Glomerulonephritis 2 Flashcards
What are the common types of primary glomerulonephritis ?
- Minial change
- FSGC
- Membranous
- IgA nephropathy
What is the typical presentation of minial change GN?
- Commonest in children
- Presents as nephrotic syndrome
What is the treatment of minimal change glomerulonephritis ?
Because nephrotic syndrome it doesn’t really hypertension and reanl impairment is usually absent so:
- 1st line = oral steroids + treat the nephrotic syndrome
- 2nd line = cyclophosphamide/CSA
What is the classic histological appearance of minimal change GN?
- Normal renal biopsy on LM & IF with foot process fusion on EM.
- So normal everything except foot process fusion on electron microscopy (EM)
Do patients with minimal change progress to end stage renal failure ?
No
What is focal segmental glomerulosclerosis ?
- It is the commonest cause of nephrotic syndrome in adults
- Can be primary or secondary (HIV/Heroin use/Obesity/ Reflux nephropathy) in the cause of it
What is classically seen on renal biopsy for focal segmental glomeruloscleorsis ?
- So light microscopy in FSGS will show damage which is affecting less than 50% of the glomerulus in the kidney and if the glomeruli is affected it will be segmental
- Immunofluorescence will be midly positive and there will be effacement of foot processes on EM
Whereas in minimal change light microscopy wont show anything but on EM you get effacement of foot processes
What is the treatment for focal segmental glomerulosclerosis?
- 1st line = steroids + treat the nephrotic syndrome
- 2nd line = cyclophosphamide
Do patients with focal segmental glomerulosclerosis develop end stage renal failure ?
Yes in 50%
What is membranous nephropathy ?
It is the 2nd most common cause of nephrotic syndrome in adults
Can be primary or secondary due to:
- Infections (hepatitis B/ parasites)
- Connective tissue diseases (lupus)
- Malignancies (carcinomas/ lymphoma)
- Drugs (gold/penicillamine)
Upon diagnosis with renal biopsy what is the characteristic appearance of membranous nephropathy?
- Subepithelial immune complex deposition in the basement membrane – it is a podocyte injury
- Thickened Basement Membranes
What is the antibody associated with membranous nephropathy ?
Anti PLA2r antibody
What is the treatment of membranous nephropathy ?
1st line = Treat nephrotic syndrome + steroid + cyclophosphamide
What is IgA nephropathy?
- It is the commonest cause of GN in the world
- It is where there is increased IgA possibly due to infection which forms immune complexes which deposit in mesangial cells
How does IGA nephropathy typically present ?
- Asymptomatic microhaematuria ± non-nephrotic range proteinuria
- Macroscopic haematuria after resp/GI infection
- Presents as nephritic syndrome
What condition is IgA nephropathy associated with ?
Henoch-Schonlein Purpura (HSP)
How is IgA nephropathy diagnosed and what is characteristically seen?
Renal biopsy - Mesangial cell proliferation and expansion on light microscopy with IgA deposits and C3 in mesangium on IF
What is henoch-schonlein purpura?
This is a systemic variant of IgA nephropathy, causing small vessel vasculitis
What are the typical features of henoch schonlein purpura ?
- Purpuric rash on extensor surfaces (esp legs)
- Abdominal pain
- Nephritis
- Flitting polyarthritis
How is henoch schonlein purpura diagnosed and treated ?
By either skin or renal biopsy - showing IF positive for IgA and C3
What is the treatment of both IgA nephropathy and henoch scholein purpura ?
BP control/ ACE inhibitors & ARBs/ Fish oil
What is rapidly progressive glomerulonephritis ?
- This is the most aggressive glomerulonephritis
- Which results in acute renal failure
What are the causes of RPGN?
ANCA-Positive
Systemic Vasculitis
- GPA (Wegener’s granulomatosis)
- Microscopic polyangiitis
ANCA negative:
- Goodpasture’s disease-Anti-GBM
- Henoch Scholein Purpura HSP/IgA
- Systemic Lupus Erythematosus (SLE)
What is the characteristic feature on biopsy which all the causes of RPGN have ?
Glomerular crescents
What is goodpastures disease?
- This is also known as anti-glomerular basement membrane (GBM) disease
- This is where anti-GBM antibodies are produced against type IV collagen which is found in the alveoli of the lung and GBM
What is the typical presentation of goodpastures disease ?
- Pulmonary haemorrhage
- Haematuria/nephritic syndrome
- AKI - may occur within days of symptoms
What is the treatment of RPGN ?
- High dose Steroids + cyclophosphamide
- Plasmapharesis also given
What do red cell casts on microscopy tell you ?
Indicates a proliferative GN so mesangial or endothelial cells affected
