Vasculitis Flashcards

1
Q

What are primary vasculitides?

A

A group of autoimmune conditions, characterised by inflammation of the blood vessels

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2
Q

Blood vessels in any organ can be affected by primary vasculitides, but particularly. vessels in which organs are affected?

A
Skin
Kidneys
Lungs
Joints
Nerves 
ENT
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3
Q

Give examples of large vessel vasculitis?

A

Takaysu arteritis

Giant cell arteritis

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4
Q

Give examples of medium vessel vasculitis?

A

Polyarteritis nodosa

Kawasaki disease

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5
Q

Give examples of ANCA-associated small vessel vasculitis?

A

Microscopic polyangitis
Granulomatosis with. polyangitis
Eosinophilic granulomatosis with polyangitis

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6
Q

Give. examples of immune complex. small vessel vasculitis?

A

Cryoglobulinaemic vasculitis
IgA vasclitis
Hypocomplementemic urticarial vasculitis

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7
Q

Which vessels are affected. by giant cell arteritis?

A

Aorta and its major branches

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8
Q

What is the peak age of. incidence of giant cell arteritis?

A

70-79 years

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9
Q

In which groups is giant cell arteritis most common?

A

Women

Caucasian

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10
Q

What are the symptoms of giant cell arteritis?

A

Headache - temporal headache with tenderness, subacute onset, constant and little relief with analgesics
Visual symptoms
Jaw claudication - pain on chewing can lead to weight. loss
Polymyalgia rheumatic symptoms
Constitutional upset

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11
Q

How can giant cell arteritis lead to blindness?

A

Causes ischaemic optic neuropathy due to loss of blood flow in the posterior ciliary. arteries

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12
Q

What. are the clinical examination findings of giant cell arteritis?

A

Temporal artery. symmetry
Thickening
Loss of pulsatility

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13
Q

What is the gold standard test for giant cell arteritis?

A

Temporal artery biopsy

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14
Q

What is a positive. finding in a temporal artery biopsy. for. giant cell. arteritis?

A

Interruption of the internal elastic laminae with mononuclear inflammatory cell infiltrate within the vessel wall

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15
Q

Absence of positive findings on temporal artery biopsy rules out giant cell arteritis. T/F?

A

True

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16
Q

Other than temporal artery biopsies how can giant cell arteritis be investigated?

A

Temporal artery US, MRI and PET CT

17
Q

How is giant. cell arteritis treated?

A

1mg/kg/day of prednisolone up to a maximum 60mg - should be maintained for 1. month then tapering to 15mg by 12. weeks. The aim is the discontinue corticosteroids by 12-18 months.
IV methylprednisolone can be used if. visual symptoms are present
Corticosteroid sparing therapies - methotrexate, tocilizumab, mycophenolate
75mg aspirin daily to reduce ischaemic complications

18
Q

What are. the differential diagnoses of IgA vasculitis?

A

Idiopathic
Drugs
Infection: HCV, HBV, gonococcus, meingococcus, staph
Secondary RA
Malignancy
Manifestation of small/medium vessel ANCA associated vasculitis

19
Q

In what age group is IgA vasculitis most common?

A

Children aged 2-11 years

20
Q

IgA vasculitis is usually a self-limiting illness. T/F?

A

True

21
Q

What are the signs of IgA vasculitis?

A

purpuric rash on the buttocks, thighs and occasionally lower legs
Urticarial rash, confluent petechiae, ecchymosis, ulcers
Arthralgia and arthritis in the lower limb

22
Q

What are the potential complications of. IgA vasculitis?

A

GI. pain, bleeding, diarrhoea, intussusception
IgA nephropathy
Orchitis
CNS signs

23
Q

How can the extent of involvement of IgA vasculitis be assessed?

A

Urinalysis

Urine PCR

24
Q

Often no treatment is required for IgA vasculitis. T/F?

A

True

25
Q

When can corticosteroids be used in the treatment of IgA. vasculitis?

A

Testicular torsion
GI. disease
Arthritis

26
Q

What type of vasuculitis has the most associated morbidity and mortality?

A

ANCA Associated vasculitis

27
Q

What cells are ANCA autoantibodies directed against?

A

Neutrophils

Monocytes

28
Q

Why is detection of ANCA autoantibodies helpful in vasculitis?

A

Diagnosis
Assessing response to treatment
Monitoring for early signs of relapse
Gives prognostic information

29
Q

cANCA with PR3. is very suggestive of…?

A

Granulomatosis with polyangiitis

30
Q

pANCA with strong mPO is suggestive of

A

Microscopic polyangiitis

31
Q

What treatments can be used to induce remission of ANCA associated vasculitis?

A

prednisolone with Oral or IV cyclophosphamide or rituximab in severe disease

Prednisolone with methotrexate. or mycophenolate in moderate. disease

32
Q

What risks are associated with the use of cyclophosphamide in ANCA associated vasculitis?

A

Infection
Cytopaenia
Malignancy
Infertility

33
Q

What is the mechanism of action of rituximab?

A

Anti-B cell biologic

34
Q

What drugs are used in maintenance of remission of ANCA associated vasculitis?

A

Azathioprine
Methotrexate
Mycophenolate

35
Q

What characterises granulomatosis with polyangiitis?

A

Granulomatous necrotising ifnlammatory lesions of the upper and lower respiratory tract and often paucity-immune glomerulonephritis

36
Q

Describe the classic triad of disease seen in granulomatosis with polyangiitis?

A

Upper airway / ENT - rhinitis, chronic sinusitis, chronic. otitis media, saddle nose deformity, nasal/septal perforation
Renal - rapidly progressive pouch-immune glomerulonephritis
Lower respiratory - parenchymal nodules +/- cavitation, alveolar haemorrhage

37
Q

What constitutional symptoms are there in granulomatosis with polyangiitis?

A
Fatigue
Weight loss
Fever/sweats
Myalgia/arthralgia
Failure to thrive in the elderly