VASCULITIS Flashcards

1
Q

What is vasculitis?

A
  • a histological term describing inflammation of the vessel wall
  • can be seen in many diseases including; RA, SLE, polymyositis, and some allergic drug reactions
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2
Q

Describe the pathophysiology of vasculitis

A

Inflammation and necrosis of blood vessel walls with subsequent impaired blood flow resulting in:

  • Vessel wall destruction - aneurysm, rupture and stenosis—> perforation and haemorrhage into tissues
  • Endothelial injury:–>thrombosis + ischaemia/infarction of dependent tissues
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3
Q

How is vasculitis classified?

A

-By size of blood vessel involved and the presence or absence of antineutrophil cytoplasmic antibodies (ANCA)

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4
Q

Give examples of large-vessel vasculitis

A

Refers to the aorta and its major tributaries

Examples:

  • Giant cell arteritis/polymyalgia rheumatic
  • Takayasu’s arteritis
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5
Q

Give examples of medium-vessel vasculitis

A

Refers to medium and small-sized arteries and arterioles

Examples:

  • Classical polyarteritis nodosa (PAN)
  • Kawasaki’s disease
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6
Q

Give examples of small-vessel vasculitis

A

Refers to small arteries, arterioles, VENULES and capillaries

Examples: 
>ANCA-associated: 
-Microscopic polyangitis 
-Granulomatosis with polyangitis
>ANCA-negative: 
-Essential cryoglobulinaemia 
-Cutaneous leucocytoclastic vasculitis
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7
Q

What size vasculitis tends to be ANCA positive?

A

Small-vessel vasculitis e.g microscopic polyangitis and granulomatosis with polyangitis

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8
Q

What are all vasculitis associated with?

A

-All associated with anaemia and a raised ESR

pAre ALL RARE except giant cell (temporal) arteritis

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9
Q

What is PMR?

A

Systemic disease of the elderly, the pathogenesis is unknown (Large cell vasculitis)

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10
Q

What are the risk factors of PMR

A
  • SLE
  • Polymyositis/dermatomyositis
  • age>50
  • female
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11
Q

What is the epidemiology of PMR?

A
  • Affects those over 50 yrs

- More common in FEMALES than males

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12
Q

What are the signs/symptoms of PMR?

A

S-udden onset of severe pain and stiffness of the shoulders and neck, and of the hips and lumbar spine; a limb girdle pattern

  • worse in the morning, lasting from 30 mins - several hours
  • Mild polyarthritis of peripheral joints
  • 1/3rd experience; fatigue, fever, weight loss, depression
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13
Q

What are the investigations for PMR?

A
  • Clinical history is usually diagnostic and the patient is ALWAYS OVER 50
  • Bloods; ESR & CRP raised, ANCA negative, ALP raised, mild normochromic, normocytic anaemia may be present
  • Temporal artery biopsy: Shows giant cell arteritis in 10-30% cases
  • Note: creatinine kinase is normal - helps to distinguish from myositis/ myopathies
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14
Q

What is the treatment and management of PMR?

A
  • Corticosteroids; produce a dramatic reduction of symptoms of PMR within 24-48 hours of starting treatment e.g. ORAL PREDNISOLONE
  • If improvement does not occur then diagnosis should be questioned
  • Decrease dose slowly
  • Used long-term so give GI and bone protection (to prevent osteoporosis due to steroid use) e.g. LANSOPRAZOLE and ALENDRONATE and Ca2+ and vitamin D
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15
Q

What is GCA?

A

Inflammatory granulomatous arteritis of large Cerebral Arteries as well as other large vessels e.g aorta, which occurs in association with PMR (Large cell vasculitis)

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16
Q

Describe the pathophysiology of GCA?

A
  • Arteries become inflamed, thickened and can obstruct blood flow
  • Cerebral arteries affected in particular e.g. temporal artery
  • Opthalmic artery can also be affected potentially resulting in permanent or temporary vision loss
17
Q

What are the risk factors of GCA?

