Week 4 - I(1) - Glomerulonephritis types Flashcards by Chris Egubjo

What are the main types of glomerulonephritis? - they get their name from their morphological appearance

Minimal change disease

Focal Segmental Glomerulosclerosis

Membranous

Membranoproliferative

Rapid progressive glomerulonephritis

IgA nephropathy

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What will effacement of the podocytes cause?

This will cause minimal change disease - associated with the loss of the size and charge selective barrier

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In minimal change, unsure of what the circulating antibody is

Will this cause nephrotic or nephritic?

this causes nephrotic syndrome

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Because minimal change disease is minimal, what can you see it on?

Cannot see it on light microscopy or immunoflorurescence but can see the change on electron microscopy

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What is the commonest cause of nephrotic syndrome in children? What is the commonest cause of nephrotic syndrome in adults?

Children - minimal change disease - 77%

Adults - focal segemental glomerular sclerosis

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What is the 1st and 2nd line treatment for minimal change disease?

1st line - steroids oral

2nd line - cyclophosphamide or ciclsporin (CSA)

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Does minimal change disease cause progressive renal failure?

NO It can be associated with Il-13

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Commonest cause of nephrotic syndrome in adults (35%) ? What may it be due to?

FSGS

Can be due to HIV, heroin, Obesity, reflux nephropathy

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What does the renal biopsy of FSGS on light microscopy show?

It shows as the name suggests - focal segmental glomerulasclerosis

Can see the FSGS on the right glomerulus

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What percentage of patients achieve remission on steroids? What percentage progress to ESRD over 10years?

60% patinets achieve remission with prolonged steroids

50% progress to ESRD after 10 years

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What is the second most common cause of nephrotic syndrom in adults?

Membranous nephropathy

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What are the important secondary causes of membranous nephropathy?

Hep B, Lupus, Gold, penicillamine. Also carcinomas

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What is seen on renal biopsy in membranous glomerulonephritis?

Renal biopsy: diffusely thickened basemement membrane due to subepithelial immune complex deposition (creates a spike and dome apperance)

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What is given as the treatment of membranous nephropathy? (same as the others)

Steroids + cyclophasphamide

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What is the antibody that can be tested for in membranous nephropathy?

Test for anti-phospholipase A2 antibody - it is present in more than 70% of patients

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The antigen is within the podocyte – call phospholipase A2 receptor (PLA2r) The antibody then attacks this in membranous nephropathy Again state the antibody and the important secondary causes?

Antibody - anti - PLA2 (anti-phospholipase A2) receptor antibody Hepatitis B, lupus, gol & penicillamine (and malignancies)

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Because in membranous nephropathy, there is the deposition of the complexes in the glomerular membrane, what does it look like on light microscopy?

Thickened Basement Membranes - Silver Stain

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What is the commonest glomerulonephritis in the world?

IgA nephropathy

Asymptomatic microhaematuria +/-non-nephrotic range proteinuria

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What typically comes pre IgA nephropathy?

Resp or GI infection typically - usually causes macroscopic haematuria

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What disease is IgA nephropathy associated with?

Associated with Henoch Schnolein Purpuura

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What does IgA nephropathy show on renal biopsy?

On renal biopsy - Mesangial cell proliferation and expansion on light microscopy with IgA deposits in mesangium on IF

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What percentage of people with IgA nephropathy progress to renal failure in 10-30 years?

25%

In IgA nephropathy, it is different to the other glomerulonephrtiis as the immune complexes form in the circulation Where do the immune complexes then deposit?

They deposit in the mesangial cells of he kidney

Does IgA nephropathy lead to nephrotic or nephritic syndrome?

Leads to nephritic syndrome

What are some of the secondary causes of IgA nephroapthy?

HSP upper GI infection URTI / post-strep / sore throat

What is the difference in timing between IgA nephropathy vs post-infectious glomerulonephritis?

IgA characteristically causes visible haematuria **a few days** after URTI Post-infectious GN has lag time of around **2 weeks** before haematuria occurs and would be a less benign presentation if associated with visible haematuria).

A treatable cause of acute renal failure Rapid deterioration in renal function over days/weeks Active urinary sediment (RBC’s, RBC & Granular Casts) May be part of systemic disease. Associated with glomerular crescents on biopsy. What is this?

This is rapidly progressive glomerulonephritis

What is RPGN associated with on biopsy? What disease do you think?

Associated with glomerular crescents on biopsy Either think ANCA positive or ANCA negative vasculitis

Name 3 ANCA positive and 3 ANCA negative causing conditions of RPGN?

ANCA positvie - GPA, EGPA, MPA ANCA negative - Goodpastures, SLE, HSP

Treatment should be prompt and consist of strong immunosuppression with supportive care including dialysis if needed What is the treatment given in RPGN?

Steroids (IV Methylprednisolone / Oral Prednisolone) Cytotoxics (Cyclophosphamide/ Mycophenolate/ Azathioprine

74 year old woman. Hypoxic. Haemoptysis. creat 430. blood and protein on dip. Red cell casts on microscopy. Purpuric rash. Cells affected? Diagnosis? What test next?

