Vasculitis

Question 1

What are primary vasculitides?

Answer 1

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

Question 2

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

Answer 2

Skin
Kidneys
Lungs
Joints
Nerves 
ENT

Question 3

Give examples of large vessel vasculitis?

Answer 3

Takaysu arteritis

Giant cell arteritis

Question 4

Give examples of medium vessel vasculitis?

Answer 4

Polyarteritis nodosa

Kawasaki disease

Question 5

Give examples of ANCA-associated small vessel vasculitis?

Answer 5

Microscopic polyangitis
Granulomatosis with. polyangitis
Eosinophilic granulomatosis with polyangitis

Question 6

Give. examples of immune complex. small vessel vasculitis?

Answer 6

Cryoglobulinaemic vasculitis
IgA vasclitis
Hypocomplementemic urticarial vasculitis

Question 7

Which vessels are affected. by giant cell arteritis?

Answer 7

Aorta and its major branches

Question 8

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

Answer 8

70-79 years

Question 9

In which groups is giant cell arteritis most common?

Answer 9

Women

Caucasian

Question 10

What are the symptoms of giant cell arteritis?

Answer 10

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

Question 11

How can giant cell arteritis lead to blindness?

Answer 11

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

Question 12

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

Answer 12

Temporal artery. symmetry
Thickening
Loss of pulsatility

Question 13

What is the gold standard test for giant cell arteritis?

Answer 13

Temporal artery biopsy

Question 14

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

Answer 14

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

Question 15

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

Answer 15

True

Question 16

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

Answer 16

Temporal artery US, MRI and PET CT

Question 17

How is giant. cell arteritis treated?

Answer 17

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

Question 18

What are. the differential diagnoses of IgA vasculitis?

Answer 18

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

Question 19

In what age group is IgA vasculitis most common?

Answer 19

Children aged 2-11 years

Question 20

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

Answer 20

True

Question 21

What are the signs of IgA vasculitis?

Answer 21

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

Question 22

What are the potential complications of. IgA vasculitis?

Answer 22

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

Question 23

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

Answer 23

Urinalysis

Urine PCR

Question 24

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

Answer 24

True

Question 25

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

Answer 25

Testicular torsion GI. disease Arthritis

Question 26

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

Answer 26

ANCA Associated vasculitis

Question 27

What cells are ANCA autoantibodies directed against?

Answer 27

Neutrophils | Monocytes

Question 28

Why is detection of ANCA autoantibodies helpful in vasculitis?

Answer 28

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

Question 29

cANCA with PR3. is very suggestive of...?

Answer 29

Granulomatosis with polyangiitis

Question 30

pANCA with strong mPO is suggestive of

Answer 30

Microscopic polyangiitis

Question 31

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

Answer 31

prednisolone with Oral or IV cyclophosphamide or rituximab in severe disease Prednisolone with methotrexate. or mycophenolate in moderate. disease

Question 32

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

Answer 32

Infection Cytopaenia Malignancy Infertility

Question 33

What is the mechanism of action of rituximab?

Answer 33

Anti-B cell biologic

Question 34

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

Answer 34

Azathioprine Methotrexate Mycophenolate

Question 35

What characterises granulomatosis with polyangiitis?

Answer 35

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

Question 36

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

Answer 36

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

Question 37

What constitutional symptoms are there in granulomatosis with polyangiitis?

Answer 37

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