Week 224 - Vasculitis

Question 0

Temporal Arteritis AKA Giant Cell Arteritis, Polymyalgia Rheumatica, Weger’s Granulomatosis and Polyarteritis Nodosa are all forms of what?

Answer 0

Primary Vasculitis

Question 1

What are the two more common forms of Primary Vasculitis?

Answer 1

Polymyalgia Rheumatica and Giant Cell Arteritis

Question 2

How does Polymyalgia Rheumatica (the milder form) commonly present?

Answer 2

Pain and stiffness in neck, shoulders and pelvic girdle > worse in the morning, improves with activity
Systemic
In middle aged and elderly (if presents

Question 3

How does Giant Cell Arteritis (GCA - the more severe of the two) tend to present?

Answer 3

Fairly sudden onset and constant headache

Tenderness around scalp and temples

Question 4

Which vessels are affected in GCA?

Answer 4

Large ones - aorta and branches coming off it

Limited to vessels with internal elastic

Question 5

If left untreated what possible outcomes might there be?

Answer 5

• irreversible blindness
• stroke/MI/Tissue necrosis

EARLY DIAGNOSIS AND MANAGEMENT CRUCIAL

Question 6

How do you diagnose GCA?

Answer 6

PET scan - radioactive isotope takes up glucose

Temporal artery biopsy (not everyone has them)

Question 7

What is the immediate treatment for GCA?

Answer 7

Prednisolone

Question 8

Along with Wegner’s Granulomatosis and Polyarteritis Nodosum name two more causes of ‘small vessel vasculitis’

Answer 8

Microscopic polyangiitis

Churg-Strauss

Question 9

List the 3 main non-specific symptoms of small vessel vasculitis

Answer 9

1) malaise
2) fever
3) weight loss

Question 10

What is the reason that small vessel vasculitis can be mistaken for these other conditions?

Answer 10

The non-blanching rash (purpura / petichial rash)

Question 11

List 4 things vasculitis can mimic

Answer 11

1) infectious endocarditis
2) septicaemia
3) left atrial myxoma
4) ???

Question 12

What causes purpura / petichae?

Answer 12

Leaky blood vessels

Question 13

What is ANCA?

Answer 13

"Anti neutrophil cytoplasmic antibody"
A heterogenous group of antibodies:
- cANCA (cytoplasmic)
- pANCA (perinuclear)
- aANCA (atypical)
Not very specific but indictor of vasculitis!

Question 14

ANCA together with what 2 other antibodies indicates Wegner’s Granulomatosis?

Answer 14

MPO and PR3

Question 15

Wegner’s Granulomatosis is associated with what, pathologically?

Answer 15

Destructive vasculitis
Midline structures
Granulomas - can look like TB

Question 16

What lung manifestations are associated with Wegner’s?

Answer 16

SOB; Pleurisy; Haemoptysis due to Haemorrhagic alveolitis

Question 17

What ENT manifestations are associated with Wegner’s?

Answer 17

Sinusitis (most commonly); Otitis Media; Nasal Discharge; URT stenosis; Saddle Nose Deformity

Question 19

What is associated with / characterised by aneurysms in medium sized vessels, wrist drop, foot drop and Hepatitis B?

Answer 19

Polyarteritis Nodosa (uncommon condition)
Necrotizing vasculitis of medium-sized vessels

Question 19

What are the main aims of vasculitis (particularly small vessel) treatment?

Answer 19

To induce and then maintain remission

Question 20

How do Polyarteritis Nodosa and Microscopic Polyangiitis differ?

Answer 20

Microscopic Polyangiitis - necrotizing vasculitis of small vessels; rapidly progressive glomerulonephritis usually features; associated with p-ANCA (to lesser ext. c-ANCA)

Question 21

What are the 3 overarching drug types used to induce remission of primary vasculitis?

Answer 21

> Steroids
Cyclophosphamides
Biologics e.g. Anti-TNFs / Rituximab

Question 22

List 3 key recurrences that occur in Antiphospholipid Syndrome

Answer 22

> Recurrent vascular thrombosis
Recurrent migraine
Repeated miscarriages

Question 23

List some drugs used to maintain remission of primary Vasculitis?

Answer 23

• Azathioprine (immune-suppressant)
• Methotrexate “
• Mycophenolate “
• corticosteroids