Vasculitidies Flashcards

1
Q

What is Giant Cell Arteritis? (temporal arteritis)

A
  • 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)
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2
Q

What are the risk factors of Giant Cell Arteritis?

A
  • Age is the greatest risk factor
  • Common in white Genetic (HLA-DR4)
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3
Q

What is the histology of Giant Cell Arteritis?

A

Histology shows changes which characteristically ‘skips’ certain sections of affected artery whilst damaging others.

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4
Q

What can Temporal Arteritis lead to?

A
  • 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
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5
Q

What are features of Temporal Arteritis?

A
  • 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
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6
Q

What are criteria for diagnosis of Temporal Arteritis?

A
  • 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
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7
Q

What is the treatment for Temporal Arteritis?

A
  • 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
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8
Q

What is Polymyalgia Rheumatica?

A
  • 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
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9
Q

What is the pathophysiology of Polymyalgia Rheumatica?

A
  • Histology shows vasculitis with giant cells, characteristically ‘skips’ certain sections of affected artery whilst damaging others
  • Muscle bed arteries affected most in polymyalgia rheumatica
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10
Q

What are symptoms of Polymyalgia Rheumatica?

A
  • 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
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11
Q

What are examination findings of Polymyalgia Rheumatica?

A
  • Decrease range of motion of shoulder, neck and hips
  • Muscle stretch usually normal but muscle tenderness
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12
Q

What is the investigation for Polymyalgia Rheumatica?

A
  • ESR > 40 mm/hr
  • Note CK and EMG normal
  • Reduced CD8+ T cells
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13
Q

What is the treatment for Polymyalgia Rheumatica?

A
  • Prednisolone
    • 15mg/od - dramatic response
  • Methotrexate can be steroid sparing in relapsing patients
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14
Q

What is vasculitis?

A
  • 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
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15
Q

What results in clinical features of Vasculitis?

A

Clinical features result from the damage of blood vessel walls with subsequent thrombosis, ischemia, bleeding, and/or aneurysm formation.

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16
Q

What is found on physical examination of Vasculitis?

A
  • Vital signs: blood pressure (hypertension) and pulse (regularity and rate)
  • Skin: Palpable purpura, Livedo reticularis, nodules, digital ulcers, gangrene, nail bed capillary changes
  • Neurologic: cranial nerve exam, sensorimotor exam • Ocular exam: visual fields, scleritis, uveitis episcleritis
  • Cardiopulmonary exam: Crackles, pleural rubs murmurs, arrhythmias
  • Abdominal exam: tenderness, organomegaly
17
Q

What investigations are done for Vasculitis?

A
  • Renal involvement is often clinically silent.
    • Urine dipstick +/- microscopy are needed to identify underlying glomerulonephritis.
  • Initial tests exclude alternate diagnoses and guide therapy
    • FBC, U&Es, LFTs, CRP, PV, ESR,
    • Specific serology: ANA, ANCA, RF
    • Complement levels C3, C4
    • Hepatitis screen for B and C
    • HIV Cryoglobulins
    • Serum and urine protein electrophoresis
    • Miscellaneous: CK, Blood cultures, ECG
    • CXR, CT scan, MRI, arteriography and CT-PET may be required to delineate extent of organ involvement.
18
Q

What is the treatment for Vasculitis?

A
  • 1st line: Corticosteroids
  • 2nd Line: Cytotoxic medications, immunomodulatory, or biologic agents
    • Cyclophosphamide, Methotrexate, Azathioprine, Leflunomide, Mycophenolate mofetil, Rituximab, IVIG