Vasculitis Flashcards

(44 cards)

1
Q

What is vasculitis

A

Inflammation of blood vessels

Prolonged inflammation can lead to thrombosis, ischemia, organ infarction necrosis and even death

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

Which blood vessels can vasculitis

A

ANY

Arteries, arterioles, veins, venules or capillaries

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

What causes secondary vasculitis

A

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

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

How do you classify vasculitis

A

Based on which type of blood vessel is affected

Small, medium or large

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

How does vasculitis present

A

Very variable - dependant in which vessels are affected
Systemic symptoms such as fever, malaise, weight loss and fatigue are common to all types
Can cause thrombosis and infarction as the inflammation of the blood vessel narrows the lumen

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

What does Giant cell arteritis typically cause

A

Temporal arteritis

May affect aorta and other large vessels

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
7
Q

What is seen in blood vessel walls in vasculitis

A

Granuloma formation

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
8
Q

What are the presenting features of large vessel vasculitis

A

Symptoms are dependant on which arterial branches are affected
Bruit - most commonly in carotid
Blood pressure difference in extremities

Takayasu - cannot palpate peripheral pulses

GCA
Headache
Diplopia or visual loss
Claudication - jaw and tongue

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

What are the signs of temporal arteritis

A

Unilateral temporal headache
Scalp tenderness
Jaw claudication
Temporal arteries become prominent but with reduced pulsation

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

What is the major risk with temporal arteritis

A

Blindness

Due to ischaemia of the optic nerve

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

What investigations do you do for large vessel vasculitis

A

ESR, plasma viscosity and CRP - will be raised
Temporal artery biopsy - GOLD STANDARD
MR angiogram or PET CT

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

How is small vessel vasculitis divided

A

Into ANCA associated vasculitis (AAV) and ANCA negative

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

Describe the pathology of GPA

A

Granulomatous inflammation of respiratory tract, ENT system and small and medium vessels
Necrotising glomerulonephritis common

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

Describe the pathology of EGPA

A

Eosinophilic granulomatous inflammation of respiratory tract, ENT system, small and medium vessels.
Associated with asthma

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
15
Q

Describe the pathology of MPA

A

Necrotising vasculitis with few immune deposits.

Necrotising glomerulonephritis very common

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
16
Q

What are the ENT features of GPA

A
Sinusitis
Nasal crusting
Blocked nose
Epistaxis
Mouth ulcers
Sensorineural deafness - bilateral
Otitis media and deafness
“Saddle nose” due to cartilage ischaemia and necrosis
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
17
Q

What is epistaxis

A

Nose bleed

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

What are the cutaneous features of GPA and what causes them

A

Palpable purpura
Petichial rashes
Cutaneous ulcers
Caused by inflammation of the small blood vessels in the skin

19
Q

What are the respiratory features of GPA

A
Pulmonary infiltrates
Interstitial lung disease 
Cough and SOB
Haemoptysis 
Diffuse alveolar haemorrhage
Cavitating nodules on CXR

Stridor caused by tracheal inflammation and resulting subglottic stenosis

20
Q

What is the key renal feature of GPA

A

Necrotising glomerulonephritis
Can lead to rapid renal failure

Look for blood and/or protein in urine. Any more than a trace requires further tests (red cell cast test and protein creatinine ratio)
May go onto get renal biopsy

21
Q

What are the nervous system features of GPA

A

Mononeuritis multiplex - foot and wrist drops
Peripheral neuropathy - glove and stocking
Cranial nerve palsies

22
Q

What are the ocular features of GPA

A
Conjunctivitis 
Episcleritis 
Scleritis - nodular 
Uveitis 
Optic nerve vasculitis 
Retinal artery occlusion  
Proptosis
Retro orbital granulomas mimicking tumours
23
Q

What are ANCAs

A

Anti-neutrophil cytoplasmic antibodies

Autoantibodies against the cytoplasm of neutrophil granulocytes

24
Q

How can you detect ANCA

A

Immunofluorescence

Also allows you to determine the pattern and differentiate between C-ANCA and P-ANCA

25
What is HSP
Henoch-Schönlein purpura Acute IgA mediated disorder Generalized vasculitis - not ANCA associated
26
What blood vessels are affected by HSP
Iinvolves the small vessels of the skin, the gastrointestinal (GI) tract, the kidneys, the joints, and, rarely, the lungs and the central nervous system
27
HSP usually has a preceding infection - true or false
TRUE | >75% have URTI, pharyngeal or GI infection Usually about 1-3 weeks before HSP
28
What organism most commonly causes the infection preceding HSP
Group-A strep
29
How does HSP present
``` Purpuric rash typically over buttocks and lower limbs Colicky abdominal pain Bloody diarrhoea Joint pain +/- swelling Renal involvement - 50% ```
30
How do you manage HSP
Usually self limiting and will resolve in 8 weeks | Need to check for renal involvement and treat accordingly
31
What are the main subtypes of large vessel vasculitis
Giant cell arteritis - seen in over 50s | Takayasu - seen in younger women
32
Which vessels are affected in large vessel vasculitis
Involves the aorta and branches
33
What are the main subtypes of medium vessel vasculitis
Kawasaki disease - seen in kids polyarthritis nodosa Rarest types of vasculitis
34
List the ANCA associated small vessel vasculitis
Microscopic polyangitis (MPA) Granulomatosis with polyangiitis (GPA Previously Wegener's Eosinophilic Granulomatosis with polyangiitis (EGPA) Previously Churg Strauss
35
ANCA negative small vessel vasculitis is also called what
Immune complex small vessel vasculitides | Includes HSP, anti-GBM disease etc
36
What are the MSK features of GPA
Myalgia Arthralgia Arthritis
37
What are the cardio features of GPA
pericarditis, coronary | vasculitis myocarditis if coronary arteries are involved
38
What is the screening test for small vessel vasculitis
ANCA or anti-neutrophil cytoplasmic antibody
39
Which blood tests may be abnormal in vasculitis
FBC - Anaemia of chronic disease(normocytic, normochromic anaemia) is a common finding CRP/ESR/PV - usually raised due to inflammation LFTs can be deranged if liver involvement U&E deranged if renal involvement
40
Which subtypes of ANCA are seen in GPA
c-ANCA and anti-PR3
41
Which subtypes of ANCA are seen in EGPA and MPA
p-ANCA Directed against myeloperoxidase (MPO) Anti-MPO
42
Can ANCA be used to monitor disease activity
Yes | Titres often rise with increasing disease activity
43
How do you treat vasculitis
Basically immunosuppression (lifelong) with steroids and steroid sparing agents such as cyclophosphamide, rituximab, methotrexate, azathioprine etc Choice of agent depends on how severe the disease is - use the 5 factor score to see how many organ systems are involved If they score 1 or more - need high dose steroids with either cyclophosphamide or rituximab Then move to steroid sparing agent when in remission or if low severity
44
What prophylaxis is require for patients on | cyclophosphamide
Pneumocystis jirovecii prophylaxis with | Cotrimoxazole