Vasculitis Flashcards
What is the definition of vasculitis?
Vessel wall inflammation
What is the pathophysiology of vasculitis?
Immune complex disease
Antibody dependent cellular cytotoxicity leads to endothelial activation and coagulopathy
Give 5 systemic symptoms of vasculitis
Malaise Fever Weight loss Myalgia Arthralgia Sweats Fatigue
Give 3 skin changes seen in vasculitis
Purpura
Ulceration
Infarction
Rash
Give 3 GI effects of vasculitis
Mouth ulcers
Abdo pain
Diarrhoea
Give 5 respiratory signs of vasculitis
Cough
Wheeze
Haemoptysis
Dyspnoea
Give 3 ENT signs of vasculitis
Epistaxis
Change of nose shape
Sinusitis
Deafness
What investigations would be done in someone where vasculitis is suspected?
Urinalysis ESR CRP WCC Biopsy Angiogram Organ specific tests
How is vasculitis managed?
Cyclophosphamide and high dose steroids to induce remission
To maintain remission, azathioprine, methotrexate, low dose steroids
Give 2 examples of large primary vasculitis
Takavasu’s arteritis
Giant cell arteritis
Polymyalgia rheumatica
Give 2 examples of medium primary vasculitis
Polyarteritis nodosa
Kawasaki disease
Isolated CNS vasculitis
Give 2 examples of small primary vasculitis
Wegeners granulomatosis (GPA)
EGPA
Microscopic polyangitis
Give 4 secondary causes of vasculitis
RA SLE Arthritis Malignancy Infection Drugs- cocaine
What common facial deformity is seen in Wegener’s granulomatosis (GPA)
Saddle nose deformity
What are the classic triad of systems involved in GPA?
Upper airway –> nasal obstruction, epistaxis, saddle nose
Lungs –> sinusitis, cough, chest pain, pleuritis, stridor
Kidneys –> glomerulonephritis, crescent formation, proteinuria
What investigations should be done if GPA is suspected?
ANCA (especially C-ANKA) FBC- may have reduced Hb Chest xray CT of sinuses Lung biopsy
Which vessels does Kawasaki’s disease mainly affect?
Medium vessels
Especially coronary vessels
Why are patients with Kawasaki’s disease more at risk of leucocytosis and thrombocytosis?
Endothelial cells damaged so tunica media exposed which increases risk of coagulation (virchow’s triad)
What antibody should be tested for in Kawasaki’s disease?
Anti-endothelial cell antibodies
Give some signs and symptoms of Kawasaki’s disease?
CRASH and BURN Conjunctivitis Rash Adenopathy Strawberry tongue Hands and feet redness and oedema
Burn = fever
What is the main cause of death in Kawasaki’s disease?
Myocardial Infarction
How is Kawasaki’s disease treated?
IV immunoglobulin
High dose aspirin (at risk of Reyes disease which is a rapidly progressing encephalopathy)
What is Takayasu’s arteritis also known as?
Pulselessness disease
Aortic arch syndrome
What vessels does Takayasu’s arteritis affect?
Large vessels
Give 5 signs/symptoms in Takayasu’s arteritis
Absent peripheral pulses Hypertension Heart failure Peripheral claudication Neurological symptoms Stroke
How would Takayasu’s be investigated?
Contrast Angiography
CT
MRI
(No autoantibodies associated)
How is Takayasu’s arteritis treated?
Corticosteroids
Surgical bipass
What type of vessels does polyalgia rheumatica effect?
Large vessels
What are the characteristic symptoms of polyalgia rheumatica?
Sudden onset pain and stiffness in shoulders and pelvis
Normal muscle strength
Worse in the morning and improves on exercise
Tiredness Fever Weight loss Depression Night sweats Loss of appetite
What investigations should be done in polyalgia rheumatica?
ESR/CRP FBC- normocytic anaemia LFTs - may have raised ALP Temporal artery biopsy CK Muscle biopsy
How is polyalgia rheumatica treated?
Corticosteroids
What are the symptoms of giant cell arteritis?
Headache- abrupt onset (over the temples) Scalp tenderness Jaw claudication Sudden loss of vision Malaise Tiredness Fever Thickened temporal arteries (plus symptoms of polymyalgia rheumatica)
How should giant cell arteritis be investigated?
Temporal artery biopsy FBC- normocytic anaemia ESR CRP LFTs
How is giant cell arteritis managed?
Early treatment
Corticosteroids
Osteoporosis prophylaxis
Methotrexate
What is Behcet’s disease?
Mainly seen in Turkey, Iran and Japan
Unknown cause
Systemic vasculitis
What are some of the signs and symptoms of Behcet’s disease?
Oral ulcers Genital ulcers Eye lesions Skin lesions Peripheral arthritis GI symptoms Leg thrombophlebitis
How is Behcet’s investigated?
Pethergy reaction- skin test
ESR and CRP raised
No specific autoantibodies
How is Behcet’s disease treated?
Corticosteroids Immunosuppressants Ciclosporin Colchicine Anti-TNFs (serious cases)
