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