Disease Profiles: Vasculitis

Question 1

Define early systemic ANCA-associated small vessel vasculitis

Answer 1

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

Question 2

Describe the prognosis of Henoch-Schonlein Purpura (HSP)

Answer 2

Usually self-limiting within 8 weeks, may be relapses

Question 3

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

Answer 3

MR angiogram

Question 4

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

Answer 4

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

Question 5

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

Answer 5

To check for renal involvement

Question 6

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?

Answer 6

Giant cell arteritis

Question 7

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

Answer 7

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

Question 8

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

Answer 8

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

Question 9

What causes ‘saddle nose’ in granulomatosis with polyangiitis?

Answer 9

Cartilage damage from ischaemia

Question 10

Describe the systemic symptoms common to all vasculitides

Answer 10

Fever, malaise, weight loss, fatigue

Question 11

How would you manage a patient with Takayasu arteritis?

Answer 11

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

Question 12

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

Answer 12

Necrotising glomerulonephritis

Question 13

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

Answer 13

ESR, plasma viscosity, CRP

Question 14

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

Answer 14

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

Question 15

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

Answer 15

Patients in late 60s+

Question 16

What is granulomatosis with polyangiitis?

Answer 16

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

Question 17

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

Answer 17

Microscopic polyangiitis

Question 18

Which patient group is most likely to develop polymyalgia rheumatica?

Answer 18

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

Question 19

What are anti-neutrophil cytoplasmic antibodies (ANCAs)?

Answer 19

Auto-antibodies against antigens in the cytoplasm of neutrophil granulocytes

Question 20

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

Answer 20

Vascular stenosis and aneurysms

Question 21

Describe the ocular manifestations of granulomatosis with polyangiitis

Answer 21

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

Question 22

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

Answer 22

Temporal artery USS

Question 23

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

Answer 23

IV immunoglobins

Rituximab

Question 24

Which vasculotide is polymyalgia rheumatica often associated with?

Answer 24

Giant cell arteritis

Question 25

Name the two categories of large vessel vasculitis

Answer 25

Temporal (giant cell) arteritis and Takayasu arteritis

Question 26

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

Answer 26

Cyclophosphamide and steroids, followed by azathioprine for maintenance Plasma exchange if creatinine \> 500

Question 27

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

Answer 27

Late onset asthma, high eosinophil count and ANCA specificity

Question 28

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

Answer 28

ESR, PV and CRP

Question 29

Name the ANCA-associated small vessel vasculitides

Answer 29

Granulomatosis with polyangiitis, eosinophilic granulomatosis with polyangitis, microscopic polyangiitis

Question 30

What is microscopic polyangiitis?

Answer 30

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

Question 31

Describe the articular manifestations of polymyalgia rheumatica

Answer 31

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

Question 32

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

Answer 32

Granulomatosis with polyangiitis

Question 33

Define vasculitis

Answer 33

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

Question 34

What is secondary vasculitis?

Answer 34

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

Question 35

Describe the ENT manifestations of granulomatosis with polyangiitis

Answer 35

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

Question 36

Define localised ANCA-associated small vessel vasculitis

Answer 36

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

Question 37

What is large vessel vasculitis?

Answer 37

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

Question 38

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

Answer 38

Eosinophilic granulomatosis with polyangitis

Question 39

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

Answer 39

Cyclophosphamide and steroids, followed by azathioprine for maintenance

Question 40

Define systemic ANCA-associated small vessel vasculitis

Answer 40

Renal (creatinine \< 500) or other vital organ failure

Question 41

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

Answer 41

Chronic disease

Question 42

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

Answer 42

Takayasu arteritis

Question 43

Describe the management of polymyalgia rheumatica

Answer 43

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

Question 44

What would an MR angiogram show in Takayasu arteritis?

Answer 44

Thickened vessel walls and stenosis

Question 45

What is Henoch-Schonlein Purpura (HSP)?

Answer 45

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

Question 46

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

Answer 46

Methotrexate and steriods

Question 47

Define refractory ANCA-associated small vessel vasculitis

Answer 47

Progressive disease unresponsive to steroids + cyclophosphamide

Question 48

What is primary vasculitis?

Answer 48

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

Question 49

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)

Answer 49

Giant cell arteritis

Question 50

Describe the systemic manifestations of polymyalgia rheumatica

Answer 50

Fatigue, anorexia, weight loss, fever

Question 51

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

Answer 51

Palpable purpura, oral/cutanous ulcers

Question 52

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

Answer 52

Methotrexate + steroids

Question 53

How would you manage a patient with giant cell arteritis?

Answer 53

Start with prednisolone 40-60mg daily, gradual reduction over 18 months-2 years If early relapse - leflunamide, methotrexate If resistant/relapsing - toclizumab

Question 54

Define generalised ANCA-associated small vessel vasculitis

Answer 54

Renal (creatinine \< 500) or other organ threatening

Question 55

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

Answer 55

Active disease

Question 56

Describe the respiratory manifestations of granulomatosis with polyangiitis

Answer 56

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

Question 57

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

Answer 57

Biopsy

Question 58

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

Answer 58

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

Question 59

What is the diagnostic investigation for giant cell arteritis?

Answer 59

Temporal artery biopsy

Question 60

What is polymyalgia rheumatica?

Answer 60

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

Question 61

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

Answer 61

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

Question 62

Which patient group is most likely to develop Takayasu arteritis?

Answer 62

Females \< 40 years, more prevalent in Asian populations

Question 63

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

Answer 63

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

Question 64

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

Answer 64

PET CT or CT angiogram

Question 65

What causes jaw claudication in giant cell arteritis?

Answer 65

Ischaemia of the maxillary artery