Imaging of vasculitis Dr/Ahmed Bahnassy Consultant Radiologist PSMMC
Imaging of vasculitis
Dr/Ahmed Bahnassy
Consultant Radiologist
PSMMC
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.
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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
Pathological process
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 ,aneurysmvenous thrombosis (acute or chronic)4.what secondary effects of vascular affection are
present?e.g.: infarction,bowel ischaemia
5.Look in other sites for syndromic vasculitis
e.g. :Lungs (wegener granulomatosis).
6.Estimate disease activity
Ultrasound ( for accesible arteries.Ultrasound ( for accesible arteries.
CTCT
MRIMRI
PETPET
Differential diagnosis
Large vessel vasculitis
• Giant cell arteritis
• Takayasu’s arteritis
Medium vessel Vasculitis
• Poly Arteritis Nodosa
• Kawasaki’s vasculitis
Small vessel Vasculitis
Pauci-immune (ANCA mediated)Wegener’s GranulomatosisChurg Strauss vasculitisMicroscopic PolyangiitisImmune complex mediatedHenoch Schonlein PurpuraEssential Mixed CryoglobulinemiaSLE and other collagen c=vascular diseases related vascultis
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)
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.
Takayasu’s Arteritis
• Pulseless Disease
• Middle aged females
• Aorta and its branches mainly involved
• Subclavian vessels, Carotid vessels, Mesentric vessels
• Chronic and Relapsing course
Poly Arteritis Nodosa
• Renal arteries most commonly involved leading to renovascular hypertension
• Pulmonary vessels NEVER involved
• Association with patients ofo Hepatitis Bo Hairy cell leukemia
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
Pauci immune Vasculitis
Usually Pulmonary capillaritis PLUS Glomerulonephritis
•Granulomas +, Asthma + Churg Strauss
•Granulomas +, NO asthma Wegener’s
•NO granulomas, NO asthma Microscopic Polyangiitis
Wegener’s Granulomatosis
• Chronis sinusitis, Pulmonary nodules, Pulmonary cavities, Rapidly Progressive Glomerulonephritis
• Cutaneous vasculitis, Eye lesions may be present
• Non specific symptoms may predominate
Churg Strauss Vasculitis
• Asthma, Eosinophilia with pulmonary infiltrates , glomerulonephritis
• Myocardial involvement most common cause of death
Microscopic Polyangiitis
• Pulmonary alveolar capillariitis, glomerulonephritis
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
role of imaging ?
Essential Mixed Cryoglobulinemia
• 5 % of Chronic Hepatits C pts. Have EMC
• Cryoglobulins formed agianst HCV RNA
• Pulmonary, renal ( MPGN ), cutaneous vasculitis
Thromb Angiitis Obliterans
• Chronic heavy Smokers• Inflammation of arteries, veins, nerves• Upper and lower limb gangrene, Instep
claudication, rest pain
Other primary vasculitides
• Behcet’s disease (Recurrent OculoOroGenital ulcerations with vasculitis)
Other primary vasculitides
• Idiopathic Cutaneous vasculitis
• Isolated Vasculitis of CNS
• Relapsing Polychondritis
• Polyangiitis overlap syndromes (features of more than 1 vasculitis)
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.
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
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.
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