Cells affected - endothelial cells Diagnosis - RPGN - Goodpastures or ANCA +ve vasculitis Next test - test for anti-GBM and ANCA

QUIZ STUFF First and second most common cause of nephrotic syndrome in adults? Most common cause in children? Most common glomerulonephritis in the world and what it cause?

Nephrotic syndrome in adults: Most common - focal segemntal glomerulosclerosis Second - membranous Nephrotic in children - minimal change disease Most common GN in the world is IgA and it causes nephritic syndrome

What is minimal change disease caused by? What is the treatment? What imaging is required?

Caused by effacement (fusion) of podocytes Requires 1. Steroids 2. cyclophosphamid or ciclosporin (CSA) second line Requires electron microscoopy as cant be seen on LM or IF

What type of drug is cyclophosphamide and what type is cyclosporin?

Cyclophasmphamide is an alkylating agent - cytotoxic Ciclosporin is a calcineurin inhibtor

FSGS is the commonest cause of nephrotic in adults What percentage go onto renal failure? What percentage achieve remission on steroids?

50% are on ESRD after 10 years 60% achieve remission

What are secondary causes of FSGS?

HIV, Heroin, Obesity, Reflux nephropathy

What is seen on biopsy in FSGS?

 Focal segmental glomerulosclerosis on light microscopy with minimal Ig/complement deposition on immunofluorescence

o Second commonest cause of nephrotic syndrome in adults (15-30%) What are some secondary causes?

This is mebranous nephropathy Lupus, Hepatitis B, Gold and penicillamine Maliganncies in patinets over 65

Membranous is treated with steroids/alylating agents/monoclonal antibody What are examples of alylating agents? What percentage are at ESRD after 10 years?

Alkylating agents - cyclophosphamide, tacrolimus 30% are on ESRD after 10 years

• IgA nephropathy (nephritic) o Commonest glomerulonephritis in the world o Asymptomatic microhaematuria. IgA deposits causes immune complexes and deposits in the mesangial cells What is the treatment? What is seen on renal biopsy? What is prognosis?

Treatment - control blood pressure Renal biopsy - mesagial cell proliferation on light microscopy, IgA deposits in the mesangium on immunoflouresence Prognosis - 25% progress to ESRD in 10-30 years

What are some secondary causes of IgA nephropathy?

HSP, Upper resp/GI infection

 Diffusely thickened GBM with IgG and C3 sub-epithelial deposits on immunofluorescence ?

This is mebranous nephroapathy

Which glomerulonephritis is associated with anti-PLA2 (anti-phospholipase A2) antibodies?

This is seen in membranous nephropathy

Whata re the antibodies measured in wegeners? Measured in microscopic polyangiits? Measured in lupus? Measured in Goodpastures syndrome?

Wegener's - c-ANCA (PR3) MPA - p-ANCA (MPO) LUPUS - ANA - antidsDNA, anti-Sm Goodpastures - glomerulonephritis with pulmonary alveolar haemorrhage - anti-GBM

What is the treatment of rapidly progressive glomerulonephritis?

Steroids (IV Methylprednisolone / Oral Prednisolone) Cytotoxics (Cyclophosphamide/ Mycophenolate/ Azathioprine and Plasmapheresis

What is RPGN associated with on biopsy?

Associated with glomerular crescents on biopsy

o 25% progress to end stage renal failure in 10-30 years o 50% progress to end stage renal failure after 10 years o 30% progress to end stage renal failure in 10 years o Does not cause progressive renal failure Which GN is each?

o 25% progress to end stage renal failure in 10-30 years - IgA nephropathy o 50% progress to end stage renal failure after 10 years - FSGS o 30% progress to end stage renal failure in 10 years - Membranous nephropathy o Does not cause progressive renal failure - minimal change disease

Which of the nephropathys causes nephritic syndrome?

this would be IgA mediated nephropathy

4 – What type of glomerular nephritis is associated with heroin use? A – Minimal change GN B – Focal segmental GN C – Membranous GN D – Membranoproliferative GN E – IgA GN F – Rapidly progressive GN

B - Focal segmetnal glomerulonephritis

5. What type of glomerular nephritis shows changes to podocytes? A – Minimal change GN B – Focal segmental GN C – Membranous GN D – Membranoproliferative GN E – IgA GN F – Rapidly progressive GN

A - Minimal change nephropathy

What type of CKD is this? eGFR 60, no evidence of kidney disease.

Not CKD

– What type of CKD is this? eGFR 60ml/min PmHx – Hemodialysis.

Stage 5 kidney disease

What is renal osteodystrophy? A – hereditary malformation of the kidney B – hereditary malformation of bone C – chronic decreased hydroxylation of Vitamin D in CKD D – decreased bone density due to low BMI E – pathological fractures found in AKI F – increased width of bone found in AKI

C – chronic decreased hydroxylation of Vitamin D in CKD Renal osteodystrophy has been classically described to be the result of hyperparathyroidism secondary to hyperphosphatemia combined with hypocalcemia, both of which are due to decreased excretion of phosphate by the damaged kidney It weakens your bones