Vasculitis Flashcards

1
Q

What is vasculitis?

A

Inflammation of the blood vessels

- a response to an insult with many causes (primary and secondary)

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2
Q

What are the secondary causes of vasculitis?

A
Infection
Underlying disease
- cancer
- autoimmune disease such as rheumatoid arthritis, SLE, or IBD
Cold injury
Drugs
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3
Q

What are the key examples of infection causing vasculitis?

A

Meningococcal septicaemia
Streptococcus
Hepatitis B
Hepatitis C

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4
Q

How can a cold injury cause vasculitis?

A

Frost-bite

- can cause local vascular injury and tissue necrosis

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5
Q

What is cryoglobulinaemia?

A

The cold causes autoantibodies to be activated, and then attack, clump and destroy red blood cells.
This blocks and irriates the vessels (vasculitis)
The peripheral (coldest parts of the body) are affected worst.
Blockage of the blood vessels causes gangrene

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6
Q

Which drugs can causes vasculitis?

A
Anti-thyroid drugs (carbimazole)
Minocyclin
Hydralazine
Penicillamine 
Antibiotics (penicillin/sulphonamides)
Anticonvulsants
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7
Q

What are the general features of primary vasculitis?

A
Systemic inflammation
- fever, malasie, weight loss, night sweats, algia (muscles/joints)
Large vessels affected
- end organ ischaemia or infarction 
Medium vessels affected
- localised ischaemia or infarction
Small vessels affected
- organ specific inflammation
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8
Q

What are the two types of large vessel involvement in vasculitis?

A

Giant cell arteritis
- very common in the elderly
Takayasu
- very rare

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9
Q

What are the two types of medium vessel involvement in vasculitis?

A
Polyarteritis nodosa
Kawasaki disease (only in children)
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10
Q

What are the two types of small vessel involvement in vasculitis?

A
Immune complex mediated vasculitis
- HSP - most common type of vasculitis 
- cryoglobulinaemia
ANCA associated vasculitis
- microscopic polyangiitis
- granulomatosis with polyangiitis
- eosinophilic granulomatosis with polyangiitis
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11
Q

What are the clinical features of giant cell arteritis?

A
Systemic features
Fatigue 
Headaches
Jaw claudication
Scalp tenderness
Confusion
Stroke
Sensorineural deafness
Depression
Depends on the artery affected
- ophthalmic artery (sudden painless onset blindness)
- lingual artery (tingling in unilateral tongue)
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12
Q

What tests can be run to diagnose giant cell arteritis?

A

Very high ESR

Biopsy

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13
Q

What is polymyalgia rheumatica?

A

Considered to be a related disorder to giant cell arteritis.

A syndrome of myalgia, typically in the neck, shoulders, upper arms and hips

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14
Q

What are the symptoms of polymyalgia rheumatica?

A

Pain and stiffness in the muscles of the neck, shoulder girdle and pelvic girdle
(proximal muscles - rheumatological pattern)
No weakness

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15
Q

Name a test to diagnose polymyalgia rheumatica.

A

High ESR

Improves with steroids

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16
Q

What is Takayasu’s arteritis?

A

A rare, systemic, inflammatory large-vessel vasculitis

- granulomatous inflammation of the aorta and it’s major branches

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17
Q

What is the epidemiology of Takayasu’s arteritis?

A

Young (15-25yrs)

Women

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18
Q

What are the clinical features of Takayasu’s arteritis?

A
Systemic features (early)
Vascular insufficiency features (late disease)
- bruitis
- absent, reduced pulses
- claudication
- ischaemic heart disease, heart failure or pulmonary hypertension
- headaches
- sudden, painless blindness
- BP variable
19
Q

How is Takayasu’s arteritis diagnosed?

A

Imaging angiography
MR angiography
CT scan
Shows narrowing of the aorta and larger arteries adjacent to the aorta

20
Q

What are the clinical features of Polyarteritis nodosa?

A
Medium sized vessel vasculitis
- systemic features
- malasise
- myalgia/arthralgia
- organ infarction 
E.g. Kidney
- renal artery stenosis 
- renal aneurysms
- hypertension
21
Q

Which organs are typically affected by polyarteritis nodosa?

