Glomerulonephritidies Flashcards
What are the general features of nephritic syndrome?
Haematuria
Hypertension
What are the general features of nephrotic syndrome?
Oedema
Proteinuria
What classically causes a nephritic picture?
Rapidly progressive glomerulonephritis
IgA nephropathy
What causes a mixed picture?
Diffuse proliferative glomerulonephritis
Membranoproliferative glomerulonephritis
Post-streptococcal glomerulonephritis
What classically causes a neurotic picture?
Minimal change disease
Membranous glomerulonephritis
Focal segmental glomerulosclerosis
Amyloid
Diabetic nephropathy
What is rapidly progressive glomerulonephritis?
Rapid loss of renal function associated with formation of epithelial crescents in majority of glomeruli
What are causes ion rapidly progressive glomerulonephritis?
Goodpasture’s syndrome
Granulomatosis with polyangitis
SLE
Microscopic polyarteritis
What are the features of rapidly progressive GN?
Nephritic syndrome - haematuria with red cell casts, hypertension, proteinuria, oliguria
Features specific to underlying cause - eg haemoptysis with Goodpasture’s, vasculitis rash if gramulomatosis with polyangitis
Often presents as AKI
What is IgA nephropathy?
Mesangial deposition of IgA immune complexes
What is the classic presentation of IgA nephropathy?
Classically macroscopic haematuria in young man 1-2 days after URTI
What is IgA nephropathy associated with?
Henoch-Schonlein purpura
What is seen on histology with IgA nephropathy?
mesangial hypercellularity, positive immunoifluorescence for IgA and C3
What is the management of IgA nephropathy?
1st line: ACE inhibitors
2nd line: corticosteroids
What are indications for management of IgA nephropathy?
Persistent proteinuria (>500-1000) with normal or slightly reduced GFR –> ACEi
Active disease or failure to respond to ACEi –> steroids
What are markers of poor prognosis in IgA nephropathy?
Male, heavy proteinuria >2g, hypertension, smoking, hyperlipidaemia, ACE genotype DD
What are the common causes of diffuse proliferative glomerulonephritis?
Post streptococcal GN
Most common renal disease in SLE
What causes post strep glomerulonephritis?
Immune complex (IgG, IgM, C3) deposition in glomeruli
What normally provokes post strep GN?
Group A strep, usually strep pyogenes
What is the classic presentation of post strep glomerulonephritis?
Young children 7-14 days post URTI. Proteinuria and low complement
What are the features of post strep glomerulonephritis?
Headache, malaise
Visible haematuria
Proteinuria
HTN
Oliguria
What is seen on bloods in post strep GN?
Low C3
Raised anti-streptolysin O titre
What is seen on biopsy with post strep GN?
Acute diffuse proliferative GN
Endothelial proliferation with neutrophils
What is seen on electron microscopy with post strep GN?
Subepithelial humps caused by lumpy immune complex deposition
What is seen on immunofluorescence with post strep GN?
Granular or starry sky appearance
What are the types of membranoproliferazive glomerulonephritis and causes?
type 1 - cryoglobulinaemia, hep C
type 2 - “dense deposit disease” - partial lipodystrophy, factor H deficiency
type 3 - hep B and C
What is the most common type of membranoproliferazive GN?
Type 1 - cryoglobulinaemia, hep C
How does membranoproliferazive glomerulonephritis present?
Can be nephrotic syndrome or haematuria
What is seen on renal biopsy with type 1 membranoproliferazive GN?
Tram track appearance - subendothelial and mesangium immune deposits of electron dense material
What is the pathophysiology of type 2 membranoproliferative GN?
Persistent activation of alternative complement pathway leads to intramembranous immune complex deposits with dense deposits
What is seen on bloods in type 2 membranoproliferative GN?
Low circulating C3
C3b nephritic factor - antibody to alternative pathway C3 convertase
How is membranoproliferative GN managed and what is prognosis?
Steroids may be helpful
Poor prognosis
What are the causes of m animal change disease?
Idiopathic in 80-90%
NSAIDs, rifampicin
Hodgkin’s, thymoma
Infectious mononucleosis
What is the pathophysiology of minimal change disease?
T cell and cytokine mediated damage to glomerular basement membrane –> polyanion loss –> reduction in electrostatic charge –> increased glomerular permeability to serum albumin
What are the features of minimal change disease?
Almost always presents as nephrotic syndrome - hypertension very rare
Selective proteinuria - only intermediate sized protein eg albumin and transferrin leak through glomerulus
What is seen on renal biopsy with minimal change disease?
Light microscopy: Normal glomeruli
Electron microscopy: fusion of podocytes and effacement of foot processes
Who is minimal change disease normally seen in and how common is it?
Accounts for 75% of nephrotic syndrome in children and 25% in adults
What is the management of minimal change disease?
- Oral corticosteroids - 80% steroidi responsive
- Cyclophosphamide if resistant
What is the prognosis of minimal chan ge disease?
Overall good
1/3 single episode, 1/3 infrequent relapse, 1/3 frequent relapses which stop before adulthood
How prevalent is membranous glomerulonephritis?
Most common type of GN in adults and 3rd most common cause of ESRF
What are the causes of membranous glomerulonephritis?
Idiopathic - anti-phospholipase A2 antibodies
Infection - hep B, malaria, syphilis
Malignancy - prostate, lung, lymphoma, leukaemia
Drugs - NSAIDs, penicillamine
Autoimmune - SLE, rheumatoid, thyroid
What is the management of membranous glomerulonephritis?
All: ACEi or ARB
Severe/progressive: corticosteroid + cyclophosphamide
Consider anticoagulation
What is seen on biopsy with membranous glomerulonephritis?
“Spike and dome”
Basement membrane thickened with sub epithelial electron dense deposits
What is the prognosis of membranous glomerulonephritis?
Rule of thirds
- 1/3: spontaneous remission
- 1/3 remain proteinuria
- 1/3 develop ESRF
Who is focal segmental glomerulosclerosis normally seen in?
Young adults
What are the causes of focal segmental glomerulosclerosis?
Idiopathic
Secondary to other renal pathology - eg IgA neohropathy
HIV, heroin
Alpert’s syndrome
Sickle cell
What is seen on renal biopsy with focal segmental glomerulosclerosis?
Focal and segmental sclerosis and hyalinosis
Effacement of foot processes
What is the management of focal segmental glomerulosclerosis?
Steroids +/- immunosuppression
What is the prognosis of focal segmental glomerulosclerosis?
<10% chance spontaneous remission if untreated
High relapse rate post transplant
