Glomerulonephritidies

Question 1

What are the general features of nephritic syndrome?

Answer 1

Haematuria
Hypertension

Question 2

What are the general features of nephrotic syndrome?

Answer 2

Oedema
Proteinuria

Question 3

What classically causes a nephritic picture?

Answer 3

Rapidly progressive glomerulonephritis
IgA nephropathy

Question 4

What causes a mixed picture?

Answer 4

Diffuse proliferative glomerulonephritis
Membranoproliferative glomerulonephritis
Post-streptococcal glomerulonephritis

Question 5

What classically causes a neurotic picture?

Answer 5

Minimal change disease
Membranous glomerulonephritis
Focal segmental glomerulosclerosis
Amyloid
Diabetic nephropathy

Question 6

What is rapidly progressive glomerulonephritis?

Answer 6

Rapid loss of renal function associated with formation of epithelial crescents in majority of glomeruli

Question 7

What are causes ion rapidly progressive glomerulonephritis?

Answer 7

Goodpasture’s syndrome
Granulomatosis with polyangitis
SLE
Microscopic polyarteritis

Question 8

What are the features of rapidly progressive GN?

Answer 8

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

Question 9

What is IgA nephropathy?

Answer 9

Mesangial deposition of IgA immune complexes

Question 10

What is the classic presentation of IgA nephropathy?

Answer 10

Classically macroscopic haematuria in young man 1-2 days after URTI

Question 11

What is IgA nephropathy associated with?

Answer 11

Henoch-Schonlein purpura

Question 12

What is seen on histology with IgA nephropathy?

Answer 12

mesangial hypercellularity, positive immunoifluorescence for IgA and C3

Question 13

What is the management of IgA nephropathy?

Answer 13

1st line: ACE inhibitors
2nd line: corticosteroids

Question 14

What are indications for management of IgA nephropathy?

Answer 14

Persistent proteinuria (>500-1000) with normal or slightly reduced GFR –> ACEi
Active disease or failure to respond to ACEi –> steroids

Question 15

What are markers of poor prognosis in IgA nephropathy?

Answer 15

Male, heavy proteinuria >2g, hypertension, smoking, hyperlipidaemia, ACE genotype DD

Question 16

What are the common causes of diffuse proliferative glomerulonephritis?

Answer 16

Post streptococcal GN
Most common renal disease in SLE

Question 17

What causes post strep glomerulonephritis?

Answer 17

Immune complex (IgG, IgM, C3) deposition in glomeruli

Question 18

What normally provokes post strep GN?

Answer 18

Group A strep, usually strep pyogenes

Question 19

What is the classic presentation of post strep glomerulonephritis?

Answer 19

Young children 7-14 days post URTI. Proteinuria and low complement

Question 20

What are the features of post strep glomerulonephritis?

Answer 20

Headache, malaise
Visible haematuria
Proteinuria
HTN
Oliguria

Question 21

What is seen on bloods in post strep GN?

Answer 21

Low C3
Raised anti-streptolysin O titre

Question 22

What is seen on biopsy with post strep GN?

Answer 22

Acute diffuse proliferative GN
Endothelial proliferation with neutrophils

Question 23

What is seen on electron microscopy with post strep GN?

Answer 23

Subepithelial humps caused by lumpy immune complex deposition

Question 24

What is seen on immunofluorescence with post strep GN?

Answer 24

Granular or starry sky appearance

Question 25

What are the types of membranoproliferazive glomerulonephritis and causes?

Answer 25

type 1 - cryoglobulinaemia, hep C type 2 - "dense deposit disease" - partial lipodystrophy, factor H deficiency type 3 - hep B and C

Question 26

What is the most common type of membranoproliferazive GN?

Answer 26

Type 1 - cryoglobulinaemia, hep C

Question 27

How does membranoproliferazive glomerulonephritis present?

Answer 27

Can be nephrotic syndrome or haematuria

Question 28

What is seen on renal biopsy with type 1 membranoproliferazive GN?

Answer 28

Tram track appearance - subendothelial and mesangium immune deposits of electron dense material

Question 29

What is the pathophysiology of type 2 membranoproliferative GN?

Answer 29

Persistent activation of alternative complement pathway leads to intramembranous immune complex deposits with dense deposits

Question 30

What is seen on bloods in type 2 membranoproliferative GN?

Answer 30

Low circulating C3 C3b nephritic factor - antibody to alternative pathway C3 convertase

Question 31

How is membranoproliferative GN managed and what is prognosis?

Answer 31

Steroids may be helpful Poor prognosis

Question 32

What are the causes of m animal change disease?

Answer 32

Idiopathic in 80-90% NSAIDs, rifampicin Hodgkin's, thymoma Infectious mononucleosis

Question 33

What is the pathophysiology of minimal change disease?

Answer 33

T cell and cytokine mediated damage to glomerular basement membrane --> polyanion loss --> reduction in electrostatic charge --> increased glomerular permeability to serum albumin

Question 34

What are the features of minimal change disease?

Answer 34

Almost always presents as nephrotic syndrome - hypertension very rare Selective proteinuria - only intermediate sized protein eg albumin and transferrin leak through glomerulus

Question 35

What is seen on renal biopsy with minimal change disease?

Answer 35

Light microscopy: Normal glomeruli Electron microscopy: fusion of podocytes and effacement of foot processes

Question 36

Who is minimal change disease normally seen in and how common is it?

Answer 36

Accounts for 75% of nephrotic syndrome in children and 25% in adults

Question 37

What is the management of minimal change disease?

Answer 37

1. Oral corticosteroids - 80% steroidi responsive 2. Cyclophosphamide if resistant

Question 38

What is the prognosis of minimal chan ge disease?

Answer 38

Overall good 1/3 single episode, 1/3 infrequent relapse, 1/3 frequent relapses which stop before adulthood

Question 39

How prevalent is membranous glomerulonephritis?

Answer 39

Most common type of GN in adults and 3rd most common cause of ESRF

Question 40

What are the causes of membranous glomerulonephritis?

Answer 40

Idiopathic - anti-phospholipase A2 antibodies Infection - hep B, malaria, syphilis Malignancy - prostate, lung, lymphoma, leukaemia Drugs - NSAIDs, penicillamine Autoimmune - SLE, rheumatoid, thyroid

Question 41

What is the management of membranous glomerulonephritis?

Answer 41

All: ACEi or ARB Severe/progressive: corticosteroid + cyclophosphamide Consider anticoagulation

Question 42

What is seen on biopsy with membranous glomerulonephritis?

Answer 42

"Spike and dome" Basement membrane thickened with sub epithelial electron dense deposits

Question 43

What is the prognosis of membranous glomerulonephritis?

Answer 43

Rule of thirds - 1/3: spontaneous remission - 1/3 remain proteinuria - 1/3 develop ESRF

Question 44

Who is focal segmental glomerulosclerosis normally seen in?

Answer 44

Young adults

Question 45

What are the causes of focal segmental glomerulosclerosis?

Answer 45

Idiopathic Secondary to other renal pathology - eg IgA neohropathy HIV, heroin Alpert's syndrome Sickle cell

Question 46

What is seen on renal biopsy with focal segmental glomerulosclerosis?

Answer 46

Focal and segmental sclerosis and hyalinosis Effacement of foot processes

Question 47

What is the management of focal segmental glomerulosclerosis?

Answer 47

Steroids +/- immunosuppression

Question 48

What is the prognosis of focal segmental glomerulosclerosis?

Answer 48

<10% chance spontaneous remission if untreated High relapse rate post transplant