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MBChB YR2 MSK > Disease Profiles: Vasculitis > Flashcards

Disease Profiles: Vasculitis Flashcards

1
Q

Define early systemic ANCA-associated small vessel vasculitis

A

Any systemic involvement, no organs affected and not life-threatening

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2
Q

Describe the prognosis of Henoch-Schonlein Purpura (HSP)

A

Usually self-limiting within 8 weeks, may be relapses

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3
Q

Which form of imaging would you use to investigate Takayasu arteritis?

A

MR angiogram

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4
Q

What patient group is most likely to develop Henoch-Schonlein Purpura (HSP)?

A

Children 2-11, following a preceding infection (1-3 weeks)

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5
Q

Why must urinalysis be performed in Henoch-Schonlein Purpura (HSP)?

A

To check for renal involvement

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6
Q

Which form of vasculitis should always be considered in the differential diagnosis of a new-onset headache in patients 50+ with elevated ESR, CRP or PV?

A

Giant cell arteritis

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7
Q

Why does a positive temporal artery biopsy for giant cell arteritis have 100% specificity but relatively low sensitivity?

A

Due to the presence of skip lesions - biopsy may be taken from a normal segment

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8
Q

What blood values would you expect to be increased in ANCA-associated small vessel vasculitis?

A

ESR, PV and CRP (+ deranged U+E if renal involvement)

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9
Q

What causes ‘saddle nose’ in granulomatosis with polyangiitis?

A

Cartilage damage from ischaemia

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10
Q

Describe the systemic symptoms common to all vasculitides

A

Fever, malaise, weight loss, fatigue

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11
Q

How would you manage a patient with Takayasu arteritis?

A

Start with prednisolone 40-60mg daily, use steriod sparing agents if needed e.g. leflunamide, methotrexate

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12
Q

Which renal complication is common in granulomatosis with polyangiitis and microscopic polyangiitis?

A

Necrotising glomerulonephritis

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13
Q

What blood values would you expect to be raised in polymyalgia rheumatica?

A

ESR, plasma viscosity, CRP

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14
Q

What would a temporal biopsy of a patient with giant cell arteritis show?

A

Transmural arterial inflammation, patchy infiltration by lymphocytes, macrophages and multinucleated giant cells, vessel wall thickening which may cause distal ischaemia

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15
Q

Which patient group is most likely to develop giant cell arteritis?

A

Patients in late 60s+

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16
Q

What is granulomatosis with polyangiitis?

A

Granulomatous inflammation affecting small and medium sized vessels in the upper and lower respiratory tract, eyes and/or kidneys (necrotising glomerulonephritis common)

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17
Q

Which ANCA-associated small vessel vasculitis is associated with MPO-ANCA/pANCA in ∼70% of cases?

A

Microscopic polyangiitis

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18
Q

Which patient group is most likely to develop polymyalgia rheumatica?

A

> 50 years, higher incidence in northern regions, GCA association

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19
Q

What are anti-neutrophil cytoplasmic antibodies (ANCAs)?

A

Auto-antibodies against antigens in the cytoplasm of neutrophil granulocytes

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20
Q

What causes bruit, reduced pulses and blood pressure difference of extremities in Takayasu arteritis?

A

Vascular stenosis and aneurysms

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21
Q

Describe the ocular manifestations of granulomatosis with polyangiitis

A

Conjunctivitis, episcleritis, uveitis, optic nerve vasculitis, retinal artery occlusion, proptosis

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22
Q

What is the first line imaging investigation for giant cell arteritis?

A

Temporal artery USS

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23
Q

What is the treatment for refractory ANCA-associated small vessel vasculitis?

A

IV immunoglobins

Rituximab

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24
Q

Which vasculotide is polymyalgia rheumatica often associated with?

A

Giant cell arteritis

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25
Q

Name the two categories of large vessel vasculitis

A

Temporal (giant cell) arteritis and Takayasu arteritis

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26
Q

What is the first line treatment and subsequent maintenance therapy for systemic ANCA-associated small vessel vasculitis?

A

Cyclophosphamide and steroids, followed by azathioprine for maintenance

Plasma exchange if creatinine > 500

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27
Q

What are the main differences between eosinophilic granulomatosis with polyangitis and microscopic polyangiitis?

A

Late onset asthma, high eosinophil count and ANCA specificity

28
Q

Which blood values would you expect to be elevated in Takayasu arteritis or GCA?

A

ESR, PV and CRP

29
Q

Name the ANCA-associated small vessel vasculitides

A

Granulomatosis with polyangiitis, eosinophilic granulomatosis with polyangitis, microscopic polyangiitis

30
Q

What is microscopic polyangiitis?

A

Necrotising vasculitis of small vessels with few immune deposits, typically with pulmonary, renal and skin involvement

31
Q

Describe the articular manifestations of polymyalgia rheumatica

A

Proximal myalgia of the hip and shoulder girdles with accompanying morning stiffness that lasts for more than 1 hour

Stiffness improves with movement

Usually symmetrical

Movement reduced but muscle strength normal

32
Q

Which ANCA-associated small vessel vasculitis is associated with PR3-ANCA/c-ANCA in ∼90% of cases

A

Granulomatosis with polyangiitis

33
Q

Define vasculitis

A

Inflammation of the blood vessels (arteries, arterioles, veins, venues, capillaries)

34
Q

What is secondary vasculitis?

