Vasculitis Flashcards
What is primary vasculitis?
results from an inflammatory response that targets the vessel walls and has no known cause
What is secondary vasculitis?
may be triggered by an infection, a drug, or a toxin and may occur as part of another inflammatory disorder or cancer
What is the aetiology of Takayasu Arteritis?
- Generally occurs < 40 years
- More common in females
- More prevalent in Asian populations
What is the presentation of Takayasu Arteritis?
- Early features include non specific features - low grade fever, malaise, night sweats, weight loss, arthralgia and fatigue
- Following this - claudication in upper and lower limbs
- If untreated, vascular stenosis and aneurysms can occur - results in bruit (most commonly carotid), reduced pulses, blood pressure difference of extremities
What are the investigations for Takayasu Arteritis?
- Bloods - raised inflammatory markers
- Imaging - MR angiogram can detect thickened vessel walls and stenosis
What is the aetiology of Giant cell arteritis?
- Most common cause of systemic vasculitis in adults
- Unknown aetiology
- Generally occurs > 50 years, most commonly late 60+
- Strong association with polymyalgia rheumatica
What is the presentation of giant cell arteritis?
- Symptoms:
- Unilateral acute temporal headache with focal tenderness on direct palpation
- Jaw claudication during chewing of firm foods or speaking (caused by ischaemia of the maxillary artery)
- Visual disturbances e.g. blurring
- Visual loss (amaurosis fugax)
- Constitutional manifestations e.g. fatigue, malaise and fever may also be present
- Signs:
- Tender enlarged non-pulsatile temporal arteries
- GCA should always be considered in the differential diagnosis of a new-onset headache in patients 50 years of age or older with an elevated erythrocyte sedimentation rate (ESR), CRP or plasma viscosity
What is the investigations for giant cell arteritis?
- Bloods - raised inflammatory markers
- Temporal artery USS (first line)
- Temporal artery biopsy (gold standard)
- Transmural inflammation of the intima, media, and adventitia of affected arteries
- Patchy infiltration by lymphocytes, macrophages, and multinucleated giant cells
- Vessel wall thickening can result in arterial luminal narrowing, resulting in subsequent distal ischemia
- A positive temporal artery biopsy has 100% specificity but relatively low sensitivity (15-40%) for the diagnosis of GCA due to the presence of skip lesions - biopsy may be taken from a normal segment
- PET CT or CT angiogram if other tests inconclusive/negative but high clinical suspicion remains
What is the management of giant cell arteritis and takayasu arteritis?
- Start at prednisolone 40-60mg daily
- TA - more long term, use steroid sparing agents if needed e.g. leflunomide, methotrexate
- GCA - gradual reduction in steroid dose over 18 months - 2 years
- If patient relapses in early stages of treatment - leflunomide, methotrexate
- Tocilizumab useful in resistant/relapsing GCA
What is granulomatosis with polyangiitis?
Granulomatous inflammation affecting small and medium sized vessels in the upper and lower respiratory tract, eyes and/or kidneys (necrotising glomerulonephritis common)
What is the aetiology of granulomatosis with polyangiitis?
- More common in northern Europeans
- Slightly higher incidence in males (1.5:1)
- Typically age 35-55 years
What is the presentation of granulomatosis with polyangiitis?
- Constitutional symptoms and arthralgia common
- ENT features: sinusitis, nasal crushing, epistaxis, mouth ulcers, sensorineural deafness, otitis media, ‘saddle nose’ (due to cartilage damage from ischaemia), subglottic inflammation
- Respiratory features: cough, haemoptysis, pulmonary infiltrates, diffuse alveolar haemorrhage, cavitating nodules on CXR
- Ocular features: conjunctivitis, episcleritis, uveitis, optic nerve vasculitis, retinal artery occlusion, proptosis
- Cutaneous features: palpable purpura, cutaneous ulcers
- Renal: necrotising glomerulonephritis
- Nervous system: mononeuritis multiplex, sensorimotor polyneuropathy, cranial nerve palsies
What is eosinophilic granulomatosis with polyangiitis?
Eosinophilic granulomatous inflammation affecting small and medium sized vessels most commonly in the respiratory tract and skin, but can also affect the renal, cardiovascular, gastrointestinal, central and peripheral nervous system
What is the presentation of eosinophilic granulomatosis with polyangiitis?
Many features similar to GPA, main difference is late onset asthma, high eosinophil count and ANCA specificity
What is microscopic polyangiitis?
Necrotising vasculitis of small vessels with few immune deposits, typically with pulmonary, renal and skin involvement
