vasculitis is a heterogeneous group of disorders that destroys blood vessels by inflammation.Both arteries and veins can be affected.

1. Imaging in Vasculitis
Dr. Yasna Kibria
Resident, Phase B
Department of Radiology & Imaging
BSMMU

2. A) Large vessels B)Medium vessels C)Small vessels
• Vasculitis is a general term for
heterogeneous group of conditions
that cause inflammation of the blood
vessels
• The group is sometimes called in the
plural form - Vasculitidis

3. Vasculitidis
1994 Chapel Hill Consensus: Based on size
2012 Modified Chapel Hill Consensus: Based on
size, structural & functional attributes

4. Patient with Suspected vasculitis
Establish diagnosis
Clinical findings
Laboratory workup
Properly categorise to a specific
vasculitis syndrome
Characteristic syndrome
Imaging where appropriate
Biopsy
Determine pattern & extent
of the disease
Treat vasculitis
Look for offending antigen
Treat vasculitis
No further action
Remove antigen
Syndrome resolves
Look for underlying disease
Treat underlying disease
Yes
Yes
Yes
No
No
No

5. • Diagnosis
• Supplement/complement biopsy
• Assessment of sites/extent
• Assessment of severity
• Prognostication
• Monitoring disease activity
• Detection of potential complications
• Guide treatment planning
• Post operative surveillane
Role of Imaging

6. Radiological Investigations
• In many forms of vasculitis, pathologic changes can be visualized by
imaging, either directly by visualizing the vessel lesions or indirectly
by visualizing the effects of vessel inflammation in the affected organ
• Choice of the imaging modality depends on the size and location of
the affected vessels
• A number of different imaging techniques are available for visualizing
direct and indirect signs of vessel inflammation usually in LVV

7.  Ultrasound
• Visualise hyperemia and hypervascularization of
vessel walls, typical characteristics of a florid
inflammatory process
• Measurement of the intima-media thickness
Computed tomography (CT)
• Visualization of changes in the osseous structures,
head neck region and the lungs
MRI
• Black blood- assessment of the
morphologic features of the great
vessels, delineation of the arterial
wall from the lumen and the
surrounding perivascular tissue
• Post-contrast MR- allow
visualization of contrast
enhancement of the arterial wall
• Vessel wall imaging- assessment of
walls of the vessels beyond luminal
abnormality, also assess the lumen
calibre
DSA
• is more sensitive than MRI for the
detection of vasculitic luminal
changes of small- and medium-sized
peripheral arteries
• However, it does not visualize the
vessel walls.
• It has high invasiveness with
relatively low specificity.
PET-CT/MR
• can detect active disease and also
determine the extension

8. Large vessel vasculitis
 Chronic granulomatous inflammation
predominantly involving the aorta and its major
branches.
 The two major entities in this group are:
• Giant cell arteritis : affects older individuals
(typically over 50)
• Takayasu arteritis : affects younger individuals
(typically under 50)
Other Conditions That Can Involve Large Caliber
Vessels:
•Hughes-stovin Syndrome
•Kawasaki Disease
•Behcet Syndrome
•Rheumatoid Arthritis
•Syphilis
•Tuberculosis
•Isolated Angiitis Of The Central Nervous System

9. Giant cell arteritis
• Giant cell arteritis derives its name from the presence of giant
cells in the infiltrative process in all layers of the blood vessel
wall. Mononuclear cells, lymphocytes, T-cells, and macrophages
are more commonly present
• Vasculitis affecting medium- to large-sized arteries.
• Also known as temporal arteritis or cranial arteritis, given its
propensity to involve the extracranial external carotid artery
branches such as the superficial temporal artery

11. ULTRASOUND:
• US/color Doppler US is a well-established imaging modality for vessel
assessment in patients with suspected vasculitis.
• It is easy to perform, cost effective, and does not require exposure to ionizing
radiation or IV contrast.
• Two main imaging features or signs are described as characteristics of
vasculitis:
(1) the so-called halo sign and
(2) the compression sign.
Radiographic features

13. US:
Stenosis may be present but is not a
specific sign for giant cell arteritis

14. The European League Against
Rheumatism (EULAR) recommends US of
the temporal arteries and axillary arteries
as a first-line imaging modality in patients
with suspected predominantly cranial
GCA.
limitations of US:
• operator dependent
• not suitable for intrathoracic vessels.