A

Vasculitis triggered by e.g. an infection, drug or toxin, may occur as part of another inflammatory disorder or cancer

35
Q

Describe the ENT manifestations of granulomatosis with polyangiitis

A

Sinusitis, nasal crushing, epistaxis, mouth ulcers, sensorineural deafness, otitis media and deafness, ‘saddle nose’, subglottic inflammation

36
Q

Define localised ANCA-associated small vessel vasculitis

A

Upper and/or lower respiratory tract disease without any other systemic involvement or constitutional symptoms

37
Q

What is large vessel vasculitis?

A

Primary vasculitis that causes chronic granulomatous inflammation predominantly of the aorta and its major branches

38
Q

Which ANCA-associated small vessel vasculitis is associated with MPO-ANCA/pANCA in ∼40% of cases?

A

Eosinophilic granulomatosis with polyangitis

39
Q

What is the first line treatment and subsequent maintenance therapy for generalised ANCA-associated small vessel vasculitis?

A

Cyclophosphamide and steroids, followed by azathioprine for maintenance

40
Q

Define systemic ANCA-associated small vessel vasculitis

A

Renal (creatinine < 500) or other vital organ failure

41
Q

What does anaemia indicate in ANCA-associated small vessel vasculitis?

A

Chronic disease

42
Q

Which form of vasculitis presents with systemic symptoms, claudication of the upper and lower limbs, and can progress to bruit and reduced pulses?

A

Takayasu arteritis

43
Q

Describe the management of polymyalgia rheumatica

A

Start at prednisolone 15mg daily, reduce dose over 18 months - 2 years

44
Q

What would an MR angiogram show in Takayasu arteritis?

A

Thickened vessel walls and stenosis

45
Q

What is Henoch-Schonlein Purpura (HSP)?

A

IgA mediated generalised vasculitis (not ANCA associated) involving small vessels of the skin, GI tract, kidneys, joints, rarely the lungs and CNS

46
Q

What is the first line treatment for early systemic ANCA-associated small vessel vasculitis?

A

Methotrexate and steriods

47
Q

Define refractory ANCA-associated small vessel vasculitis

A

Progressive disease unresponsive to steroids + cyclophosphamide

48
Q

What is primary vasculitis?

A

Vasculitis that results from an inflammatory response that targets the vessel walls and has no known cause

49
Q

Which form of vasculitis presents with a unilateral acute temporal headache with focal tenderness on direct palpation, jaw claudication, visual disturbances/loss and tender enlarged non-pulsatile temporal arteries? (+ systemic symptoms)

A

Giant cell arteritis

50
Q

Describe the systemic manifestations of polymyalgia rheumatica

A

Fatigue, anorexia, weight loss, fever

51
Q

Describe the cutaneous manifestations of granulomatosis with polyangiitis and eosinophilic granulomatosis with poluangitis

A

Palpable purpura, oral/cutanous ulcers

52
Q

What is the first line treatment for localised ANCA-associated small vessel vasculitis?

A

Methotrexate + steroids

53
Q

How would you manage a patient with giant cell arteritis?

A

Start with prednisolone 40-60mg daily, gradual reduction over 18 months-2 years

If early relapse - leflunamide, methotrexate

If resistant/relapsing - toclizumab

54
Q

Define generalised ANCA-associated small vessel vasculitis

A

Renal (creatinine < 500) or other organ threatening

55
Q

What does decreased complement (C3+C4) indicate in ANCA-associated small vessel vasculitis?

A

Active disease

56
Q

Describe the respiratory manifestations of granulomatosis with polyangiitis

A

Cough, haemoptysis, pulmonary infiltrates, diffuse alveolar haemorrhage, cavitating nodules on CXR

57
Q

What is the diagnostic test for ANCA-associated small vessel vasculitis?

A

Biopsy

58
Q

Which organism is most commonly associated with the infections that usually precede Henoch-Schonlein Purpura (HSP)?

A

Group A Strep (GAS) - URT, pharyngeal, GI

59
Q

What is the diagnostic investigation for giant cell arteritis?

A

Temporal artery biopsy

60
Q

What is polymyalgia rheumatica?

A

Relatively common chronic inflammatory condition of unknown aetiology that affects elderly individuals

61
Q

Which patient group is most likely to develop an ANCA-associated small vessel vasculitis?

A

Males (0.5x higher) age 35-55 years, Northern European

62
Q

Which patient group is most likely to develop Takayasu arteritis?

A

Females < 40 years, more prevalent in Asian populations

63
Q

Describe the clinical presentation of Henoch-Schonlein Purpura (HSP)

A

Purpuric rash typically over buttocks and lower limbs, colicky abdominal pain, bloody diarrhoea, joint pain +/- swelling, may be renal involvement

64
Q

Which investigations would you perform in suspected giant cell arteritis where other results have been inconclusive and high clinical suspicion remains?

A

PET CT or CT angiogram

65
Q

What causes jaw claudication in giant cell arteritis?

A

Ischaemia of the maxillary artery

MBChB YR2 MSK (36 decks)

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