Glomerulonephritis

Question 1

Clinical features of IgA nephropathy

Answer 1

Usually young male
Recurrent episodes of macroscopic haematuria
Typically associated with mucosal infections
Rarely has nephrotic range proteinuria

Question 2

Diagnostic features of IgA nephropathy

Answer 2

Mesangial hypercellularity in histology
Positive immunofluorescence for IgA and C3

Question 3

Clinical features of post-strep glomerulonephritis

Answer 3

URTI precedes GN - typically an interval of 1-6 weeks
Nephritis syndrome
- haematuria (tea, cola-coloured urine)
- hypertension
- oedema
Associated with LOW C3 and normal C4 levels levels

Question 4

GN that can recur

Answer 4

IgA nephropathy (most common)
FSGC (most rapid)
Membranoproliferative GN (Mesangiocapillary)
Membranous GN

Question 5

Features of lupus nephritis

Answer 5

Proteinuria
Haematuria
Hypertension
Oedema
Reduced kidney function
Renal colic or flank pain
Fever and fatigue

Question 6

What GN is associated with low C3 levels

Answer 6

Poststreptococcal GN
Lupus nephritis
Membranoproliferative GN

Question 7

Histological features of proliferative lupus nephritis

Answer 7

Subendothelial immune deposits/wire loops
Hypercellularity
Leukocyte infiltration
Fibrinoid necrosis
Hyaline thrombi
Crescents in severe LN

Question 8

Treatment of lupus nephritis

Answer 8

Immunosuppressants - steroids, cyclophosphamide, MMF, azathioprine
Biologics - rituximab, belimumab
ACE inhibitors
NSAIDs
Anticoagulants
Dietary and lifestyle changes
Dialysis or kidney transplant

Question 9

Principle of treatment in lupus nephritis

Answer 9

Induction - prednisone, cyclophosphamide, MMF, rituximab (refractory cases)
Maintenance - MMF or azathioprine for at least 2 years post remission

Question 10

Feature of pauci-immune rapidly progressive GN

Answer 10

Severe form of kidney disease
Characterised by inflammation of glomeruli and rapidly declining kidney function
Associated with autoimmune disorders

Question 11

Treatment of pauci-immune RPGN

Answer 11

Immunosuppressive therapy - steroids, cyclophosphamide, rituximab
Plasmapheresis
Maintenance therapy
Supportive Care
Kidney transplant

Question 12

Features of membranoproliferative GN

Answer 12

Nephrotic syndrome
Significant proteinuria
Haematuria with dysmorphic red cells and red cell casts**

Question 13

Histology features of FSGS

Answer 13

Podocyte detachment leading to segmental sclerosis ***
Collapsing lesions
Hyalinosis
Adhesions

Question 14

Clinical features of FSGS

Answer 14

Characterised by sclerosis affecting only some segments of glomeruli
Nephrotic syndrome

Question 15

Poor prognostic markers of IgAN

Answer 15

Elevated serum Cr
HTN
Proteinuria > 1g/day

Question 16

Clinical features of MN

Answer 16

Proteinuria (nephrotic range or sub-nephrotic range)
Microscopic haematuria
Normal or impaired renal function
HTN
Nephrotic syndrome **
Hyperlipidaemia *
Thromboembolic complications*

Question 17

Secondary causes for MN

Answer 17

Lupus nephritis
Hepatitis B and C
Malignancy (lung, colon, stomach and kidney)
Medications (NSAIDs, gold, penicillamine)

Question 18

Biopsy results for MN

Answer 18

LM - immune complex deposition in basement membrane “spike and hold”
Granular staining for IgG

EM - electron dense deposits in GBM in subepithelial regions

Question 19

Antibodies present in MN

Answer 19

Anti-PLA2R (70-80% of primary cases), correlates with activity
THSD7a antibodies (in 3-5% of pts who were anti-PLA2R negative)

Question 20

GN most likely to rapidly recur

Answer 20

FSGS
Characterised by massive proteinuria within hours to days after implantation of new kidney

Question 21

Features of Alport’s syndrome

Answer 21

Progressive renal failure, hearing loss ** and ocular abnormalities **
Associated with corneal opacities, anterior lenticonus **

Question 22

Genetic mutation of Alport’s

Answer 22

COL4A5 (X-linked) or COL4A3 and COL4A4 (AR)
Results in absence of collagen IV a3a4a5 network

Question 23

Clinical features of TBMN

Answer 23

Persistent microhaematuria (most common cause of persistent haematuria)
NO proteinuria **
Normal renal function and BP
AD inheritance of haematuria **

Question 24

Histopathology features of TBMN

Answer 24

Uniform thinning of GBM

Question 25

Genetic mutation in TBMN

Answer 25

HETEROzygous COL4A3 or COL4A4 mutations

Question 26

Histological features of type 1 MGPN

Answer 26

Global and diffuse thickening of capillary walls Caused by mesangial interposition into subendothelial zone of capillary loops - "train track formation"

Question 27

Types of MGPN

Answer 27

Type 1 - immune mediated Type 2 - complement mediated, dense deposit disease

Question 28

Indications for renal biopsy in patients with suspected diabetic nephropathy

Answer 28

Rapid onset of proteinuria Absence of retinopathy Presence of haematuria Active urinary sedment Rapid decrease of renal function Suspicion of other nephropathies secondary to systemic disease

Question 29

Clinical features of Goodpasture's syndrome/anti-GBM

Answer 29

Pulmonary haemorrhage and rapidly progressive GN with renal failure

Question 30

Histopathology features of anti-GBM

Answer 30

Crescent formation, uniform age IM - strong linear ribbon-like appearance of IgG

Question 31

Possible pathogenesis of hypercoagulable state in nephrotic syndrome

Answer 31

Decreased levels of antithrombin and plasminogen (due to urinary losses), increased platelet activation, hyperfibrinogenaemia, inhibition of plasminogen activation and presence of high molecular weight fibrinogen in circulation

Question 32

Causes of nephrotic syndrome

Answer 32

Idiopathic membranous nephropathy Membranoproliferative GN Minimal change disease

Question 33

Most common causes of secondary MCD

Answer 33

Medications (NSAIDs, selective COX-2 inhibitors) Malignancy Infection Allergy

Question 34

Features of collapsing glomerulopathy

Answer 34

Segmental and global collapse of glomerular capillaries Marked hypertrophy Hyperplasia of podocytes Most commonly seen in association with HIV Also associated with TB, CMV, hepatitis B and hepatitis C

Question 35

Features of Chinese herbal nephropathy

Answer 35

Mild low-molecular-weight proteinuria Hypertension Severe anaemia Development of uroepithelial atypia

Question 36

Histopathological features of CHN

Answer 36

Unusual extensive, virtually hypocellular cortical interstitial fibrosis Associated with tubular atrophy and global sclerosis of glomeruli

Question 37

Histopathology findings on MCD

Answer 37

Minimal changes Effacement of podocyte foot processes

Question 38

Management of MCD

Answer 38

Prednisone - very responsive

Question 39

Histology features of diabetic nephropathy

Answer 39

Thickening of GBM Eosinophilic nodular glomerulosclerosis (Kimmelsteil-Wilson nodules) Mesangial matrix expansion

Question 40

Histopathology scoring system for IgA nephropathy

Answer 40

MEST Mesangial hypercellularity Endocapillary hypercellularity Segmental glomerulosclerosis Tubullar atrophy/interstitial fibrosis

Question 41

Causes of FSGS

Answer 41

Can be idiopathic Heroin HIV infection Sickle cell disease Obesity Interferon treatment Congenital malformations i.e. CMT NPHS1 and NPHS2 mutations