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MSK > Vasculitis > Flashcards

Vasculitis Flashcards

1
Q

What is vasculitis

A

Inflammation of blood vessels

Prolonged inflammation can lead to thrombosis, ischemia, organ infarction necrosis and even death

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2
Q

Which blood vessels can vasculitis

A

ANY

Arteries, arterioles, veins, venules or capillaries

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3
Q

What causes secondary vasculitis

A

May be triggered by an infection, drugs, a toxin or may occur as part of another inflammatory disorder or cancer

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4
Q

How do you classify vasculitis

A

Based on which type of blood vessel is affected

Small, medium or large

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5
Q

How does vasculitis present

A

Very variable - dependant in which vessels are affected
Systemic symptoms such as fever, malaise, weight loss and fatigue are common to all types
Can cause thrombosis and infarction as the inflammation of the blood vessel narrows the lumen

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6
Q

What does Giant cell arteritis typically cause

A

Temporal arteritis

May affect aorta and other large vessels

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7
Q

What is seen in blood vessel walls in vasculitis

A

Granuloma formation

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8
Q

What are the presenting features of large vessel vasculitis

A

Symptoms are dependant on which arterial branches are affected
Bruit - most commonly in carotid
Blood pressure difference in extremities

Takayasu - cannot palpate peripheral pulses

GCA
Headache
Diplopia or visual loss
Claudication - jaw and tongue

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9
Q

What are the signs of temporal arteritis

A

Unilateral temporal headache
Scalp tenderness
Jaw claudication
Temporal arteries become prominent but with reduced pulsation

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10
Q

What is the major risk with temporal arteritis

A

Blindness

Due to ischaemia of the optic nerve

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11
Q

What investigations do you do for large vessel vasculitis

A

ESR, plasma viscosity and CRP - will be raised
Temporal artery biopsy - GOLD STANDARD
MR angiogram or PET CT

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12
Q

How is small vessel vasculitis divided

A

Into ANCA associated vasculitis (AAV) and ANCA negative

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13
Q

Describe the pathology of GPA

A

Granulomatous inflammation of respiratory tract, ENT system and small and medium vessels
Necrotising glomerulonephritis common

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14
Q

Describe the pathology of EGPA

A

Eosinophilic granulomatous inflammation of respiratory tract, ENT system, small and medium vessels.
Associated with asthma

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15
Q

Describe the pathology of MPA

A

Necrotising vasculitis with few immune deposits.

Necrotising glomerulonephritis very common

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16
Q

What are the ENT features of GPA

A 
Sinusitis
Nasal crusting
Blocked nose
Epistaxis
Mouth ulcers
Sensorineural deafness - bilateral
Otitis media and deafness
“Saddle nose” due to cartilage ischaemia and necrosis
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17
Q

What is epistaxis

A

Nose bleed

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18
Q

What are the cutaneous features of GPA and what causes them

A

Palpable purpura
Petichial rashes
Cutaneous ulcers
Caused by inflammation of the small blood vessels in the skin

19
Q

What are the respiratory features of GPA

A 
Pulmonary infiltrates
Interstitial lung disease 
Cough and SOB
Haemoptysis 
Diffuse alveolar haemorrhage
Cavitating nodules on CXR

Stridor caused by tracheal inflammation and resulting subglottic stenosis

20
Q

What is the key renal feature of GPA

A

Necrotising glomerulonephritis
Can lead to rapid renal failure

Look for blood and/or protein in urine. Any more than a trace requires further tests (red cell cast test and protein creatinine ratio)
May go onto get renal biopsy

21
Q

What are the nervous system features of GPA

A

Mononeuritis multiplex - foot and wrist drops
Peripheral neuropathy - glove and stocking
Cranial nerve palsies

22
Q

What are the ocular features of GPA

A 
Conjunctivitis 
Episcleritis 
Scleritis - nodular 
Uveitis 
Optic nerve vasculitis 
Retinal artery occlusion  
Proptosis
Retro orbital granulomas mimicking tumours
23
Q

What are ANCAs

A

Anti-neutrophil cytoplasmic antibodies

Autoantibodies against the cytoplasm of neutrophil granulocytes

24
Q

How can you detect ANCA

A

Immunofluorescence

Also allows you to determine the pattern and differentiate between C-ANCA and P-ANCA

25
Q

What is HSP

A

Henoch-Schönlein purpura
Acute IgA mediated disorder
Generalized vasculitis - not ANCA associated

26
Q

What blood vessels are affected by HSP

A

Iinvolves the small vessels of the skin, the gastrointestinal (GI) tract, the kidneys, the joints, and, rarely, the lungs and the central nervous system

27
Q

HSP usually has a preceding infection - true or false

A

TRUE

>75% have URTI, pharyngeal or GI infection Usually about 1-3 weeks before HSP

28
Q

What organism most commonly causes the infection preceding HSP

A

Group-A strep

29
Q

How does HSP present

A 
Purpuric rash typically over buttocks and lower limbs
Colicky abdominal pain
Bloody diarrhoea
Joint pain +/- swelling
Renal involvement - 50%
30
Q

How do you manage HSP

A

Usually self limiting and will resolve in 8 weeks

Need to check for renal involvement and treat accordingly

31
Q

What are the main subtypes of large vessel vasculitis

A

Giant cell arteritis - seen in over 50s

Takayasu - seen in younger women

32
Q

Which vessels are affected in large vessel vasculitis

A

Involves the aorta and branches

33
Q

What are the main subtypes of medium vessel vasculitis

A

Kawasaki disease - seen in kids
polyarthritis nodosa

Rarest types of vasculitis

34
Q

List the ANCA associated small vessel vasculitis

A

Microscopic polyangitis (MPA)

Granulomatosis
with polyangiitis (GPA
Previously Wegener’s

Eosinophilic Granulomatosis
with polyangiitis (EGPA)
Previously Churg Strauss

35
Q

ANCA negative small vessel vasculitis is also called what

A

Immune complex small vessel vasculitides

Includes HSP, anti-GBM disease etc

36
Q

What are the MSK features of GPA

A

Myalgia
Arthralgia
Arthritis

37
Q

What are the cardio features of GPA

A

pericarditis, coronary

vasculitis myocarditis if coronary arteries are involved

38
Q

What is the screening test for small vessel vasculitis

A

ANCA or anti-neutrophil cytoplasmic antibody

39
Q

Which blood tests may be abnormal in vasculitis

A

FBC - Anaemia of chronic disease(normocytic, normochromic
anaemia) is a common finding

CRP/ESR/PV - usually raised due to inflammation

LFTs can be deranged if liver involvement
U&E deranged if renal involvement

40
Q

Which subtypes of ANCA are seen in GPA

A

c-ANCA and anti-PR3

41
Q

Which subtypes of ANCA are seen in EGPA and MPA

A

p-ANCA
Directed against myeloperoxidase (MPO)
Anti-MPO

42
Q

Can ANCA be used to monitor disease activity

A

Yes

Titres often rise with increasing disease activity

43
Q

How do you treat vasculitis

A

Basically immunosuppression (lifelong) with steroids and steroid sparing agents such as cyclophosphamide,
rituximab, methotrexate, azathioprine etc
Choice of agent depends on how severe the disease is - use the 5 factor score to see how many organ systems are involved
If they score 1 or more - need high dose steroids with either cyclophosphamide or rituximab
Then move to steroid sparing agent when in remission or if low severity

44
Q

What prophylaxis is require for patients on

cyclophosphamide

A

Pneumocystis jirovecii prophylaxis with

Cotrimoxazole

MSK (21 decks)

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