Vasculitis Flashcards

0
Q

What material is in each of the heart layers

A
Intimate = endothelial cells resting on BM
Media = smooth-muscle
Adventitia = connective-tissues
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
1
Q

What is vasculitis

A

Inflammation of
small sized vessels
Medium sized vessels
OR COMBO

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
2
Q

What is the aetiology of vasculitis

A

Unknown - most cases NOTTTTT infectious

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
3
Q

Non-specific symptoms of vasculitis

A

Fever, fatigue, weight loss, myalgia

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
4
Q

What does vasculitis preferentially involve?

Symptoms of organ ischaemia?

A

Vasculitis preferentially involves certain organs vascular beds

  1. Damage endotheliocyte all – >
    expose subendothelial collagen – >
    activate thromboplastin -> activate coag cascade ->
    THROMBOSIS
  2. Inflammation of wall -> healing –> fibrosis BV wall –> narrow lumen = decreased blood flow – >
    organ ischaemia
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
5
Q

Give two large vessel vasculitis
How do they present?

GreaT PaKi Buerger
GWanny Chemist

A

GreaT (large!!)

Giant cell/temporal arteritis
Takayasu arteritis

Present with loss of pulse/ stroke

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
6
Q

Give two medium vessel vasculitis
How do they present?

GreaT PaKi Buerger
GWanny Chemist

A

Polyarteritis nodosa
Kawasaki

Present = vessel thrombosis + infarction/aneurysm

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
7
Q

Give two small vessel vasculitis
How do they present?

GreaT PaKi Buerger
GWanny Chemist

A

Granulomatosis with polyangiitis = wegener
Churg Strauss
HEnoch Schonlein purpura
Microscopic Polyarteritis

Present = skin overlying vasculitis =
haemorrhagic raised painful @ palpation

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
8
Q

Who does giant cell temporal Arteritis happen in?

Classic location of giant cell temporal arteritis?

A

Females
> 50

Branches of carotid
Temporal – headache
Ophthalmic – visual issues
Artery of jaw muscles - Jaw claudication

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
9
Q

What other disease is giant cell temporal arteritis associated with

A

Polymyalgia rheumatica – flulike symptoms + joint and muscle pain
Increased ESR > 100

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
10
Q

What does biopsy of giant cell temporal arteritis reveal

A

Inflamed vessel wall
Giant cells = granulomatous inflammation
Intimal fibrosis

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
11
Q

For giant cell temporal arteritis what should you be wary of during biopsy

Treatment? What happens if you don’t treat?

A

Lesions are segmental

Only one area could be involved

@biopsy

  • take long piece
  • examine all of piece under a microscope
  • if negative – doesn’t mean there is no disease = could have taken part of vessel where disease isn’t present

corticosteroids - without treatment = blindness

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
12
Q

Explain how Takayasu arteritis differs from giant cell temporal Arteritis.

Treatment for Takayasu arteritis?

A

Giant cell temporal arteritis =
Females >50

Takayasu arteritis =
Young Asian female < 50

GCTA + Carotid branches

Takayasu Arteritis - branch points of Aorta
Granulomatous thickening + narrowing aortic arch

CORTICOSTEROIDS

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
13
Q

What organs does polyarteritis nodosa involve

A

Most organs except lungs - NOT pul art!!

CNS vessels - NEURO dysfunction
Visceral art. - mesenteric vessels ab pain + melena
Renal vessels - HTN/renal damage
Necrotising skin lesions - cutaneous eruptions

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
14
Q

In 30% of patients with polyarteritis nodosa say what serum surface antigen is present?

A

Hep B

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
15
Q

Explain the pathogenesis of polyarteritis nodosa

Treatment for polyarteritis nodosa

A

Early lesions = transmural inflammation – whole vessel is inflamed with fibrinoid necrosis
Bumps = aneurysm
Bits in between bumps = fibrosis

After healing

End lesions = massive fibrosis – string of pearls
bumpy @ blood vessel (Nodosa = feels like a node)

Corticosteroids + CYCLOPHOSPHAMIDE

16
Q

Who does Kawasaki disease happen in?

A

Asian kids < 4

FCETH DCL

fETCh CoLD

Erythematous rash @ palms/soles
Thrombosis/rupture cos of Coronary Art aneurysm
Cervical lymphadenitis

COnjunctivitis
Lips + oral mucosa strawberry tongue
Desquamating rash

17
Q

How do you treat a kid with Kawasaki?

A

Aspirin –> inhibit Cox = Decrease THx A2 ->

Decreased aggregation -> destroy thrombus

18
Q

What happens in buerger thromboangiitis

A

Intermittent claudication – >

bUeRGArS disease

B--
Ulceration
E--
Raynaud of fingers
Gangrene
Auto-amputation 
R--
Superficial nodular phlebitis of leg veins
19
Q

Who does buergers thromboangiitis happen in?

How do you treat this?

A

Heavy smokers < 40yrs

Stop smoking

20
Q

What happens in the weCener granulomatosis

A

Necrotising granulomatous vasculitis @

Nasopharynx - sinusitis/nasopharyngeal ulcer

Lung - Hemoptysis/bilateral nodular lung infiltrate

Kidney - hematuria - rapidly progressive glomerulonephritis

21
Q

What are the ANCA results for the small vessel Vasculitis

A

WeCener = c-ANCA - anti-proteinase 3

Micro. polyangiitis = p-ANCA - anti-myeloperoxidase

Churg Strauss = p-ANCA

22
Q

What does biopsy revealed the wegener granulomatosis

Treatment?

A

Necrotising granuloma @ lung/upper airway

Corticosteroids + Cyclophophamide

23
Q

How does microscopic polyangiitis differ from wegener?

Treatment?

A

No nasopharyngeal involvement
P – ANCA
No granuloma

pauci-immune glomerulonephritis
Palpable purpura

Corticosteroids + Cyclophophamide

24
Q

Which vasculitis require corticosteroids and cyclophosphamide?

Which vasculitis requires corticosteroids only?

Which vasculitis require
smoking cessation +
IV Ig + aspirin

A

Polyarteritis nodosa, Wegener, microscopic polyang

Giant cell temporal arteritis, Takayasu

Burger disease, Kawasaki

25
Q

Which vasculitis diseases are necrotising

A

Burger, Wegener, Microscopic polyangiitis,

Churg Strauss

26
Q

What do you get Churg Strauss syndrome

A

GAPe PaPES

Granuloma necrotizing, Asthma, PEripheral eosinophilia

Palpable purpura, Peripheral neuropathy, Eosinophils, Sinusitis

27
Q

What is the most common vasculitis in children?
Pathophys?
What is the classic presentation?

A

HEnoch-Schonlein Purpura

Upper respiratory infection – > XS IgA production ->

Arthralgia, GI pain/Melena, PALPABLE purpura

28
Q

Which vasculitis are similar to each other

A

Giant cell temporal = Takayasu

Wegener = Microscopic Polyangiitis = Churg Strauss