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Systemic renal Flashcards

1
Q

If you had to break small vessel vasculitis into two categories, what would they be?

A

Typically we talk about:

  1. pauci-immune (no immune complexes on IF)
  2. immune-complex mediated
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2
Q

What are a couple of causes of pauci-immune vasculitis?

A

This is another name for the ANCA associated vasculidites.

Microscopic polyangiitis
Wegener’s granulomatosis (now known as granulomatosis with polyangiitis)
Churg-Strauss syndrome
Drug-induced ANCA associated vasculitis (hydralazine, penicillamine, PTU)

Atypical ANCA is associated with IBD

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3
Q

What are some causes of immune-complex renal vasculitis

A

this list is not exhaustive:

cryoglobulinaemic vasc

HSP (IgA)

Infection - induced immune complex vasc - Hep C, endocarditis

SLE

Rheumatoid vasculitis

Serum sickness vasculitis

Behcet’s disease

Drug induced immune-complex vasculitis

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4
Q

How on earth does ANCA cause disease?

A

They are antibodies against the neutrophils, and it causes activation of these cells!

The activated neutrophils then adhere to endothelial cells and release inflamm mediators

It is crucial (for understanding of immunofluorescence) that you understand that ANCA is NOT in the kidney. It is the neutrophils that cause disease. There is no immune complex deposition

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5
Q

What are the ANCA-associated disease syndromes?

A

Overall the renal pathology is similar.

WG - necrotising granulomatous disease often involving the resp tract. Can also have sinusitis, rhinitis, otitis media and ocular inflammation.
Horrible granulomatous lesions in the lungs

Microscopic polyangiitis

Churg-Strauss - associated with asthma, eosinophils and to lesser extent, necrotising granulomatous inflammation

idiopathic necrotising GN

All of WG, CS and MP can be associated with pulmonary haemorrhage

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6
Q

What is the crucial finding on renal biopsy test for ANCA vasculitis?

A

There are no deposits on the immunofluorescence.

IF stains are for IgA, IgM, IgG, C3, C1q

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7
Q

What is the treatment of lung haemorrhage of ANCA assoc vasculitis?

A

Plasma exchange may be beneficial in this setting

obviously supportive treatment also essential

Can you think of any other situations with ANCA vasc where plasma exchange has emerging evidence?

in dialysis dependent renal disease

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8
Q

What is the treatment of ANCA-vasculitis?

A

Immunosuppression is the name of the game. We go for hardcore induction first.

IV ‘roids and cyclophosphamide

Then for maintenance we switch to pred and aza or pred and MMF

Ritux may have a place, particularly in relapsed disease

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9
Q

What are the major complications of cyclophosphamide?

A

The most important for patients is gonadal toxicity

for men, the cumulative toxic dose is 100mg/kg
for women, the toxic is 200mg/kg

women who receive this treatment who are older than 30 frequently become menopausal

It is also teratogenic
It causes bone marrow suppression

It causes nasty infections - PCP, nocardia, TB and HPV

There are malignancies associated - bladder CA, skin, lymphoma

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10
Q

What is Anti-GBM disease?

A

This is also known as Goodpasture’s disease.

It is GN +/- pulm haemorrhage

It is a well-described antibody to type IV collagen (chromosome 2)

The antibody is pathogenic. It can even reach levels of 1% of circulating IgG!

It binds rapidly to the GBM with high affinity and can cause fulminant disease

It is believed that there needs to be an environmental stimulus for the pulm haemorrage (e.g. smoking will damage the lung ever so slightly and set it all off)

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11
Q

What do we see on histology in the setting of Goodpasture’s?

A

We see focal and segmental GN.
There is leucocyte infiltrate and segmental necrosis
Crescents will be visible
Destruction of GBM will be seen

On IF, linear IgG will be seen

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12
Q

How do we treat anti-GBM disease?

