vasculitis Flashcards
(43 cards)
1
Q
two types of vasculitis
A
- necrotising - direct inflammation of vessel wall, infection
- immune complex vasculitis
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
3
Q
large vessel vasculitis
A
takayasu arteritis
GCA
4
Q
medium vessel vasculitis
A
polyarteritis nodosa
kawasaki disease
5
Q
ANCA associated small vessel vasculitis
A
microscopic polyangitis
granulomatosis with polyangitis
eosinophilic granulamtosis with polyangitis
6
Q
immune complex - small vessel vasculitis
A
- cryoglobulinemic vasculitis
- IgA vasculitis (henoch schonlein)
- hypocomplementtermic urticarial vasculitis
7
Q
causes of secondary vasculitis
A
immune complex mediated drugs malignancy direct infection of the vessel rheumatological disease
8
Q
immune complex mediated
A
hep B
hep C
HIV
SLE
9
Q
drugs
A
propthyruacil
hydralazine
allopurinol
sulphasalazine
10
Q
malignancy
A
haematological
cutaneous small vessel disease
11
Q
direct infection
A
rickets
syphillis
staph
strep
12
Q
rheumatological
A
RA
SLE
primary sjogrens syndrome
spondyloarthropathies
13
Q
most common vasculitides
A
- HSP - children
- GCA - adults
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
15
Q
cranial symptoms of GCA
A
temporal headache jaw claudication scalp tenderness polymalgia visual
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
17
Q
systemic symptoms of GCA
A
-constitutional symptoms e.g. PUO
-weight loss
-fever
-limb claudication
-abdo pain
HTN
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
symptoms of Kawasaki disease
```
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
ANCA negative small vessel vasculitis
HSP
27
types of ANCP (anti neutrophil cytoplasm associated antibodies)
MPO ANCA (pANCA) = MPA/EGPA
PR3 ANCA (cANCA) = GPA
28
symptoms of GPA
- 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
presentation of GPA
```
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
investigations of localised GPA
- 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
investigations of systemic GPA
- 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
symptoms of MPA
- constitutional symptoms
- renal: GLOMERULONEPHRITIS
- skin: MACULOPAPULAR PURPURA - lower limb
- neuropathy - MONONEURITIS MULTIPLEX
- pulmonary - HAEMORRHAGE< INFILTRATES & EFFUSIONS
- GI tract - abdo pain, bleeding, ischamia, ulceration
33
investigations in MPA
- -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
what is EGPA
- -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
symptoms of EGPA
ENT - sinusitis/polyposis
NEUROLOGY - mononeuritis
CUTANEOUS - biopsy shows eosinophil;s/granulomas
CARDIAC 25% (myocarditis) CCF >> angina
GI abdo pain/bleeding/poor prognostic sign
36
pathogenesis of HSP
immune complex deposition disease
linear IgA deposits in dermis/renal biopsy
leucocytocalastic vasculitis with extravasation of erythrocytes
37
symptoms of HSP
- rash (extensor surfaces)
- inflammatory arthriits
- abdo pain
- renal
- often follows URTI/sore throat
- usually self limiting (analgesia only)
38
adverse prognostic features of HSP
- severe abdo pain/bloody diarrhoea - may beintersusseption
- renal involvement
- persistent rash 1/12
39
investigations in HSP
- diagnosis usually clinical
| - biposy - skin and renal- immunofluorescence with IgA
40
what is cryoglobulinaemic vasculitis
immunoglobulin (poly or monoclonal) that precipitated in vitro at temperatures below 37 degrees and redissolve on warming
frequent association wih Hep C
41
treatment of large vessel vasculitis
- IV or oral CS
| - toclizumab for flares
42
treatment of medium vessel vasculitis
-IV steroid (antiviral agents)
43
treatment of small vessel vasculitis
- IV CS
| - rituximab for flares
