2. WHAT IS VASCULITIS?
• Vasculitis is a clinicopathologic
process characterized by inflammation
and damage to blood vessels,leading
to compromise of the vascular lumen
resulting in ischemia of the tissues
supplied by the involved vessels.
3. 3
pathogenesis
• Immune complex production &
deposition
• Production of ANCA
• T-Lymphocyte response and
granuloma formation
group of autoantibodies, mainly IgG ,
detected in autoimmune disorders,
particularly systemic vasculitis
5. Steps of thinking
1.Which vessels are affected ?
arteries,veins ,or both.
2.which arteries are affected?
Large,medium or small sized.
3.How vessels are affected?
stenosis ,occlusion ,aneurysm
venous thrombosis (acute or chronic)
4.what secondary effects of vascular affection are
present?
e.g.: infarction,bowel ischaemia
6. 5.Look in other sites for syndromic vasculitis
e.g. :Lungs (wegener granulomatosis).
6.Estimate disease activity
Ultrasound (( ffoorr aacccceessiibbllee aarrtteerriieess..
CCTT
MMRRII
PPEETT
10. Small vessel Vasculitis
Pauci-immune (ANCA mediated)
Wegener’s Granulomatosis
Churg Strauss vasculitis
Microscopic Polyangiitis
Immune complex mediated
Henoch Schonlein Purpura
Essential Mixed Cryoglobulinemia
SLE and other collagen c=vascular diseases
related vascultis
11. Other primary vasculitides
• Thromb Angiitis Obliterans
• Behcet’s disease
• Idiopathic Cutaneous vasculitis
• Isolated Vasculitis of CNS
• Relapsing Polychondritis
• Polyangiitis overlap syndromes (features
of more than 1 vasculitis)
12. Giant cell arteritis
• Temporal arteritis
• Elderly persons more than 50 yrs. of age
• Non specific symptoms, Headache,
Elevated ESR
• BLINDNESS-most serious complication
• Jaw claudication, Scalp pain, Scalp
Tenderness
• Polymyalgia Rheumatica.
13. Takayasu’s Arteritis
• Pulseless Disease
• Middle aged females
• Aorta and its branches mainly involved
• Subclavian vessels, Carotid vessels,
Mesentric vessels
• Chronic and Relapsing course
14. Poly Arteritis Nodosa
• Renal arteries most commonly involved
leading to renovascular hypertension
• Pulmonary vessels NEVER involved
• Association with patients of
o Hepatitis B
o Hairy cell leukemia
15. Kawasaki’s Vasculitis
• MucoCutaneous Lymph node
syndrome
• Children < 5 years of age
mostly
• Desquamative erythematous
rashes involving the skin,
mucus membranes, cervical
lymphadenopathy
• 25 % develop coronary artery
aneurysms in the convalescent
stage of the illness
16. Pauci immune Vasculitis
Usually Pulmonary capillaritis PLUS
Glomerulonephritis
•Granulomas +, Asthma + Churg Strauss
•Granulomas +, NO asthma Wegener’s
•NO granulomas, NO asthma
Microscopic Polyangiitis
17. Wegener’s Granulomatosis
• Chronis sinusitis, Pulmonary nodules,
Pulmonary cavities, Rapidly Progressive
Glomerulonephritis
• Cutaneous vasculitis, Eye lesions may be
present
• Non specific symptoms may predominate
18.
19. Churg Strauss Vasculitis
• Asthma, Eosinophilia with pulmonary
infiltrates , glomerulonephritis
• Myocardial involvement most common
cause of death
Microscopic Polyangiitis
• Pulmonary alveolar capillariitis,
glomerulonephritis
20.
21. Henoch Schonlein Purpura
• 2nd decade
• Palpable purpura over
lower limbs,
• Gastrointestinal
complaints (abd.colicky
pain, blood in stools),
• Fever, polyarthralgia
• Increased IgA levels in
blood
27. Other primary vasculitides
• Idiopathic Cutaneous vasculitis
• Isolated Vasculitis of CNS
• Relapsing Polychondritis
• Polyangiitis overlap syndromes (features
of more than 1 vasculitis)
28. Pearls in requesting
imaging study
Angiography is useful in demonstrating vessel lumen alterations such
as stenoses or aneurysms. However, it is unable to reveal initial
vasculitic lesions such as vessel wall oedema and thickening, and is
thus not useful to diagnose vasculitis early.
29. CDS, CT angiography, MRI and MRA are
able to delineate both the vessel wall
and the lumen. Therefore, they can reveal
vessel wall alterations when the lumen is
still unaffected on angiography
30. 18 FDG PET does not
delineate the vessel wall,
but is very sensitive in
revealing
vessel wall inflammation.
It is particularly useful to
make a diagnosis and
disclose the extent of
large vessel vasculitis, as
well as to monitor the
disease course.
31. other pearls
Not every vessel with active disease on
imaging will develop structural changes
Vessels that appear to be unaffected on
imaging at a given time may still develop
alterations at a later stage
CT is the investigation of choice for
demonstrating lung lesions
MRI is the most appropriate technique to
study brain involvement
MRI has shown promising results in the
evaluation of muscle disease