Renal - Glomerulopathies Flashcards
What is the most common cause of nephrotic syndrome in children?
Minimal change disease
List some of the features of nephritic syndrome?
Haematuria (macro or micro)
Proteinuria
Oliguria
Fluid retention
What is the criteria a patient must fulfil to have nephrotic syndrome?
Peripheral oedema
Proteinuria of >3g in 24 hours
Serum albumin of <25g per litre (hypoalbuminemia)
(Can also present with or have the complication of hypercholesterolemia)
List the different types of glomerulonephritis:
Minimal change disease IgA nephropathy (aka Berger's disease or mesangioproliferative) Goodpasture syndrome Membranous glomerulonephritis Focal segmental glomerulosclerosis Mesangiocapillary glomerulonephritis Rapidly progressive glomerulonephritis Post streptococcal (aka diffuse proliferative)
Define interstitial nephritis:
Inflammation of the space between the tubules (interstitium) and the cells of the kidney.
What are the two different forms of interstitial nephritis:
Acute interstitial nephritis
Chronic Tubulointerstitial nephritis
Which types of glomerulonephritis tend to present with nephritic syndrome symptoms?
IgA
Rapidly progressive
Alport’s syndrome
A male patient presents with some of the following symptoms:
microscopic haematuria and progressive renal failure
bilateral sensorineural deafness
lenticonus, retinitis pigmentosa
What is the likely diagnosis?
Alport’s syndrome
How is Alport’s syndrome diagnosed?
Genetic testing
Renal biopsy:
electron microscopy: characteristic finding is of the longitudinal splitting of the lamina densa of the glomerular basement membrane, resulting in a ‘basket-weave’ appearance
What is the mode of inheritance in Alport’s syndrome?
Autosomal dominant
Alport’s syndrome is caused by a defect in collagen type ? which leads to defective basement menbranes.
4
List the glomerulonephritides which cause nephrotic syndrome:
Focal segmental Membranous Minimal change Diabetic Amyloidosis
List the glomerulonephritides which can cause a mixture of nephritic and nephrotic syndrome?
Post streptococcal
Diffuse proliferative
Membranoproliferative
What is the most common type of glomerulonephritis in adults?
Membranous
At what age does membranous glomerulonephritis peak?
20’s and 60’s
There is a bimodal peak
What is the main symptom associated with membranous GN?
Proteinuria
A lot tend to be asymptomatic
List some of the main causes of membranous GN?
Idiopathic (>70%)
Infection
NSAIDs
Malignancy
What is the main course of management for Membranous GN?
30% will resolve on their own
All should receive either an ACE inhibitor or an ARB
What are the two main principles of management in any glomerulonephritis?
Steroids (immunosuppression)
ACE inhibitors or ARBs to control BP
What can nephrotic syndrome predispose a patient to?
Thromboembolism Hypertension Hypercholesterolemia Increased risk of infections Hypocalcemia
Loss of what substance can lead to the development of thrombus in patients with nephrotic syndrome?
Antithrombin III
Which form of glomerulonephritis is the most common cause of nephrotic syndrome in adults?
Focal segmental glomerulosclerosis
What are the main causes of minimal change disease?
Mainly idiopathic
Others include: Hodgkin's lymphoma Thymoma NSAIDs & rifampicin Infectious mononucleosis
Why are patients with nephrotic syndrome more predisposed to infections?
There is a decrease in serum IgG levels and complement as well as a decreased T cell funciton
What does minimal change disease look like under:
Light Microscopy
Electron microscopy
Light : minimal change (hence name)
Electron: Podocyte foot process effacement
What are the main principles of treatment for nephrotic syndrome?
Reduce oedema
Reduce proteinuria
Reduce risk of complications e.g. anticoagulate
Treat underlying cause
Injury to what cell type in the kidney leads to high loss of protein and why?
Podocytes
They wrap around the glomerular capillaries and maintain the filtration barrier. when damaged there is a high excretion of protein
What changes are seen on histology in membranous glomerulonephritis?
Diffusely thickened glomerular basement membrane with IgG and complement deposits
What antibodies are found in up to 80% of patients with idiopathic membranous glomerulonephriits?
Anti-phospholipase A2 antibodies
Berger’s disease is another name for what?
