treatment for minimal change disease?
Prednisolone - 1mg/kg for up to 16 weeks
Once remission achieved then slow taper for 6 months
Initial relapse treated with further steroid course
Subsequent relapses - cyclophosphamide, cyclosporin, tacrolimus, mycophenolate mofetil and rituximab
What is the prognosis of minimal change disease?
Despite relapsing behaviour - prognosis is favourable
Risk of end stage kidney disease is low
Steroid toxicity
Describe focal and segmental glomerulonephritis (FSGS)
Syndrome with multiple causes
Presents with nephrotic syndrome
Pathology reveals focal and segmental sclerosis - tip lesion, collapsing, cellular and perihilar
Primary or secondary
Generally steroid resistant and high chance progression to end stage kidney disease
What is the treatment for FSGS?
General measures
Trail of steroids, positive response, even partial remission and carries better prognosis
Alternative options - cyclosporin, cyclophosphamide and rituximab
May need transplant if advanced
Describe membranous nephropathy
Commonest cause of nephrotic syndrome in adults and majority occur in isolation
Other causes include - malignancies, SLE, rheumatoid arthritis and drugs
What are serological markers of membranous nephropathy?
Anti-phospholipase A2 receptor antibody positive
Thrombospondin type 1 containing 7A
What is the treatment for membranous nephropathy?
General measures for 6 months
Immunosuppression if symptomatic nephrotic syndrome, rising proteinuria or deteriorating renal function
Cyclophosphamide and steroids - 6 months
Tacrolimus
Rituximab
What is the prognosis for membranous nephropathy?
Resolves spontaneously in a 1/3rd
Good in treated patients with proteinuria resolved
About 25% on dialysis after 10 years
Can re-occur in renal transplants
