Vasculitidies Flashcards
What is Giant Cell Arteritis? (temporal arteritis)
- Chronic vasculitis of large- and medium-sized vessels that occurs among individuals over 50 yr of age
- Median age of onset is 72.
- Most commonly causes inflammation of arteries originating from the arch of the aorta
- Around 50% have features of Polymyalgia Rheumatica: aching, morning stiffness in proximal limb muscles (not weakness)
What are the risk factors of Giant Cell Arteritis?
- Age is the greatest risk factor
- Common in white Genetic (HLA-DR4)
What is the histology of Giant Cell Arteritis?
Histology shows changes which characteristically ‘skips’ certain sections of affected artery whilst damaging others.
What can Temporal Arteritis lead to?
- Occlusive arteritis can result in anterior ischemic optic neuropathy (AION) and acute visual loss.
- Visual symptoms are an ophthalmic emergency
- Inflammation of arteries supplying the muscles of mastication results in jaw claudication and tongue discomfort
What are features of Temporal Arteritis?
- Headache (found in 70%)
- Often localized, unilateral, boring in quality over temple
- Jaw claudication and tongue claudication upon mastication
- Visual disturbances secondary to anterior ischemic optic neuropathy. Symptoms include:
- amaurosis fugax
- blindness, diplopia and blurring
- Tender, palpable temporal artery and may present with scalp tenderness
- Lethargy
- Depression
- Low-grade fever
- Anorexia
- Night sweats
What are criteria for diagnosis of Temporal Arteritis?
- Presence of any 2 or more of the following in patients >50 years with:
- Raised ESR, CRP or PV
- New onset of localized headache
- Tenderness or decreased pulsation of temporal artery
- New visual symptoms
- Biopsy revealing necrotizing arteritis
What is the treatment for Temporal Arteritis?
- Prednisolone 60 mg PO per day for at least 2 weeks before considering tapering down slowly if there are visual symptoms
- 40-60mg. (0.75/kg)
- Start if suspected before biopsy
- For acute onset visual symptoms
- Consider 1g methylprednisolone IV pulse therapy for the 1–3 days
- Give Aspirin
What is Polymyalgia Rheumatica?
- Clinical syndrome characterized by pain and stiffness to shoulder and hip girdles and neck.
- Primary impact elderly and associated with morning stiffness and elevated inflammatory markers
- Peaks between ages of 70-80 with usually rapid onset (e.g. < 1 month)
- Associated with Giant Cell Arteritis
What is the pathophysiology of Polymyalgia Rheumatica?
- Histology shows vasculitis with giant cells, characteristically ‘skips’ certain sections of affected artery whilst damaging others
- Muscle bed arteries affected most in polymyalgia rheumatica
What are symptoms of Polymyalgia Rheumatica?
- Mild polyarthralgia
- Lethargy
- Depression
- Low-grade fever
- Anorexia
- Night sweats and night pain
- Weight loss
- Difficulty rising from chair or combing hair
- Stiffness in neck shoulder and hip and pain in proximal limbs
What are examination findings of Polymyalgia Rheumatica?
- Decrease range of motion of shoulder, neck and hips
- Muscle stretch usually normal but muscle tenderness
What is the investigation for Polymyalgia Rheumatica?
- ESR > 40 mm/hr
- Note CK and EMG normal
- Reduced CD8+ T cells
What is the treatment for Polymyalgia Rheumatica?
- Prednisolone
- 15mg/od - dramatic response
- Methotrexate can be steroid sparing in relapsing patients
What is vasculitis?
- An inflammatory blood vessel disorder
- Vasculitis is a large, heterogeneous group of diseases classified by the predominant size, type, and location of involved blood vessels
What results in clinical features of Vasculitis?
Clinical features result from the damage of blood vessel walls with subsequent thrombosis, ischemia, bleeding, and/or aneurysm formation.
