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Musculoskeletal > Vasculitis > Flashcards

Vasculitis Flashcards

1
Q

What?

A

Inflammation of blood vessels

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2
Q

What can this lead to?

A

Inflammation/ Ischaemia/ Necrosis of tissues that vessel supplies

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3
Q

Primary vasculitis?

A

Results from an inflammatory response that targets the vessel walls and has no known cause.

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4
Q

Secondary vasculitis?

A

May be triggered by an infection, a drug, or a toxin or may occur as part of another inflammatory disorder (e.g. RA or lupus) or cancer

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5
Q

3 main categories?

A

Large, medium and small vessel

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6
Q

What is large vessel vasculitis?

A

Causes granulomatous inflammation predominantly of aorta and its major branches

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7
Q

2 main categories of large vessel vasculitis?

A 
Temporal (giant cell) arteritis
Takayasu arteritis (TA)
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8
Q

Age of onset in large vessel vasculitis?

A

GCA - after 50

TA - before 50

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9
Q

Who gets takayasu arteritis?

A

> In East Asian countries
Young women in 2nd and 3rd decades
Females

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10
Q

Pathology of large vessel vasculitis?

A

Granulomatous infiltration of walls of large vessels

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11
Q

Takayasu arteritis?

A

Claudication
Pain in arm muscles e.g. when doing housework
Bruit – carotid artery
BP difference – between left and right side extremities -> pulseless disease
Angiogram

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12
Q

GCA?

A 
Temporal arteritis
Prominent temporal arteries
Strong assoc. with polymyalgia rheumatica
Pain in jaw when chewing food
Tongue claudication - linguine artery
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13
Q

Features of temporal arteritis?

A 
Unilateral acute temporal headache
Scalp tenderness
Temporary visual disturbances
Blindness
Jaw claudication
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14
Q

Clinical features of large cell vascultiis?

A 
Low- grade fever
Malaise
Night sweats
Weight loss
arthralgia
Fatigue
Claudicant symptoms (both upper and lower limbs)
Untreated -> vascular stenosis and aneurysms (reduced pulses and bruits)
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15
Q

Investigations for large vessel vasculitis?

A

ESR, CRP and plasma viscosity elevated
MR angiography –> thickened vessels & vessel stenosis
PET CT – if inflammation present

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16
Q

Investigations for GCA?

A

TEMPORAL ARTERY BIOPSY
- Skip lesions occur so biopsy may be false neg
Ultrasound
- 24- 48 hrs after start steroids

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17
Q

Treatment for large vessel vasculitis?

A

Corticosteroids (prednisolone 40- 60mg)

Methotrexate and azathioprine (may be added)

18
Q

Immunosuppressives in TA?

A

Leflunamide, methotrexate

19
Q

Immunosuppressives in relapsing or resistant GCA?

A

Tocilizumab – IL-6 blocking agent

20
Q

How long is GCA treatment?

A

18 months to 2 years

21
Q

4 types of ANCA associated vasculitis?

A

Granulomatosis with polyangitis
Microscopic polyangitis
Renal limited vasculitis
Eosinophilic granulomatosis with polyangitis

22
Q

Who gets granulomatosis with polyangitis (GPA/ wagerers)?

A

Northern Europe
Males >
35- 55 years

23
Q

Symptoms of GPA?

A 
Constitutional symptoms and arthralgia
ENT symptoms
Respiratory symptoms 
Cutaneous
Renal
24
Q

ENT symptoms of GPA?

A 
Nose bleeds 
Deafness 
Recurrent sinusitis 
Nasal crusting (over time can be collapse of nose) 
Epistaxis 
Mouth ulcers 
“Saddle nose” 
Sub- glottic inflammation (trouble breathing, inflammation) 
Proptosis
25
Q

Resp symptoms of GPA?

A 
Haemoptysis 
Cavitating lesions on x-ray 
Cough  
Pulmonary infiltrates 
Diffuse alveolar heamorrhages
26
Q

Cutaneous symptoms of GPA?

A

Palpable purpura

Cutaneous ulcers

27
Q

Renal symptoms of GPA?

A

Necrotizing glomerulonephritis

28
Q

What is GPA associated with?

A

cANCA and PR3

29
Q

EGPA?

A

Characterized by late onset asthma, rhinitis and raised peripheral blood eosinophil count
Mono neuritis multiplex

30
Q

Complications of microscopic polyangitis?

A

Glomerulonephritis

31
Q

Investigations for ANCA associated vasculitis?

A

ESR, PV and CRP raised
Anaemic of chronic disease common
U + E (renal involvement?)
Anto- neutrophil cytoplasmic antibody (ANCA)
Urinalysis (looking for renal vasculitis)
CXR
Biopsy (of affected area)

32
Q

Management of ANCA assoc vasculitis?

A

IV steroids and cyclophosphamide

33
Q

Henoch Schonlein purpura?

A

Acute immunoglobulin A (IgA) mediated disorder
Generalized vasculitis involving small vessels of skin, GI tract, kidneys, joints & rarely lungs and CNS
Group A streptococcus (most common)

34
Q

Who gets Henoch Schonlein purpura?

A

Children (2-11)
History of upper resp tract infection (few weeks)
Rare in infants

35
Q

Symptoms of Henoch Schonlein purpura?

A 
Purpuric rash over buttocks and lower limbs 
Abdominal pain (colicky) 
Vomiting  
Joint pain +/- swelling 
Bloody diarrhea 
Renal involvement
36
Q

Management of Henoch Schonlein purpura?

A

Self- limiting – settles over weeks to months

37
Q

Investigations for vasculitis?

A

Urine dipstick
FBC, Liver and renal profile, inflammatory markers
ANCA and specific antibodies, connective tissue disease screen, compliment levels
Imaging investigations – Chest x-ray and/or CT scan
Nerve conduction tests (neurological symptoms?)
Tissue biopsy (most definitive test) - GOLD

38
Q

ANCA?

A

Anti- neutrophil cytoplasmic antibodies

Autoantibodies against antigens in cytoplasms of neutrophil granulocytes

39
Q

ANCA in GPA?

A

cANCA

40
Q

ANCA in EGPA?

A

pANCA

41
Q

ANCA in MPA?

A

pANCA

Musculoskeletal (12 decks)

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