Vasculitis Flashcards

1
Q

What occurs in large vessel vasculitis?

A

Chronic granulomatous inflammation usually of the aorta and main branches.

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2
Q

Two main types of large vessel vasculitis

A

Takayasu under 50 years

Giant Cell arteritis over 50 years

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3
Q

Takayasu

A

Under 50 year old East Asian female.
Claudication and bruits usually in the carotids.
Blood pressure differences in extremities

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4
Q

Giant Cell Arteritis

A

Over 50

Temporal artery involvement is common but can also affect other large arteries.

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5
Q

If untreated what can occur in large vessel vasculitis.

A

Vascular stenosis and distal ischaemia and aneurysm.

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6
Q

What investigations are used in large vessel vasculitis?

A

FBC- raised CRP and plasma viscosity
MR angiography- shows stenosis and thickened walls
Temporal artery biopsy - GCA
PET scan- shows increased metabolic activity in large vessels.

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7
Q

Treatments for large vessel vasculitis?

A

40-60mg of prednisolone.

Steroid sparing methotrexate may be used.

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8
Q

What is the primary medium vessel vasculitis and who does it usually affect?

A

Kawasaki

Usually children under 5

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9
Q

What are the to main types of small vessel vasculitis?

A

ANCA +ve

ANCA -ve

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10
Q

How are all small vessel vasculitis presentations similar?

A
Fever and weight loss
Raised non blanching purpuric rash
Arthralgia
Glomerulonephritis
Lung opacities on x-ray
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11
Q

Name 4 types of ANCA positive vasculitis

A

Granulomatosis with polyangitis
Microscopic Polyangitis
Renal Limited vasculitis
Eosinophilc granulomatosis with polyangitis

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12
Q

What is Granulomatosis with polyangitis also known as?

A

Wegeners

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13
Q

How does GPA usually present?

A
Sinusitis
Mouth Ulcers
Saddle Nose
Otitis media
Deafness
Uveitis and conjunctivitis
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14
Q

GPA with respiratory involvement

A

Haemoptysis

Pulmonary infiltrates and cavitating nodules on X-ray

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15
Q

GPA with cutaneous involvement

A

palpable purpura

and ulceration

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16
Q

GPA with renal involvement

A

Necrotising glomerulonephritis

17
Q

GPA with CNS involvement

A

Nerve palsy

18
Q

What is GPA associated with?

A

cANCA and PR3 proteinase 3

19
Q

Is GPA more common in men or women?

A

M:F 1.5:1
Over 35
90% N.Europe

20
Q

How is eosiniphilc granulmatoses with polyangitis different to GPA

A

Presents the same but Late onset Asthma
Raised blood eosinophil count
Rhinitits

21
Q

What is different immunological between EGPA and GPA

A

EGPA is pANCA
GPA in cANCA
Low complement levels in both

22
Q

What antibodies are associated with EGPA

A

pANCA

Anti NPO

23
Q

Small Vessel Vasculitis Investigations

A

FBC- PV and CRP raised
Anaemie- common in chronic
Immunology- ANCA Biopsy
Imaging- Chest Xray

24
Q

Investigations if suspected renal involvement?

A

Urine dipstick for proteinuria

Urgent kidney biopsy

25
Q

Management of ANCA +ve disease

A
Localised/Early
        - Methotrexate
Systemic/Agresive/Late
        - Cyclophosphamide and steroid
        - Rituximab and steroid
Followed by azathioprine or methotrexate once controlled.
26
Q

What is used in refractory ANCA +ve disease?

A

IV immunoglobulins and rituximab

27
Q

Henoch Schnlien Purpura

A

ANCA -ve disease

Ig-A dominant immune deposits.

28
Q

Who is usually affected by HSP?

A

75% children with a preceding infection Upper respiratory tract infection around 2-3 weeks prior.

29
Q

What group of bacteria are commonly linked to HSP

A

Group A Streptococcus

30
Q

What is HSP

A
Generalized small vessel vasculitis affecting the:
Skin
GI Tract
Kidneys
Joints.
31
Q

Where does HSP rarely affect?

A

The lungs

32
Q

How does HSP present?

A

Purpuric rash over buttocks and lower limbs.
Abdominal pain
D+v
Arthralgia

33
Q

Does HSP require treatment?

A

Its usually self limiting treatment is usually supportive.