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SPM- Renal > Kidneys and ANCA Vasculitis > Flashcards

Kidneys and ANCA Vasculitis Flashcards

1
Q

How do ANCA vasculitic diseases cause problems to blood vessels?

A

ANCA- Anti Neutrophil Cytoplasm

There is inappropriate degranulation of neutrophils within blood vessels which causes release of pro-inflammatory cytokines and proteins resulting in damage and inflammation. Neutrophils also adhere to the endothelium.

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2
Q

What are the two types of ANCA? What do they target?

A 
C-ANCA = Anti-proteinase 3 (seen in GPA)
P-ANCA= Anti-myeloperoxidase (seen in MPA)
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3
Q

What are the three ANCA associated vasculitic disorders?

A 
GPA (c-ANCA)
Microscopic Polyangitis ((p-ANCA)
Eosinophilic GPA (Churg-Straus Syndrome)- but around 70% are ANCA -Ve
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4
Q

What are some general symptoms of vasculitis?

A

Fever
Weight loss
Fatige

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5
Q

What are some specific symptoms of vasculitis?

A 
Recurrent nose bleeds
Rash
Pulmonary haemorrhage and haemoptysis
Haematuria
Temporal Headache- Giant cell arteritis
Jaw Claudication- Giant Cell Arteritis
Anterior Ischaemic optic neuropathy- Giant Cell Arteritis
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6
Q

What type of vasculitis is associated with ANCA?

A

Small vessel vasculitis- GPA, MPA, Eosinophilic GPA

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7
Q

What is the most common small vessel vasculitis?

A

GPA- Granulomatosis with Polyangitis

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8
Q

What does c-ANCA target?

A

Proteinase 3

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9
Q

What does p-ANCA target?

A

Myeloperoxidase

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10
Q

What are some of the symptoms of GPA?

A

Affects:
Nasopharynx- Recurrent Nose Bleeds, Chronic Sinusitis (swelling seen around eyes) Saddle nose deformity
Lungs- Haemoptysis, Difficulty breathing
Kidneys- Haematuria, increased blood pressure

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11
Q

What is the treatment for GPA?

A

Steroids
Cyclophosphamide
Other immunosupression

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12
Q

What type of ANCA is associated with microscopic poly-angitis?

A

p-ANCA- Anti-Myeloperoxidase

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13
Q

Where does myeloperoxidase affect?

A

Does not affect the naso-pharynx so is not associated with recurrent nose bleeds (unlike GPA)

Affects kidneys, eyes (causes scleritis), lungs

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14
Q

How can you differentiate between MPA and GPA on a biopsy?

A

GPA shows granulomatous inflammation

MPA does not show granulomatous inflammation

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15
Q

What is Churg-Strauss syndrome also called?

A

Eosinophilic GPA

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16
Q

What does Churg-Strauss cause to be elevated on an FBC?

A

Eosinophils are raised- eosinophilia

Churg-Strauss is associated with late onset asthma

17
Q

What are some areas affected by Churg-Strauss?

A 
Sinuses
Lungs- Pulmonary Infiltrates 
Kidney
GI
Skin
Heart- Myocarditis
Nerves- Peripheral Neuropathy
18
Q

What do Churg-Strauss and GPA have in common?

A

Both are associated with granuloma formation

Churg-Strauss is also called eosinophilic GPA

19
Q

In a patient with small cell vasculitis what may be seen on a chest X-ray?

A

Pulmonary haemorrhage

Granulomas- but these are not due to TB which is another cause of granulomas in the lungs

20
Q

What kind of rash is seen with a small vessel vasculitis?

A

Purpuric rash- due to bleeding from small vessels. These rashes are palpable (slightly raised)

21
Q

If suspecting a vasculitic problem what investigations should be done?

A

Raised ESR and CRP (ESR particularly raised in small vessel vasculitis)
Immunology tests- ANCA (p or C), FBC (Eosinophilia is associated with Churg-Strauss)
Biopsy of affected areas- GPA and Churg-Strauss involve granuloma formation

22
Q

What drugs may cause a drug induced vasculitis?

A

Anti-TNF
Hydralazine
Propythiouracil
Cocaine

23
Q

What are the two steps to managing a vasculitis?

A

Induce remission

Maintain remission

24
Q

What is the treatment to induce remission for the small vessel vasculitis?

A

Induce remission using:
Steroids e.g. prednisolone

If severe:
Cyclophosphamide
Rituximab (anti-B Cell)
Plasma Exchange to remove ANCA

If mild or moderate:
Methotrexate
Mycophenolate Mofetil

25
Q

What treatments may be used to maintain remission in small vessel vasculitis?

A

Generally want to avoid steroid regimes

Cyclophosphamide
Rituximab (Anti B Cell)
Methotrexate
Mycophenolate Mofetil
Azathioprine
26
Q

For safe immunosuppression what should be checked first?

A 
Current infections
HIV, HBV, HCV status
TB status
VZV immunity
PJP Prophylaxis (ABx treatment)

SPM- Renal (9 decks)

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