15. Giant cell arteritis
Radiographic features
CT
Wall thickening of affected segments
Calcification
Mural thrombi
CTA & DSA (angiography) is useful for
assessing luminal abnormalities:
Stenoses
Occlusions
Dilatations
Aneurysm formation
MRI (T1 C+ Gd):
• Best sequence for assessment
• Shows mural inflammation very
well
• Mean wall thickness increased &
luminal diameter decreased in the
affected region

22. Takayasu arteritis
• AKA Idiopathic Medial Aortopathy or
Pulseless Disease, Predominantly affects the
aorta and its major branches.
• It may also affect the pulmonary arteries.
• The exact cause is not well known but the
pathology is thought to be similar to giant cell
arteritis
Epidemiology:
• Strong female predominance (F: M ~ 9:1)
• Prevalence in Asian populations
• Affect younger patients (<50 years of age)
• Typical age of onset is at around 15-30
years

23. Clinical presentation:
Ischemic symptoms due to stenotic lesions or
thrombus formation, dependent on the territory of
vascular involvement.
Two phases of the disease are classically described:
Pre-pulseless phase: characterized by non-
specific systemic symptoms
Pulseless phase: presents with limb ischemia or
reno-vascular hypertension
Takayasu arteritis
Asymmetric BP
between leg & arm

24. Type I: Involving solely the aortic arch
branches: brachiocephalic trunk, carotid and
subclavian arteries
Type II:
IIa: involvement of the aorta solely at its
ascending portion and/or at the aortic arch +/-
branches of the aortic arch
IIb: involvement of the descending thoracic
aorta +/- ascending or aortic arch + branches
Type III: involvement of the thoracic and
abdominal aorta distal to the arch and its
major branches
Type IV: sole involvement of the abdominal
aorta and/or the renal arteries
Type V: generalized involvement of all
aortic segments
Classification:

25. Radiographic features
Ultrasound:
 Long segment, smooth, homogeneous and
moderately echogenic circumferential thickening
of the arterial wall
 On transverse section, this finding is termed as
the 'macaroni sign' and is highly specific for
Takayasu arteritis
 Vascular occlusion may be seen due to intimal
thickening and/or secondary thrombus formation
 Flow velocities depend on the level of occlusion
 Aneurysms may be noted
 There may be a loss of pulsatility of the vessel

26. CT/MRI
• Wall thickening & enhancement :
active acute phase
• Aortic valve disease: stenosis,
regurgitation
• Occlusion of major aortic branches
• Aneurysmal dilatation of the aorta
or its branches
• Pseudo-aneurysm formation
• Diffuse narrowing distally (i.e.
descending and abdominal aorta): in
the late phase
• The pulmonary arteries are also
commonly involved, with the most
common appearance being
peripheral pruning

30. Polyarteritis Nodosa (PAN)
A systemic inflammatory necrotizing vasculitis that involves
small to medium-sized arteries (larger than arterioles) of the
abdominal visceral organs, the heart, and the hands and feet
Epidemiology
• PAN is more common in males (M:F=2:1)
• Typically presents around the 5th to 7th decades
Associations
• Hepatitis B
• Hepatitis C
Frequent sites of involvement are:
• Renal: 80-90%, tends to be the prominent site and
major cause of death
• Cardiac: ~70%
• Gastrointestinal tract: 50-70%
• Hepatic: 50-60%
• Spleen: 45%
• Pancreas: 25-35%
• CNS complications: 20-45%

32. Transmural
necrotizing
inflammation
Weakening of
the wall
Micro-
aneurysm
Subsequent
focal rupture
Fibrinoid necrosis
of vessels
Thrombosis
Infarction of the
tissue suplied
 Fibrous thickening and mononuclear
infiltration occur at a later stage
 Different stages of inflammation can
occur in the same vessel at different
points

35. Kawasaki disease
 Kawasaki disease (mucocutaneous lymph node syndrome)
is a small to medium vessel autoimmune vasculitis
 predominantly affecting young children
 It can affect any organ but there is a predilection for
the coronary vessels.
 Epidemiology
•Affects children usually under the age of 12 years with the
peak incidence in children 18 to 24 months old
•Japan has the highest incidence
•Worldwide, it is the commonest vasculitis in children
•It is slightly more common in males, M: F, 1.4:1

37. Radiographic features
Plain radiograph:
Abnormal findings are non-specific and include a
reticulogranular pattern, peribronchial
cuffing, pleural effusion, atelectasis and/or air
trapping
Rarely, a few years after resolution of the initial
episode, the patient may present with
calcified coronary artery aneurysms visible on the
chest x-ray

39. Coronary angiography / CT angiography
• Angiography is the most sensitive and
specific for vascular assessment
• May show small coronary aneurysms
or stenoses
• Coronary artery aneurysms can be
classified as:
• small: <5 mm
• medium: 5–8 mm
• large: >8 mm
MRI / MR angiography
• Useful in assessing myocardial perfusion,
wall thinning and aneurysms
Echocardiography
• Transthoracic echocardiography especially
useful in the approach to patients who fall
short of full clinical criteria

40. LAD

41. RCA
LAD

42. Systemic lupus erythematosus
Magnified, subtracted angiogram of the
hand shows areas of digital artery
narrowing with multiple occlusions
(arrows). here are no intraluminal filling
defects or other evidence of emboli.

43. Radiation arteritis
A, Normal pulmonary angiogram of the left lung. B, Left pulmonary angiogram from the same patient obtained 7 years
after radiation treatment for breast carcinoma shows narrowing, branch vessel occlusions and pleural thickening consistent
with late radiation fibrosis.