A

Plasma exchange is best

then immunosuppression - ‘roids and cyclophos

if you can turn the disease off, then it’s about the prior damage.

if dialysis dependent at presentation, then never get off

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13
Q

Which of the antibodies are strongly associated with SLE nephritis?

A

anti-dsDNA and anti-SM Ab are strongly associated

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14
Q

are the sero-neg and drug associated lupus more, or less, associated with renal involvement?

A

they tend to have little renal involvement.

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15
Q

does renal nephritis stay at the same stage, or switch from one to another?

A

it can do anything. people without involvement can get involvement, and people can switch from stage to stage.

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16
Q

Describe the pathogenesis of SLE?

A

Overall it is an immune complex GN.

The complexes are formed from DNA-antiDNA, C1q, Ro, chromatin, laminin

The big complexes and negatively charged complexes seem to bind to subendothelial - this represents class III or IV nephritis

The positive seem to bind to epithelial and create a more indolent membranous pattern (V)

It may come to pass that we find out that IgG subclass will be important.

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17
Q

What do we see on renal biopsy in stage III/IV lupus nephritis?

A

this is a proliferative GN

this is the “full house” of immune deposits (IgG, IgA, IgM, complement etc).

these patients also often have high serological titres

The biopsy will also show cellular prolif, crescents, PMNs, “wire loops”

fibrin and thrombi and necrosis too

18
Q

What is the prognosis of SLE nephritis Class IV?

what about recurrence post transplant?

A

complete remission is 25-85% at 12 months! (complete is <0.5g/day proteinuria)

ESRF 2 - 20% at 5 years

relapses are common. depends on disease activity. poor compliance associated with relapse (WHAT A SURPRISE!)

recurrent post transplant is about 30%, but rarely causes graft loss

prognosis of renal survival is much better if you can get patient into remission (95 v 60% at 10 year)

19
Q

What is the treatment of Class IV lupus nephritis?

A

Induction therapy (3-6 months):

  1. IV or oral glucocorticoid
  2. oral MMF OR: IV cyclophosphamide

Maintenance:
oral glucocorticoid, oral MMF with plan to step down to oral Aza once stable

there is emerging evidence that plaquenil is useful in maintenance too

20
Q

what are the benefits of oral mycophenolate v cyclophosphamide in the SLE population

when would you elect for cyclophos?

A

There were many benefits, from less leucopenia, to better achievement of remission.

However, given the population that often suffers SLE nephritis - MMF was less associated with amenorrhoea cf cyclophos

according to Dr Van Eps, you would look towards cyclophos if the disease was very severe. (I’m not sure if this is because of lack of data, or weaker mech of action)

21
Q

What is the histopath of class IV SLE nephritis?

What is the treatment?

A

It is also known as membranous lupus nephritis. It looks almost the same as idiopathic membranous, but with idiopathic IF shows IgG and maybe some comp, and class V lupus has “full house”

treatment in bad disease is same as class IV - cyclophos and aza or MMF

if okay, then calcineurin inhibitor

NOTE: this is different treatment to idiopathic membranous

22
Q

What is the clinical triad of haemolytic uraemic syndrome

A

clinical triad of renal failure, microangiopathic haemolytic anaemia and thrombocytopenia

23
Q

What are the types of HUS?

A
  1. diarrhoeal (typical) - Shiga toxin
    - E coli
    - Shigella toxin
  2. non-diarrhoeal (atypical)
    - infection - pneumococcus, Q fever, HIV, CMV
    - drugs - cyclosporine, OCP, cisplatin, bleomycin
    - radiation
    - pregnancy (?is it therefore oestrogen dependent?)
    - familial/sporadic
24
Q

What is the long term prognosis of typical HUS?

A

recovery of renal function seen in 95% of cases

about 50% had CKD in the Adelaide outbreak

25
Q

What is the story with the pathogenesis of atypical HUS?

