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MSK > Vasculitis > Flashcards

Vasculitis Flashcards

1
Q

what is vasculitis

A

inflammation of blood vessels, often with ischemia, necrosis and organ inflammation- presentation depends on the size and location affected

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2
Q

describe primary vasculitis

A

results from inflammatory response that targets the vessels walls and has unknown cause- sometimes autoimmune

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3
Q

describe secondary vasculitis

A

may be triggered by an infection, a drug or toxin or may occur as part of another inflammatory disorder or cancer

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4
Q

what happens to the vessel in vasculitis

A

activated dendritic cell release activation of T cells and vascular inflammation. activated T cells promotes inflammation, granuloma formation and macrophage activation. macrophages produce a variety of mediators that lead t progressive vascular inflammation, endothelial damage, narrowing of lumen

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5
Q

what are the different classifications of vasculitis

A

split on the size of the vessels

small vessel vascultits split into anca and non anca associated and

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6
Q

what systemic symptoms are common to all vasculitides

A

fever, malaise, weight loss, fatigue

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7
Q

what are the two main causes of large vessel vasculitis and who do they usually affect

A 
takayasu arteritis (TA)- females under 40 
giant cell arteritis (GCA)- over 40
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8
Q

what type of reactions is seen in large vessel vasculitis

A

granulomatous infiltrations of the walls of the large vessel

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9
Q

what are the presenting features of large vessel vasculitis

A

bruit (most commonly carotid artery)
blood pressure difference of extremenities
cluadication

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10
Q

what is temporal arteritis associated with

A

polymyalgia rhematica

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11
Q

what are the symptoms of temporal arteritis

A

unilateral temporal headache, scalp tenderness and jaw claudication

temporal arteries prominent with reduced pulse

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12
Q

what is there a risk of in temporal arteritis

A

blindness- ischaemia of the optic nerve

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13
Q

what investigations can be done into temporal arteritis

A

ESR, PV, CRP will be raised

temporal artery biopsy (skip lesion might cause false negative)

MR angiogram or PET CT

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14
Q

What is the management for temporal arteritis

A

40-60mg prednisolone/steroid sparing agent

increased dose if visual loss

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15
Q

what is AAV

A

anca associated vasculitis

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16
Q

AAV with granulomas present=?

A

ask about asthma and eosinophilia present

if yes= churg strauss syndrome

if no= wengers granolmatosis

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17
Q

AAV without granulomas present=?

A

microscopic molyangitis

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18
Q

what is eosinophillic granulomatous with polyagniitis (EGPA) (churg-strauss syndrome)

A

eosinophilic granulomatous inflammation of resp tract, small and medium vessels

19
Q

what is microscopic polyangiitis (MPA)

A

necrotising vasculitis with few immune deposits

20
Q

what is granulomatosis with polyangitis (GPA) (wegener’s granulomatous)

A

granulomatous inflammation of respiratory tract, small vessels

21
Q

what is the diagnostic signs of granulomatosis with polyangitis

A

nasal or oral inflammation
abnormal chest x ray
urinary sediment
granulomatous inflammation on biopsy

22
Q

what are the ENT features of granulomatosis with polyangitis

A 
sinusitis
nasal crusting 
epistaxis 
mouth ulcers 
sensorineural deafness
otitis media and deafness 
saddle nose due to cartilage ischaemia
23
Q

what are the respiratory symptoms of granulomatosis with polyangitis

A 
pulmonary infiltrates 
cough 
haemoptysis 
diffuse alveolar haemorrhage 
cavitating nodules on CXR
24
Q

what are the cutaneous reactions of granulomatosis with polyangitis

A

palpable purpura

cutaneous ulcers

25
Q

what are the renal signs of granulomatosis with polyangitis

A

necrotising glomerulonephritis

26
Q

what are the nervous signs of granulomatosis with polyangitis

A

Mononeuritis multiplex

Sensorimotor polyneuropathy

Cranial nerve palsies

27
Q

what are the occular signs of granulomatosis with polyangitis

A 
Conjunctivitis 
Episcleritis 
Uveitis 
Optic nerve vasculitis 
Retinal artery occlusion  
Proptosis
28
Q

what is the main difference of eosinophillic granulomatosis with polyangitis (EGPA) and granulomatosis with polyangitis (PGA)

A

EPA has late onset asthma and a high eosinophil count

29
Q

what are the diagnostic features of EGPA

A

asthma, eosinophilia of more than 10% in blood, paranasal sinusitis, pulmonary infiltrates, histological proof of vasculitis with extravascular eosinophils, mononeuritis multiplex or polyneuropathy

30
Q

what does ANCA stand for

A

anti-neutrophil cytoplasmic antibodies

31
Q

what are ANCAs

A

auto-antibodies against antigens in the cytoplasm of neutrophils granulocytes

32
Q

what is used to differentiate between anca and non anca

A

immunofluoresence

33
Q

what types of anca in GPA and MPO (microscopic polyangitis) and EGPA

A

cANCA- GPA

pANCA- MPO and EGPA

34
Q

what immune process is associated with AAV

A

the formation of immune complexes

35
Q

what autoantibodies are associated with GPA

A

cANCA and anti-PR3

36
Q

what is the treatment for AAV

A

localised/ early systemic- methotrexate + steroids

generalised/ systemic- cyclophosphamide (or rituximab) + steroids

refractory- IV immunoglobulins, rituximab

37
Q

is recurrent sinusitis a common feature of EGPA

A

yes

38
Q

in non ANCA small vessel vasculitis how are the sub types classified

A

IgA dominant immune deposits?

if yes- henoch-schlein purpura

if no- check for serum cryoglobulin

39
Q

what are the types of non IgA dominant immune deposit non ANCA small vessels vasculitis

A

if serum cryoglobulin- cryoglobulinemia

if non other non ANCA vasculitis e.g. IBD vasculitis

40
Q

describe henoch-schonlein purpura (HSP)

A

an acute IgA mediated disorder causing generalised vasculitis involving the small vessels of the skin, GI tract, kidneys, joints and rarely lungs and CNS

41
Q

who gets HSP

A

children aged 2-11

42
Q

what is HSP commonly predisposed by

A

upper resp tract infection (usually group A strep)

43
Q

what is the presentation of HSP

A

purpuric rash typically over buttocks and lower limbs

colicky abdo pain

bloody diarrhoea

joint pain +/- swelling

renal involvement

44
Q

how is HSP managed

A

usually self limiting

tend to resolve in 8 weeks

relapses may occur

screen for renal involvement

MSK (71 decks)

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