anti gbm

Question 1

renal biopsy:
* Crescentic Necrotising Glomerulonephritis
* – and – deposition along the basement membrane
* Diagnosis – – disease

Answer 1

IgG + C3
anti gbm
( CHECK SLIDES 1-13 IMPORTANT )

Question 2

mechanism of tissue injury in anti gbm:
* Type – Hypersensitivity
Antibody mediated –
* Antibody binds to – component
* – is activated
* Influx of — cells lead to tissue
damage

Answer 2

II
cytotoixty
tissue
complement
inflammatory

Question 3

autoantibodies:
* Found in many – diseases
* Presence does not imply role in –
* Useful in —
* In anti-GBM disease, antibodies are –
* Antibody is directed against – domain of type —
* Type IV collagen found in – , and – of alveoli

Answer 3

autoimmune
pathogenesis
diagnosis
pathogenic
alpha 3 domain of type iv collagen
GBM and basement memberane

Question 4

natural history of the disease:
* Rapidly progressive glomerulonephritis (RPGN)
* If untreated results in — within days
* Risk of –
* Medical emergency

Answer 4

renal failure
catastrophic pulmonary haemorrhage

Question 5

( treatment for disease)
steroids are potent — , at high doses will be — and several raise of high dose – steroids

Answer 5

anti inflammatories
immunosuppressive
IV

Question 6

plasma exchange:
- — removal of
plasma; replacement with
appropriate – .
- Removal of – , and other –
- Used – / every –
- Rapid reduction of –

Answer 6

mechanical
fluid
antibodies
plasma protein
used daily every second of the day
anti GBM

Question 7

in plasma exchange ;
* Rapid removal of–
* Need – lines and high –
* Significant – risk
* Haemodynamic –
* Can worsen –
* Removes –
* Does not reduce –
* Risk of – and increase in antibody
level when you stop

Answer 7

antibody
central
high blood flow
infection
instability
anaemia
antibody
production
rebound
( check slide 20,2123,24,25)

Question 8

• patients parents are keen to be considered as donors
• – is the optimal form of renal replacement therapy – better survival and better Quality of Life compared to dialysis
• Transplant can be considered if risk of – is reasonable
• What affects risk of recurrence in anti-GBM disease?

Answer 8

transplantation
recurrence

Question 9

patient worked up for transplant:
* Blood Group –
* – type performed and checked
* – antibodies against 45% of Irish population
* Why do you think he has anti-HLA antibodies?

Answer 9

A
HLA
anti HLA
(check slide 28)

Question 10

post transplant:
* Low risk of –
* Was negative for anti-GBM at the time of transplant
* Anti-GBM is usually a – disease
* Even without treatment, anti-GBM usually – modulates within 2 years
* Management post transplant is standard— as: .
– –
– –
– –

Answer 10

recurrence
one phase
self
immunosuppression
steroids
tacrolimus
mycophenolate

Question 11

summary:
* Anti-GBM disease is a medical –
* Rapid – and – can be life & organ- saving
* Pathogenesis is antibody mediated –
* Treatment geared to remove – quickly, modulate – and prevent antibody – .
* Measurement of the – is a sensitive diagnostic tool
* Antibody measurement is useful to – treatment, and risk assess recurrence post transplant
* If antibody – at transplant prognosis excellent

Answer 11

medical emergency
diagnosis n treatment
cytotoxuty
antibody
inflammation
resynthesis
monitor
negative