A
  • Over 50
  • Female
  • RA, SLE, scleroderma
18
Q

Describe the epidemiology of GCA

A
  • Primarily in those OVER 50
  • Incidence increases with age
  • More common in FEMALES than males
19
Q

What are the signs of GCA?

A
  • Tenderness and swelling of one or more temporal or occipital arteries
  • SUDDEN PAINLESS VISION LOSS - EMERGENCY - Arteritic anterior ischaemic optic neuropathy - optic disc is very pale/swollen
20
Q

What are the symptoms of GCA?

A
  • Severe headaches (temporal pulsating)
  • Tenderness of scalp (combing hair can be painful) or temple
  • Claudication of the jaw (pain in jaw) when eating
  • Malaise, lethargy, fever
21
Q

WHat are the investigations for GCA?

A
  • Temporal artery biopsy; DEFINITIVE DIAGNOSTIC TEST - Should be taken BEFORE or within 7 days of starting high dose corticosteroids
  • Bloods; CRP&ESR high, ANCA negative, ALP may be raised, Normochromic, normocytic anaemia
22
Q

What is the diagnostic criteria of GCA?

A
  • Over 50
  • New headache
  • Temporal artery tenderness or decreased pulsation
  • ESR raised
  • Abnormal artery biopsy - inflammatory infiltrates present
23
Q

What is the treatment of GCA?

A
  • HIGH DOSE CORTICOSTEROIDS RAPIDLY e.g. PREDNISOLONE to stop vision loss - gradual reduction of steroids over 12-18 months
  • Used long-term so give GI and bone protection (to prevent osteoporosis due to steroid use) e.g. LANSOPRAZOLE and ALENDRONATE and Ca2+ and vitamin D
  • Monitor treatment progress by looking at ESR/CRP (should fall)
24
Q

What is polyarteritis nodosa(PAN)?

A
  • Necrotising vasculitis that causes aneurysms and thrombosis in medium sized arteries, leading to infarction in affected organs
  • Has severe systemic symptoms
25
Q

What are the risk factors for PAN?

A
  • Male
  • Hep B
  • RA, SLE, scleroderma
26
Q

What is the epidemiology of PAN?

A
  • Rare in the UK
  • Usual occurs in middle-aged men
  • More common in MALES than females
  • Associated with Hep B
27
Q

What are the initial symptoms of PAN?

A

Fever, malaise, weight loss and myalgia

these initial symptoms are followed by dramatic acute features that are due to organ infarction

28
Q

What are neurological symtoms of PAN?

A

-Mononeuritis multiplex due to arteritis of the vasa nervorum - Numbness, tingling, abnormal/lack of sensation and inability to move part of the body

29
Q

What are the abdominal symptoms of PAN?

A
  • Pain due to arterial involvement of the abdominal viscera, mimicking acute cholecystitis, pancreatitis or appendicitis
  • GI haemorrhage due to mucosal ulceration
30
Q

What are the renal symptoms of PAN?

A
  • Presentation is with haematuria and proteinuria

- Hypertension and acute/chronic kidney disease occur

31
Q

What are the cardiac and skin symptoms of PAN?

A

Cardiac:
Coronary arteritis causes myocardial infarction and heart failure

Skin:
Subcutaneous haemorrhage and gangrene occur

32
Q

What are the investigations for PAN?

A
  • Bloods; Anaemia, WCC raised, ECR raised, ANCA negative
  • Biopsy, of kidney in particular to look for hypertension and other damage - can be diagnostic
  • Angiography - Demonstrates micro-aneurysms in heaptic, intestinal or renal vessels
33
Q

What is the treatment of PAN?

A
  • CONTROL BLOOD PRESSURE - ACE-inhibitors e.g. RAMIPRIL
  • Corticosteroids e.g. PREDNISOLONE in combination with immunosuppressive drugs e.g. AZATHIOPRINE or CYCLOPHOSPHAMIDE
  • Hep B should be treated with an antiviral after initial treatment with steroids