Glomerulonephritis

Question 1

What is nephrotic syndrome?

Answer 1

Triad of:
1. Proteinuria (>3g/24h)
2. Hypoalbuminaemia (<30g/L)
3. Oedema
Other: hypercholesterolaemia, hypercoagulopathy,

Question 2

What are the basic/non basic investigations for suspected glomerulonephritis?

Answer 2

Basic - U+Es, Urinalysis, quantify proteinuria, check albumin and ultrasound.
Non basic - GN screen, myeloma screen and kidney biopsy

Question 3

What is seen in a GN screen?

Answer 3

ANCA,
ANA,
C3/4,
Anti-GBM,
Rh factor

Question 4

What are some causes of pure nephrotic syndrome?

Answer 4

Minimal change disease,
Membranous GN,
Focal segmental GN,
Amyloidosis,
Diabetic nephropathy

Question 5

What can a kidney biopsy show?

Answer 5

Light microscopy,
Immunofluorescence,
Electron microscopy (looks at ultrastructure)
It is the gold standard for diagnosis of GN

Question 6

What are features of Minimal Change disease?

Answer 6

Most commonly occurs in children.
T cell and cytokine mediated damage to glomerular BM
Presentation: Nephrotic syndrome, normotensive
Biopsy: Normal glomeruli on LM. Electron microscopy shows fusion of podocytes.

Question 7

What is the management of minimal change disease?

Answer 7

1st line: Oral corticosteroids
2nd line if resistant to steroids: Cyclophosphamide

Question 8

Describe the presentation and causes of membranous glomerulonephritis

Answer 8

Causes: Idiopathic: Anti-phospholipase A2 antibodies, infection (hep B, malaria or syphilis), malignancy, drugs (penicillamine, NSAIDs), autoimmune diseases.
Presents with nephrotic syndrome or proteinuria. Biopsy shows thickened basement membrane with spike and dome appearence.

Question 9

What is the management of membranous glomerulonephritis

Answer 9

All patient’s given an ACEi or ARB.
Most resolve spontaneously and don’t need immunosupression but if needed then give steroids AND cyclophosphamide as steroids alone are not effective.

Question 10

What are the complications of nephrotic syndrome?

Answer 10

Increased risk of thrombosis due to loss of antithrombin III and plasminogen in urine,
Hyperlipiaemia,
CKD,
Increased risk of infection,
Hypocalcaemia (vitamin D lost in urine)

Question 11

What is membranoproliferative GN?

Answer 11

GN more likely to cause nephritic syndrome but can overlap. Can be caused by Hep C. Typically affects patients under age 30.

Question 12

Describe features and investigations of anti-GBM disease

Answer 12

Caused by anti GBM antibodies. Presents with Haemoptysis, pulmonary haemorrhage, AKI rapidly progressive.
Investigations: Unrinalysis, urine microscopy and culture, U&Es, CXR, GN screen, and renal biopsy

Question 13

What is the treatment of goodpasture’s syndrome?

Answer 13

Plasma exchange,
Steroids and cyclophosphamide.

Question 14

What is nephritic syndrome?

Answer 14

Syndrome with: Haematuria (microscopic or macroscopic), oliguria, hypertension, proteinuria (<3g in 24h)

Question 15

What disease can cause nephritic syndrome?

Answer 15

SHARP AIM
SLE
HSP,
Anti-GBM disease,
RPGN,
Post-infectious GN,
Alport syndrome,
IgA nephropathy,
Membranoproliferative glomerulonephritis

Question 16

WHat are the investigations for nephritic syndrome

Answer 16

Urine dipstick,
Urine microscopy (looking for casts),
Urine PCR,
FBC, U&Es, LFTs, bone profile,
GN screen,
Ultrasound of kidneys,
Kidney biopsy

Question 17

Describe features of IgA nephropathy

Answer 17

Caused by excess IgA which deposits in the mesangium. On biopsy there is hypercellularity.
Presents 1-2 days after URTI with nephritic syndrome

Question 18

What is the management of IgA nephropathy?

Answer 18

1. If only isolated haemturia, protienuria < 500-1000mg/day and normal eGFR then no treatment needed.
2. Persistent proteinuria (>500-1000mg/day) or slightly reduced eGFR then ACEi.
3. Falling eGFR or failure to respond to ACEi then steroids

Question 19

What are the causes of rapidly progressive glomerulonephritis?

Answer 19

Type 1 - Anti-GBM antibody disease,
Type 2 - Immune complex deposition eg, lupus nephritis, IgA nephropathy, HSP
Type 3 - Pauci immune disease (absence of anti GMB antibodies or immune complexes), ANCA vasculitis

Question 20

What are the signs and symptoms of rapidly progressive glomerulonephritis?

Answer 20

Nephritic syndrome, peripheral oedema, fatigue, loss of appetitie, nausea and vomiting, persistent hiccups

Question 21

What are the investigations for RPGN?

Answer 21

ANCA,
Anti-GBM,
Renal biopsy

Question 22

What is the general management of nephrotic syndrome?

Answer 22

Furosemide with care,
ACE inhibitor/ARB (not in minimal change),
Statin (not in minimal change),
SGLT2 (not in vasculitis)

Question 23

What are features of amyloidosis?

Answer 23

Deposition of abnormal proteins.
AA amyloidosis -(occurs when increased serum amyloid A) malignancy or chronic microbial infection.
AL amyloidosis (L=light chain) - myeloma or waldenstrom’s macroglobulinaemia.
It can present with nephrotic syndrome, neuropathy, periorbital purpura, malabsorption, joint inflammation

Question 24

What is the investigation for amyloidosis?

Answer 24

Congo red staining shows apple green bifringence.

Question 25

What are the causes of membranoproliferative GN?

Answer 25

Type 1 - cryoglobinaemia, hepatitis C. Tram track appearance of immune deposits
Type 2 - Partial lipodystrophy, factor H deficiency. Results in persistant activation of complement pathway so low levels of c3.
Type 3 - Hepatitis B and C

Question 26

What is cryoglobinaemia?

Answer 26

Abnormal blood proteins call cryoglobulins clump together in cold temperature causing reduced blood flow. Presents with purpura, arthralgia and weakness