Vasculitis Flashcards by Izzie Setford

What is vasculitis?

Inflammation of the blood vessels

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

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What are the secondary causes of vasculitis?

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

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What are the key examples of infection causing vasculitis?

Meningococcal septicaemia
Streptococcus
Hepatitis B
Hepatitis C

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How can a cold injury cause vasculitis?

Frost-bite

- can cause local vascular injury and tissue necrosis

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What is cryoglobulinaemia?

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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Which drugs can causes vasculitis?

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

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What are the general features of primary vasculitis?

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

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

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

Polyarteritis nodosa
Kawasaki disease (only in children)

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

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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What are the clinical features of giant cell arteritis?

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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What tests can be run to diagnose giant cell arteritis?

Very high ESR

Biopsy

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What is polymyalgia rheumatica?

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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What are the symptoms of polymyalgia rheumatica?

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

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Name a test to diagnose polymyalgia rheumatica.

High ESR

Improves with steroids

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What is Takayasu’s arteritis?

A rare, systemic, inflammatory large-vessel vasculitis

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

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What is the epidemiology of Takayasu’s arteritis?

Young (15-25yrs)

Women

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What are the clinical features of Takayasu’s arteritis?

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

How is Takayasu’s arteritis diagnosed?

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

What are the clinical features of Polyarteritis nodosa?

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

Which organs are typically affected by polyarteritis nodosa?

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

What is Kawasaki disease?

A disease of children and babies

Aneurysms forms in medium arteries

Which specific arteries does Kawasaki disease affect?

Coronary arteries - MI
Axillary arteries
Iliac arteries
Popliteal arteries

What are the early features of Kawasaki disease?

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

What is HSP?

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

What is the diagnostic triad of HSP?

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

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

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

What is ANCA positive vasculitis?

Anti-neutrophil cytoplasmic antibodie positive vasculitis | - cytoplasm of cells stain green on ANCA test

What are the two types of ANCA vasculitis?

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

Which blood vessels does granulomatous polyangiits normally affect?

``` Nose Sinuses Ears Lungs Kidneys Although other organs can be affected ```

What are the symptoms of granulomatous polyangiitis?

``` 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 ```

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

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

What are the common symptoms of eosinophilic granulomatous angiitis?

``` Asthma Eosinophilic infiltrates (transient pulmonary infiltrates) Eosinophilia - everywhere Eosinophil rich granulomata Peripheral neuropathy ```

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

``` Kidney glomerulonephritis Weight loss Skin lesions Peripheral neuropathy, mononeuritis multiplex Fevers ```

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

Temperature chart Height and weight ESR and CRP FBC

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

``` 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 ```

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

ANCA

What is the treatment for vasculitis?

Corticosteroids | Other immunosuppression

What is the most common vasculitis in children?

Henoch Schonlein Purpura

What are the symptoms associated with Henoch Schonlein Purpura?

Palpable purpura Abdominal pain Arthritis

Which organ requires the most monitoring in HSP?

Kidney | - BP and urinalysis

In which non-vasculitis conditions can ANCA be positive?

IBD SLE pANCE and MPO negative typically

What is the most common cause of vasculitis?

Infection