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MSK > Vasculitis > Flashcards

Vasculitis Flashcards

1
Q

What is vasculitis?

A

Inflammation of blood vessels, often with ishemia, necrosis and organ inflammation

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2
Q

What blood vessels can vasculitis affect?

A

Arteries, arterioles, vein, venules or capillaties

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3
Q

What is primary vasculitis?

A

Results from an inflammatory response that targets vessel walls with no known cause. Commonly autoimmune

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4
Q

What is secondary vasculitis?

A

Triggered by an infection, drug (e.g. cocaine) or a toxin that may occur as part of another inflammatory disorder or cancer

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5
Q

What will occur at a cellualr level in vasculitis?

A

Release of inflammatory cytokines activating T cells and vascular inflammation. This promotes granuloma formation and macrophage activation.
This will lead to inflammation, endothelial damage, disruption of elastic lamina and intimal hyperperplasia

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6
Q

What are examples of large-vessel vsaculitis?

A

Takayasu arteritiis

Giant Cell arteritis

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7
Q

What are examples of medium-vessel vasculitis?

A

Polyarteritis nodosa

Kawasaki disease

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8
Q

What are ANCA-positive small vessel vsaculitis?

A

Microscopic polyangitis
Granulomatosis with polyangitis (wegner’s)
Esoinophilic granulomatosis with polyangitis (churg-strauss)

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9
Q

What will churg strauss also present with along with vasculitis?

A

Rhinorea, asthma and eosinophilia

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10
Q

What non-ANCA small vessel vasculitis is Ig-A dominant?

A

Henoch-Sschnelin purpura

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11
Q

What are non-ANCA, immune complex small vessel vasculitis?

A 
Cryoglubinemic vasculitis
IgA vasculitis (henoch-Shconlein)
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12
Q

What non-ANCA small vessel vasculitis has high serum cryoglobulin?

A

Cryglobulinemia

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13
Q

What systemic symptoms can present with vasculitis?

A

Fever
Malaise
Weight loss
Fatigue

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14
Q

Who will TA affect?

A

Under 40
Females
Asian population

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15
Q

Who wil GCA affect?

A

Over 50

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16
Q

What are the presenting features of large cell vasculitis?

A

Bruit - most commonly around carotid artery
BP difference in extremities
Claudication

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17
Q

What muscle disease does GCA have an association with?

A

Polymyalgia Rheumatica

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18
Q

What are the symptoms of GCA?

A

Unilateral temporal headache
Scalp tenderness
Jaw claudication

19
Q

What occular symptom can GCA present with?

A

Blindness due to ischaemia of optic nerve and opthalmic artery

20
Q

What are the investigations for GCA?

A

ESR, PV and CRP
Temporal artery biopsy
MR anigiogram or PET CT

21
Q

How is GCA managed?

A

40-60mg prednisolone

Higher dose if visual loss

22
Q

What is the pathology of GPA?

A

Granulomatous inflammation of resp tract, small and medium vessels
Necrotising glomerulonephritis

23
Q

What is the pathology of EGPA?

A

Eosinophilic granulomatous inflammation of resp tract, small and medium vessels
Associated with asthma

24
Q

What is the pathology of MPA?

A

Necrotising vasculitis with few immune deposits

Necrotising glomerulonephritis very common

25
Q

Who is likey to get GPA?

A

Slightly more common in males
35-55 years
Constitutional symptoms and arthralgia are common

26
Q

What is the classification of GPA?

A

Nasal or oral inflammation
Abnormal chest radiograph - nodules, fixed infiltrates or cavities
Urinary sediment - microhaematuria or red cell casts
Granulomatous inflammation on biopsy

27
Q

What ENT features can GPA show?

A 
Sinusitis
Nasal crusting
Epistaxis
Mouth ulcers
Saddle nose
28
Q

What resp features can GPA show?

A 
Pulmonary infiltrates
Cough
Heamoptysis
Diffuse alveolar haemorrhage
CAvitating nodules
29
Q

What cutaneous features can GPA show?

A

Palpable purpura

Cutaneous ulcers

30
Q

What renal features can GPA show?

A

Necrotising golemrulonephritis

31
Q

What nervous system features can GPA show?

A

Mononeuritis multiplex
Sensorimotor polyneuropathy
Cranial nerve palsies

32
Q

What occular features can GPA show?

A 
Conjunctivitis
Episcleritis
Uveitis
Optic nerve vasculitis
Retinal artery occlusion
33
Q

What is the main difference between GPA and EGPA?

A

EGPA will occur with late onset asthma and a high eosinophil count

34
Q

What is the critera for EGPA?

A

Ashtma
Eosinophilia of more than 10% in peripheral blood
Paranasal sinusitis
Pulmonary infiltrates
Histological proof of vasculitis with extravascular eosinophils
Mononeuritis multiplex or polyneuropathy

35
Q

What condition will have c-ANCA and PR3 antibodies?

A

GPA

36
Q

What condition will have p-ANCA MPO antibodies?

A

MPA and EGPA

37
Q

How is anca associated vasculitis managed?

A

Cyclophosphamide

38
Q

What is henoch-schonlein purpura (HSP)?

A

Acute IgA mediated disorder that causes generalised vasculitis

39
Q

What can HSP affect?

A 
Small vessels of skin
GI trct
Kidneys 
Joints 
RARE: lungs and CNS
40
Q

Who is likely to get HSP?

A

Children aged 2-11

41
Q

What will usually precede HSP?

A

Preceding URTI, pharyngeal infection or GI infection

Commonly group A streptococci

42
Q

How will HSP present?

A 
Purpuric rash over buttocks and lower limbs
Colickly abdo pain
Bloody diarrhoea
Joint pain +/- swelling
Renal involvement in 50%
43
Q

How is HSP managed?

A

Usually self-limiting
Symptoms tend ot reslove within 8 weeis
Essential to perform urinalysis to screen for renal involvement

MSK (34 decks)

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