Vasculitis Flashcards
(44 cards)
What is vasculitis
Inflammation of blood vessels
Prolonged inflammation can lead to thrombosis, ischemia, organ infarction necrosis and even death
Which blood vessels can vasculitis
ANY
Arteries, arterioles, veins, venules or capillaries
What causes secondary vasculitis
May be triggered by an infection, drugs, a toxin or may occur as part of another inflammatory disorder or cancer
How do you classify vasculitis
Based on which type of blood vessel is affected
Small, medium or large
How does vasculitis present
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
What does Giant cell arteritis typically cause
Temporal arteritis
May affect aorta and other large vessels
What is seen in blood vessel walls in vasculitis
Granuloma formation
What are the presenting features of large vessel vasculitis
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
What are the signs of temporal arteritis
Unilateral temporal headache
Scalp tenderness
Jaw claudication
Temporal arteries become prominent but with reduced pulsation
What is the major risk with temporal arteritis
Blindness
Due to ischaemia of the optic nerve
What investigations do you do for large vessel vasculitis
ESR, plasma viscosity and CRP - will be raised
Temporal artery biopsy - GOLD STANDARD
MR angiogram or PET CT
How is small vessel vasculitis divided
Into ANCA associated vasculitis (AAV) and ANCA negative
Describe the pathology of GPA
Granulomatous inflammation of respiratory tract, ENT system and small and medium vessels
Necrotising glomerulonephritis common
Describe the pathology of EGPA
Eosinophilic granulomatous inflammation of respiratory tract, ENT system, small and medium vessels.
Associated with asthma
Describe the pathology of MPA
Necrotising vasculitis with few immune deposits.
Necrotising glomerulonephritis very common
What are the ENT features of GPA
Sinusitis Nasal crusting Blocked nose Epistaxis Mouth ulcers Sensorineural deafness - bilateral Otitis media and deafness “Saddle nose” due to cartilage ischaemia and necrosis
What is epistaxis
Nose bleed
What are the cutaneous features of GPA and what causes them
Palpable purpura
Petichial rashes
Cutaneous ulcers
Caused by inflammation of the small blood vessels in the skin
What are the respiratory features of GPA
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
What is the key renal feature of GPA
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
What are the nervous system features of GPA
Mononeuritis multiplex - foot and wrist drops
Peripheral neuropathy - glove and stocking
Cranial nerve palsies
What are the ocular features of GPA
Conjunctivitis Episcleritis Scleritis - nodular Uveitis Optic nerve vasculitis Retinal artery occlusion Proptosis Retro orbital granulomas mimicking tumours
What are ANCAs
Anti-neutrophil cytoplasmic antibodies
Autoantibodies against the cytoplasm of neutrophil granulocytes
How can you detect ANCA
Immunofluorescence
Also allows you to determine the pattern and differentiate between C-ANCA and P-ANCA