vasculitis Flashcards

1
Q

two types of vasculitis

A
  • necrotising - direct inflammation of vessel wall, infection
  • immune complex vasculitis
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
2
Q

red flags for vasculitis disease

A
  • rash - palpable purpura (commonest)
  • PUO and other constitutional symptoms
  • inflammatory arthritis or general arthralgia
  • myositis/myalgia
  • neuropathy - mononeuritis/polyneuritis
  • glomerulonephritis
  • end organ ischaemia (abdominal pain/acute visual loss)
  • anaemia/raisef inflammatory markers
  • multi system disease
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
3
Q

large vessel vasculitis

A

takayasu arteritis

GCA

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
4
Q

medium vessel vasculitis

A

polyarteritis nodosa

kawasaki disease

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
5
Q

ANCA associated small vessel vasculitis

A

microscopic polyangitis
granulomatosis with polyangitis
eosinophilic granulamtosis with polyangitis

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
6
Q

immune complex - small vessel vasculitis

A
  • cryoglobulinemic vasculitis
  • IgA vasculitis (henoch schonlein)
  • hypocomplementtermic urticarial vasculitis
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
7
Q

causes of secondary vasculitis

A
immune complex mediated
drugs
malignancy
direct infection of the vessel
rheumatological disease
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
8
Q

immune complex mediated

A

hep B
hep C
HIV
SLE

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
9
Q

drugs

A

propthyruacil
hydralazine
allopurinol
sulphasalazine

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
10
Q

malignancy

A

haematological

cutaneous small vessel disease

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
11
Q

direct infection

A

rickets
syphillis
staph
strep

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
12
Q

rheumatological

A

RA
SLE
primary sjogrens syndrome
spondyloarthropathies

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
13
Q

most common vasculitides

A
  • HSP - children

- GCA - adults

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
14
Q

pathology of GCA

A
  • T cells are recruited to vessel wall after initial exposure to the antigen
  • Release of cytokines acting on local macrophages and multinucleated giant cells including IL6
  • Further secretion of interleukins, further augmenting the inflammatory cascade oMacrophages produce oxygen free radicals and metalloproteases – degradation of the arterial wall and fragment the elastic lamina and growth factors (PDGF and VEGF)
  • With disruption of the internal elastic lamina, the intima becomes accessible to migrating myofibroblasts – laying down extracellular matrix oCauses an arteritis with local vascular destruction and intimal hyperplasia leading to luminal stenosis and occlusion
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
15
Q

cranial symptoms of GCA

A
temporal headache
jaw claudication 
scalp tenderness
polymalgia
visual
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
16
Q

visual symptoms

A
  • anterior ischaemic optic neuropathy (acute monocular visual loss with blurring of vision and flashes dots)
  • central retinal artery occlusion (cherry spot_
  • diplopia
  • corticol blindness - posterior circulation stroke
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
17
Q

systemic symptoms of GCA

A

-constitutional symptoms e.g. PUO
-weight loss
-fever
-limb claudication
-abdo pain
HTN

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
18
Q

examination findings in GCA

A

absent pulses or bruits

tender pulseless or thickenied temporal arteries

19
Q

blood findings in GCA

A
ESR>100
CRP high
alk phos high
platelets high
Hb down
20
Q

imaging findings

A

18F-FDG PET scan - will show areas of high cell metabolism - show areas of inflamed artery

USS carotid artery

temporal artery biopsy - diagnostic if visual symptoms

21
Q

medium vessel vasculitis types

A

polyarteritis noda

kawasaki disease

22
Q

what is polyarteritis nodosa

A

system necrotising vasculitis
primary or secondary (hep B)
rare, usually in middle aged men

23
Q

presentation of polyarteritis nodosa

A
  • myalgia/arthralgia
  • peripheral neuropathy: monneuritis multiplex - sensory deficit than motor
  • renovascular disease
  • GI ischaemia - perforation/haemorrhgae - cause of acute abdo
  • orchitis
  • cutaneous (nodules/purpura)
24
Q

what is Kawasaki disease

A

occurring in children <5

25
Q

symptoms of Kawasaki disease

A
fever>5 days
rash
swollen glands in the neck
dry, cracked lips, strawberry tongue
red fingers or toes
red eyes
complication: coronary artery aneurysm,
26
Q