A
Gut
Brain - stroke
Heart - MI/angina 
Liver
Skin - gangrene, purpura, nodules
PNS - pain over dermatome, loss of sensory or motor function
Testis - pain or infarct
Limbs - claudication 
Kidney
22
Q

What is Kawasaki disease?

A

A disease of children and babies

Aneurysms forms in medium arteries

23
Q

Which specific arteries does Kawasaki disease affect?

A

Coronary arteries - MI
Axillary arteries
Iliac arteries
Popliteal arteries

24
Q

What are the early features of Kawasaki disease?

A
High fever
Arthritis
Myocarditis
Red palms/soles
Desqaumation/Rash 
Straeberry tongue 
Cervical lymphadenopathy
Thrombocytosis
Miserable 
Mucositis
Conjunctivitis
25
Q

What is HSP?

A

The most common cause of immune complex small vessel vasculitis in childhood
Possibly has an infective cause
IgA deposits in the gut, skin and synovial lesions

26
Q

What is the diagnostic triad of HSP?

A

Palpable purpura
- covers the extensor surfaces, starting peripherally
Abdominal pain
Arthritis

27
Q

How do IgA deposits affect the gut, kidney, joints and skin?

A
Joints
- pattern of joint involvement matches the skin
- starts peripherally and works proximally 
Kidney
- nephritic syndrome
Skin
- palpable purpura
Gut
- causes abdominal pain
28
Q

What is ANCA positive vasculitis?

A

Anti-neutrophil cytoplasmic antibodie positive vasculitis

- cytoplasm of cells stain green on ANCA test

29
Q

What are the two types of ANCA vasculitis?

A
cANCA
- specific for granulomatous polyangiits
pANCA (MPO positive)
- microscopic polyangiitis
- crescentic glomerulonephritis
- eosinophilic granulomatous polyangiitis
pANCA (MPO negative)
- IBD
- RA
- Sclerosing cholangitis
30
Q

Which blood vessels does granulomatous polyangiits normally affect?

A
Nose
Sinuses
Ears
Lungs
Kidneys
Although other organs can be affected
31
Q

What are the symptoms of granulomatous polyangiitis?

A
Systemic symptoms
Malaise 
Joint pain
Persistent ear infections and reduced hearing 
Sinusitis 
Nosebleeds
Crusts around the nose
Chest pain, cough and SOB
Coughing up blood
Blood in the urine
32
Q

What are the most commonly affected blood vessels in eosinophilic granulomatous angiitis?

A
Lungs and skin are most common
Can affect all other organs
- heart
- kidneys
- nerves
- bowel
33
Q

What are the common symptoms of eosinophilic granulomatous angiitis?

A
Asthma
Eosinophilic infiltrates (transient pulmonary infiltrates) 
Eosinophilia - everywhere
Eosinophil rich granulomata
Peripheral neuropathy
34
Q

What are the five most common clinical manifestations of microscopic polyangiitis?

A
Kidney glomerulonephritis 
Weight loss
Skin lesions
Peripheral neuropathy, mononeuritis multiplex
Fevers
35
Q

What tests can you do to assess a patient for inflammation?

A

Temperature chart
Height and weight
ESR and CRP
FBC

36
Q

What vessel specific tests should you run on a person with suspected vasculitis?

A
Blood pressure in all four limbs 
Examine the vascular tress
- pulses
- feel for aneurysms
Medium and large angiography/MRA
Small - organ specific tests
- US abdomen, MRI gut, endoscopy, biopsy
- BP urianlysis, US kidneys, perfusion scan, biopsy
- echo, ECG and muscle enzymes
- US liver, LFTs, biopsy
- image the CNS, LP
- nerve conduction studies 
CXR, CT chest, pulmonary function tests, biopsy
- skin biopsy
37
Q

What disease specific tests should you run on a person with suspected vasculitis?

A

ANCA

38
Q

What is the treatment for vasculitis?

A

Corticosteroids

Other immunosuppression

39
Q

What is the most common vasculitis in children?

A

Henoch Schonlein Purpura

40
Q

What are the symptoms associated with Henoch Schonlein Purpura?

A

Palpable purpura
Abdominal pain
Arthritis

41
Q

Which organ requires the most monitoring in HSP?

A

Kidney

- BP and urinalysis

42
Q

In which non-vasculitis conditions can ANCA be positive?

A

IBD
SLE
pANCE and MPO negative typically

43
Q

What is the most common cause of vasculitis?

A

Infection