Vasculitis 11/10/18 Flashcards
What is vasculitis?
Inflammation of blood vessels, often with ischaemia, necrosis and organ inflammation
Where can vasculitis affect?
Any blood vessels Arteries Arterioles Veins Venules Capillaries
What is the mortality rate for untreated small vessel vasculitis?
90% after 2 years
What are the two types of vasculitis?
Primary
Secondary
What is primary vasculitis?
From inflammatory response that targets the vessel walls and has no known cause
Can be autoimmune
What is secondary vasculitis?
May be triggered by infection, a drug, or a toxin
May occur as part of another inflammatory disorder or cancer
Diagram on SLide 7
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What is vasculitis classified by?
What size of vessel is affected
What sizes of vessels can be affcted and what are examples?
Large vessel vasculitis -Takayasu arteritis -Giant cell arteritis Medium vessel vascullitis - Kawasaki disease Small vessel -ANCA associated -Immune complex
What systemic symptoms may be experienced with all types of vasculitis?
Fever
Malaise
Weight loss
Fatigue
What are the two main causes of large vessel vasculitis?
Takayasu arteritis
Giant Cell arteritis
Who gets TA?
<40
Females
Asian
Who gets GCA?
Over 50s
What vessels are affected in GCA?
Temporal
Aorta and other large vessels can be affected
What is seen in large vessel vasculitis?
Granulomatous infiltration of the walls of the large vessels
What are the presenting features in LVV?
Bruit (carotid)
Blood pressure difference in limbs
Claudication
What is Temporal arteritis associated with?
Polymyalgia rheumatica
What symptoms are seen in temporal arteritis?
Headache Scalp tenderness Jaw claudication Prominent temporal arteries Reduced Pulsation Risk of blindness
What investigations are done for Temporal arteritis?
ESR Plasma viscosity Temporal artery biopsy CRP MR angiogram PET CT
What is the management of TA/LVV?
40-60mg prednisolone
Steroid sparing agents
What are the two branches of small vessel vasculitis?
ANCA associated
ANCA negative
Who gets GPA?
Males
Northern european
35-55
What are the ENT features of GPA?
Sinusitis Nasal crusting Epistaxis Mouth ulcers Sensorineural deafness Otitis media Saddle nose
What are the Respiratory features of GPA?
Pulmonary infiltrates COugh Haemoptysis Diffuse alveolar haemorrhage Cavitating nodules
What are the cutaneous features of GPA?
Palpable purpura
CUtaneous ulcers
What are the renal features of GPA?
Necrotising glomerulonephritis
What are the nervous system features of GPA?
Mononeuritis multiplex
Sensorimotor polyneuropathy
Cranial nerve palsies
What are the ocular features of GPA?
Conjunctivitis Episcleritis Uveitis Optic nerve vasculitis Retinal artery occlusions Proptosis
What is the main difference between EGPA and GPA?
Late onset asthma
High eosinophil count
ACR criteria
Asthma Eosinophillia Paranasal sinusitis Pulmonary infiltrates Proof of vasculitis with eosinophils Polyneuropathy
Immunology
ANCAs
Immunoflourescence
What are the ocular features of GPA?
Conjunctivitis Episcleritis Uveitis Optic nerve vasculitis Retinal artery occlusions Proptosis
How is localised AAV treated?
Methotrexate + steroids
How is generalised AAV treated?
Steroids +
-Cyclophosphamise
Rituximab
Followed by
How is generalised AAV treated?
Steroids + -Cyclophosphamise Rituximab Followed by: Azathiprine
How is refractory AAV treated?
Iv immunoglobulins
Rituximab
How is refractory AAV treated?
Iv immunoglobulins
Rituximab
What is Henoch-Schonlein purpura?
An acute immunoglobulin A mediated disorder
What is the presentation of HSP?
Purpuric rash typically over buttocks and lower limbs Colicky abdo pain Bloody diarrhoea Joint pain Renal involvement Generalised vasculitis Small vessels of the skin Gi tract Kidneys Joints Lungs Children
What is HSP caused by?
Group A strep
How is HSP managed?
Self-limiting
Symptoms tend to resolve within 8 weeks
Relapse may occur
Essentiial to perform urinalysis to screen for renal involvement