IgA nephropathy
Which GN is the most common cause of primary glomerular nephropathy worldwide?
IgA nephropathy
What changes will be seen in a patient with IgA nephropathy?
Proliferative changes within glomerular mesangial cell with mesangial deposition of IgA
A male patient presents with macroscopic haematuria following an URTI only a few days ago, what is the most likely diagnosis?
IgA nephropathy (Bergers)
What % of patients with IgA nephropathy will go on to develop end stage renal failure?
25%
A renal biopsy taken from a young male shows the following:
acute, diffuse proliferative glomerulonephritis
endothelial proliferation with neutrophils
electron microscopy: subepithelial ‘humps’ caused by lumpy immune complex deposits
immunofluorescence: granular or ‘starry sky’ appearance. What is the likely diagnosis?
Post streptococcal nephropathy
What is another name for post streptococcal glomerulonephritis?
Diffuse proliferative glomerulonephritis
What are some of the common features of a post streptococcal GN?
Usually under 30 y/o
Presenting 1-3 weeks post URT infection (tonsillitis)
Nephritic syndrome symptoms
What levels will be low in post streptococcal GN?
Complement
What immune complexes are deposited in the glomeruli in a post streptococcal GN?
Complement (C3)
IgG
IgM
What mode of inheritance is seen in Alport’s syndrome?
X linked (dominant / recessive)
What is the difference between a glomerulonephritis and a glomerulopathy?
Glomerulonephritis - inflammation of the glomeruli can be seen
Glomerulopathy - no evidence of inflammation can be seen
How should minimal change GN be managed?
High dose corticosteroid e.g. prednisalone for 4-6 weeks IF patient has significant proteinuria. Can be withheld for spontaneous remission
Up to 2/3rds will require further courses due to relapsing or non remittance
How does focal segmental glomerulosclerosis present typically?
Massive proteinuria
Renal impairment
Hypertension
Haematuria
Where is the kidney is typically affected first by focal segmental glomerulosclerosis?
The deep glomeruli of the corticomedullary junction
WHat is seen on light microscopy for focal segmental glomerulosclerosis?
Segmental sclerosis is seen first. Then progresses to global sclerosis
Hyalinosis also seen
What deposits will be seen on immunofluorescence in focal segmental glomerulosclerosis?
C3 and IgM
What are the main causes of focal segmental glomerulosclerosis?
Idiopathic Secondary to other renal pathology e.g. IgA nephropathy, minimal change disease HIV Heroin abuse Sickle cell Alport's
How is FSGS treated?
<10% will go through spontaneous remission
Prednisolone (6 months)
+/- immunosuppressant e.g. ciclosporin
Define azotaemia?
This is a collection of biochemical abnormalities including a raised blood urea, nitrogen and creatinine
often just referred to as uraemia
In diabetic nephropathy the kidneys shrivel and get smaller. True or false?
False
They bilaterally enlarge and initially will have hyperfiltration
What histological changes can be seen from early on in diabetic nephropathy?
Basement membrane thickening and mesangial expansion
What is the most common cause of glomerular pathology and CKD in the UK?
Diabetic Nephropathy
What causes the scarring in diabetic nephropathy?
The high concentration of glucose passing through the kidneys
How are diabetics screened for diabetic nephropathy?
Albumin:creatinine ratio
Us and Es for kidney function
What medication is first line for hypertension management in diabetic patients?
ACE inhibitors (or ARBs if not well tolerated)
What form of glomerulonephritis is rarer, but has a significantly poor prognosis and is sometimes associated with hep C?
Membranoproliferative GN
A patient presents with pulmonary haemorrhage (haemoptysis) and acute glomerulonephritis. What is the likely diagnosis?
Goodpasture’s disease a.k.a anti-glomerular basement membrane GN
Anti-GBM / Goodpasture’s disease is a form of _______ glomerulonephritis?
Rapidly progressive
How is Goodpasture’s disease managed?
Plasma exchange
Steroids
Cyclophosphamide
Histology from a renal biopsy shows crescentic glomerulonephritis in an acutely unwell patient. What is the diagnosis?
Rapidly progressive GN
What causes the crescentic shape in rapidly progressive GN?
An aggregate of macrophages and epithelial cells in the Bowman’s space