A

it seems that some people have an abnormality in their complement regulation. It seems that mutations in the alternative pathway are the most likely candidate

These people with susceptible complement - have an activation event (some sort of triggering event) which then leads to cascade which leads to damaged endothelial surface

26
Q

What is the treatment of HUS?

A

Plasma exchange, but limited success.

plasma exchange WITH FFP is important

There is a monoclonal antibody, Eculizumab, which is MAb against C5. This drug is currently licensed for PNH

27
Q

What are the findings on renal biopsy of an amyloid kidney?

A

this is a non-proliferative nephropathy. that means we have more protein in the glom, but not more cells

use congo red stain

apple green birefringence under polarised light

you can also use specific stains against particular antibodies, like IgK, IgL light chains

28
Q

What is the most common of the amyloid?

A

AL amyloid.
there is an overlap with myeloma (10%)

presents with weakness and fatigue and weight loss and oedema

nephrotic range proteinuria and progressive CKD

also seen a restrictive CMP
gut infiltration causes malabsorption
can cause autonomic neuropathy

can cause painful sensory neuropathy

adrenal involvement –> Addison’s

29
Q

What is the investigations of AL amyloid?

A

urinary BJP
SEPP
free light chains

30
Q

What is AA amyloid?

A

This is due to deposition of acute phase reactant protein, SAA.

Chronic infections cause it, such as TB
Long term autoimmune diseases

Predominantly affects the kidney - uncommon to involve heart, tongue, nerves

31
Q

What are the ways that myeloma causes acute kidney injury?

What about chronic kidney disease?

A

AKI:

  1. hypercalcaemia
  2. hyperuricaemia
  3. dehydration
  4. contrast nephropathy
  5. cast nephropathy

CKD:

  1. AL amyloid
  2. cast nephropathy
  3. Ig deposition disease
  4. tubular dysfunction (Fanconi’s syndrome)
32
Q

How do we treat myeloma cast nephropathy (if it’s early)?

A

we can use high cut off filters - these are expensive but seem to remove a lot of circ. protein

serum creatinine is a predictor of outcome

33
Q

What type of GN is hep C typically associated with?

A

Mesangiocapillary GN

34
Q

What type of GN is hep B typically associated with?

A

membranous GN

35
Q

What type of GN is HIV typically associated with?

A

collapsing FSGS - but this is typically only Afrian Americans

in caucasian it would be more likely to be meds

36
Q

What are the GN associated with sore throat?

A

Post-strept GN - associated with Group A, beta-haemolytic strept

the latent period is 10 days with pharyngitis and 21 with impetigo. HOWEVER, with alcoholics etc, it is now becoming associated with staphylococcus and G neg organisms and endocarditis

IgA nephropathy is different. It is haematuria that is SYNPHARYNGITIC

37
Q

What is mesangiocapillary GN?

A

This is an immune complex GN.

It is mCgn = the C is for complement! (also known as membranoproliferative)

There are 3 subtypes

Hep C is a very common cause

38
Q

What is the treatment of MCGN?

A

mesangiocapillary GN associated with Hep C should be treated for its underlying disease (interferon alpha)

39
Q

What is the condition Thin Membrane disease, and how do we diagnose?

A

It is microscopic haematuria and normal renal function.
It has a good prognosis
The GBM < 250nm (this is the diagnostics)

there is usually an autosomal dominant inheritance (it is familial)

40
Q

What is Alport’s syndrome?

A

this is a condition of progressive renal impairment, sensorineural deafness and lens (eye) abnormality

it is predominantly X linked

overall it is a condition associated with abnormality of the non-collagenous domain of type IV collagen.

There is no specific treatment

41
Q

GN with skin lesion differential list!

A 
HSP
cryoglobunlinaemia
SLE
ANCA related vasculitis
TTP
SBE
hypersensitivity vasculitis
42
Q

Which GN are associated with pulmonary haemorrhage?

A

microscopic polyangiitis
WG
Anti-GBM disease

SLE
endocarditis
GN with pulm oedema

Renal (9 decks)

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