ANCA negative small vessel vasculitis

A

HSP

27
Q

types of ANCP (anti neutrophil cytoplasm associated antibodies)

A

MPO ANCA (pANCA) = MPA/EGPA

PR3 ANCA (cANCA) = GPA

28
Q

symptoms of GPA

A
  • constitutional- anorexia/weight loss & gever
  • migratory arthalgia/myalgia
  • ENT and upper resp tract
  • pulmonary - renal
  • glomerulonephritis
  • scleritis
  • vasculitic rash
  • episcleritis
  • cutaneous nodules
  • peripheral neuropathy
  • serious otitis media & hearing loss
29
Q

presentation of GPA

A
systemic/vasculitis - 67%
immediately life threatening:
-alveolar haemorrhage
-glomerulonephritis
-scleritis
-mononeuritis multiplex

systemic symptoms e.g. fever and weight loss

localised/granulomatous - 33%
not immediately organ threatening
-upper resp tract infection
-ENT sinusitis
-episcleritis
-skin
-saddle nose deformity
-pseudotumour
30
Q

investigations of localised GPA

A
  • less likely to be ANCA positive
  • biopsy maybe helpful - low yield in ENT
  • pulmonary function tests (disctinctive flow volume loop)
  • imaging of head (sinus/ENT/orbitis) & chest
  • BP
  • urine dip
31
Q

investigations of systemic GPA

A
  • bronchoscopy - 2/3 will have pulmonary nodules which may cavitate and cause diffuse alveolar haemorrhage
  • urine dip - may have RPGN and show haematoproteinuria
  • UPCR increased
  • renal biopsy - crescenteric/sclerosing GN
32
Q

symptoms of MPA

A
  • constitutional symptoms
  • renal: GLOMERULONEPHRITIS
  • skin: MACULOPAPULAR PURPURA - lower limb
  • neuropathy - MONONEURITIS MULTIPLEX
  • pulmonary - HAEMORRHAGE< INFILTRATES & EFFUSIONS
  • GI tract - abdo pain, bleeding, ischamia, ulceration
33
Q

investigations in MPA

A
  • -ANCA positive in 2/3, mostly pANCA
  • raised acute phase response CSR/ESR/platelets
  • FBC - decreased Hb/Hct/albumin
  • U+Es - rapidly deteriorating renal function, increased urea and creatinine, lower eGFR
  • urine microscopy - red cell casts
34
Q

what is EGPA

A
  • -churg strauss syndrome
  • necrotising small vessel vasculitis with granulomas
  • long prodromal period
  • late onset asthma, allergic rhinitis peripheral eosinophilia
  • asthma progressively worsens and becomes steroid dependent
35
Q

symptoms of EGPA

A

ENT - sinusitis/polyposis
NEUROLOGY - mononeuritis
CUTANEOUS - biopsy shows eosinophil;s/granulomas
CARDIAC 25% (myocarditis) CCF&raquo_space; angina
GI abdo pain/bleeding/poor prognostic sign

36
Q

pathogenesis of HSP

A

immune complex deposition disease
linear IgA deposits in dermis/renal biopsy
leucocytocalastic vasculitis with extravasation of erythrocytes

37
Q

symptoms of HSP

A
  • rash (extensor surfaces)
  • inflammatory arthriits
  • abdo pain
  • renal
  • often follows URTI/sore throat
  • usually self limiting (analgesia only)
38
Q

adverse prognostic features of HSP

A
  • severe abdo pain/bloody diarrhoea - may beintersusseption
  • renal involvement
  • persistent rash 1/12
39
Q

investigations in HSP

A
  • diagnosis usually clinical

- biposy - skin and renal- immunofluorescence with IgA

40
Q

what is cryoglobulinaemic vasculitis

A

immunoglobulin (poly or monoclonal) that precipitated in vitro at temperatures below 37 degrees and redissolve on warming

frequent association wih Hep C

41
Q

treatment of large vessel vasculitis

A
  • IV or oral CS

- toclizumab for flares

42
Q

treatment of medium vessel vasculitis

A

-IV steroid (antiviral agents)

43
Q

treatment of small vessel vasculitis

A
  • IV CS

- rituximab for flares