Glomerulonephritis Flashcards
Other than autoimmune what can cause glomerulonephritis? [3]
Infection
Malignancy
Drugs
What are the two major syndromes associated with glomerulonephritis?
Nephritic Syndrome - Proliferative glomerulonephritis
Nephrotic Syndrome - Non-proliferative Glomerulonephritis
What are the major types of Glomerulonephritis?
Nephritic [4]
Nephrotic [3]
Proliferative/Nephritic:
- Mesangioproliferative GN eg IgA nephropathy
- Diffuse proliferative GN eg Post-streptococcal GN
- Focal Necrotising Crescentic Nephritis
- Membrano-proliferative nephritis
Non-proliferative/Nephrotic:
- Minimal change disease
- Focal & Segmental GN
- Membranous Nephropathy
How do we distinguish nephrotic from nephritic syndrome?
Presentation - name 4 clinical features each
Describe the underlying pathology of each briefly [4]
Nephritic syndrome involves haematuria, proteinuria, hypertension and a low urine volume due to renal impairment. Immune complex deposition cause inflammatory reaction damaging capillary walls.
Nephrotic syndrome involves hypoalbuminaemia, proteinuria, oedema and hyperlipidaemia. Derangement in capillary walls of glomeruli allowing increased permeability to plasma proteins
What is the distinguishing feature of Nephritic syndrome? [1]
Red cell casts in the urine.
Explain the presentation of nephrotic syndrome [5]
- Leaky glomeruli cause protein loss (frothy urine) (3.5g/day)
- Hypoalbuminemia causes
- anasacra because of low colloidal pressure (not enough albumin in blood to osmotically keep water from moving into the Extra-vascular compartment)
- Hyperlipidaemia because liver over produces albumin to compensate and produces more lipids as a side effect
- Also raises risk of thrombosis and infection
What do diffuse, focal, global and segmental mean in terms of glomerulonephritis? [4]
Focal affects <50% of the glomeruli
Global affects 100%
Diffuse affects >50%
Segmental only affects specific parts of the glomerulus
Out of the 4 types of nephritic syndrome which 2 are diffuse and focal?
Post-infective Nephritis (diffuse) IgA nephropathy (Focal)
What causes post-infective nephritis? [2]
Genetic association [1]
Management [1]
Mostly group A streptococci, it occurs 10-20 days post- skin/throat infection
Associated with genetic predisposition (HLA-DR or HLA-DP)
Mx: supportive only
General management for nephritic syndrome [3]
Bed rest, salt restriction, careful fluid monitoring
Mx of AKI
BP control: ACEi or ARB with aim for <130/80 (or <125/75 if significant proteinuria)
What is the commenest cause of glomerulonephritis worldwide?
IgA nephropathy.
Its most common in teens/20s & men
How does Focal Necrotising Crescentic Nephritis (FNCN) look? [2]
A crescent of cells & Debris fills the Bowman’s Space on biopsy.
It causes really high creatinine (~1200)
What are the classes of FNCN? [3]
1) ANCA associated including wegener’s & microscopic polyangiitis
2) Anti-GBM (Glomerular Basement Membrane) incl anti-GBM nephritis and goodpasture’s syndrome
3) Others e.g. IgA vasculitis, post infective or SLE
How do you tell apart Anti-GBM vs ANCA positive FNCN [3]
ANCA presents with AKI + systemic features (weight loss, myalgia, fever)
Anti-GBM antibodies can be found in the serum & kidney. Quick onset AKI
Both feature lung haemorrhage (hemoptysis)
FNCN Ix [1]
Mx [5]
Ix: renal biopsy
With aggressive immunosuppression:
- High dose steroids
- Cyclophosphamide
- Plasma Exchange
B Cell Therapy - Rituximab
Complement inhibitors
In Summary:
Cases in powerpoint
There are two categories of glomerulonephritis:
Proliferative & Non-proliferative
They are associated with nephritic (haematuria, hypertension & renal impairment) and nephrotic syndrome (proteinuria, oedema, hypoalbuminae & hyperlipidaemia) respectively.
There are a number of causes within each category but mostly they are autoimmune in origin (barring post-infective and membranous which can be secondary to malignant, drugs, R arthritis & SLE).
Treatment revolves around solving the problems/risks associated with nephritic/nephrotic syndrome and steroids/immunosuppression with mainly cyclophosphamide.
IgA glomerulonephritis
Define [2]
Classical presentation [2]
Dx [2]
IgA immune complexes deposit in mesangium causing irritation, stimulates matrix and mesangium formation
It classically presents as macroscopic haematuria in young people following an upper respiratory tract infection.
Biopsy: mesangial proliferation with IgA and C3 deposits on immunofluorescence (IF)
Prognosis for IgA glomerulonephritis [2]
Association with HSP [3]
Management
Mostly self limiting
Some can progress to chronic renal failure
Henoch Schondlein Purpura - systemic syndrome whereas IgA is just kidneys (purpuric rash, GI, joints)
Mx: BP control only
How severe is IgA nephropathy?
Can be very minor with just microscopic haematuria
Or very severe with IgA crescentic GN leading to kidney failure within weeks
How does diabetic glomerular disease occur? [2]
What is a Kimmelsteil-Wilson lesion? [1]
Glycalated molecules deposited in the basal lamina and mesangial matrix [1] lead to a thick leaky basement membrane and compressed capillaries [1]
A nodule of mesangial matrix formed during Diabetic glomerular disease
Pathology of Crescentic Glomerulonephritis? [2] (FNCN)
A number of conditions cause:
cellular proliferation and influx of macrophages, forming a crescent within the bowman’s space that crushes the glomerulus
What can cause crescentic glomerulonephritis? [3]
- Wegener’s Granulomatosis
- Microscopic polyarteritis
- Antiglomerular basement membrane disease
What is Wegener’s granulomatosis? [1]
A form of vasculitis in kidneys, nose and lungs.
How do you test for and treat Wegener’s granulomatosis? [1]
Serum ANCA (Anti-neutrophil cytoplasmic antibodies) test.
Treat with cyclophosphamide, untreated mean survival is 6 months
What are the 3 parts of the glomerular membrane?
Fenestrated endothelium
Basal La`mina
Foot processes of Podocytes
Nephritic syndrome investigations [5]
Urine:
o Urinalysis: haematuria, mild proteinuria
o Urine MC&S: renal tubular cells, red cell casts, RBCs, WBCs
o 24h urine collection: assessment of proteinuria
Bloods: FBC (anaemia, leucocytosis) and film, U&E and creatinine,
Immunology: anti-dsDNA and ANA (SLE), cANCA (GPA), pANCA (MPA), anti-GBM, ASO
Renal USS
Renal biopsy
