Radiologic Features of Large and Medium-sized Vessel Vasculitis

Jonathan Jacobs11/12/07

Introduction to vasculitis

Definition: Inflammation of blood vessel walls with leukocyte infiltration and reactive mural damage.

Large vessel vasculitis:Giant cell arteritis (GCA)Takayasu arteritis

Medium-sized vessel vasculitis:Polyarteritis nodosa (PAN)Kawasaki diseasePrimary angiitis of the CNS

Small vessel vasculitis:Churg-Strauss arteritisWegener’s granulomatosisMicroscopic polyarteritisHenoch-Schönlein purpuraEssential cryoglobulinemic vasculitisHypersensitivity vasculitisVasculitis secondary to connective tissue diseasesVasculitis secondary to viral infection

Spectrum of vasculitis

Ha et al

Patient 1: History

72 year-old woman without history of autoimmunity presents with:1) Absent pulses in her left arm2) Differing upper extremity systolic blood pressures (R 160, L 120)3) ESR 80

Patient 1: Chest MR Sagittal STIR

Arrows point to high T2 signal indicating aortic wall edema

PACS/BIDMC PACS/BIDMC

Patient 1: Chest MRA

Arrows point to late enhancement of aortic wall

PACS/BIDMC

Patient 1: MRA MIP of thoracic aorta

The small arrow marks the proximal left subclavian artery occlusion.

The long arrow marks a prominent collateral vessel from the vertebral artery to the subclavian artery.

MIP = Maximum intensity projection (algorithm selects largest value in all planes from the selected viewing angle)

PACS/BIDMC

Patient 1: MR Time-of-flight

Retrograde flow in the left vertebral artery is manifested as an absence of signal on time-of- flight sequence.

Time-of-flight sequence = A plane upstream in the arterial flow is excited and a downstream plane is imaged. As a result, the only signal in the imaged plane is due to blood that has just moved into that plane.PACS/BIDMC

Patient 1: Diagnosis

Aortic wall T2 STIR signal with late contrast enhancement indicates aortitis. Proximal subclavian artery occlusion is an extension of this process.

Diagnosis: Giant cell arteritis (GCA)

Note: Bilateral temporal artery biopsies were negative, but the patient’s aortitis, elevated ESR, and age were sufficient for a clinical diagnosis.

Differential for aortitisGiant cell arteritisTakayasu arteritisRheumatoid arthritisRheumatic feverAnkylosing spondylitisSystemic lupus erythematosusRelapsing polychondritisReiter syndromeSclerodermaPsoriasisUlcerative colitisBehcet diseaseInfection (streptococcus and staphylococcus)SyphilisRadiation

GCA: Clinical features

Mean age is 72Incidence is 1 in 500 among those over the age of 50GCA is difficult to distinguish from Takayasu arteritis by radiology or pathology; typically age is the key discriminant (GCA >50, Takayasu<40)Pathogenesis: CD4+ T-cell mediated granulomatous inflammation of arterial walls, initiating factors unknownPathology: T-cells and monocytes with variable numbers of multinucleated giant cells infiltrating all layers of the artery wall (most concentrated in the media)Distribution: Large and medium arteries, most commonly the thoracic aorta and carotid artery branches

GCA: Symptoms

1) Constitutional (fever, fatigue, weight loss)2) Headache3) Jaw claudication4) Vision loss5) Polymyalgia rheumatica6) Arm claudication7) Aortic aneurysms (thoracic > AAA), insuffiency, and dissection8) TIAs/strokes9) Subclavian steal syndrome

Prognosis: Self-limited over several months to several years, no overall increased mortality but 15-20% risk of loss of visionTreatment: Glucocorticoids

GCA: Diagnostic criteria

1990 American College of Rheumatology criteria (3/5 required):1) Age greater than or equal to 50 at onset2) Localized headache of new onset3) Tenderness of temporal artery or decreased pulse4) ESR greater than 50 mm/h5) Biopsy revealing necrotizing arteritis with multinucleated giant cells or

mononuclear predominanceThe ACR criteria have 94% sensitivity and 91% specificityGCA is diagnosed primarily by temporal artery biopsy, but if a patient is biopsy negative imaging is critical

GCA: Menu of tests

1) MR and MRA – Allows for oblique plane imaging, T2 allows for ready visualization of wall edema

2) CT and CTA – IV contrast CT can demonstrate wall thickening/irregularity and aneurysms

3) Conventional angiography – Almost completely supplanted by MRA

Tests under investigation: 1) PET2) Ultrasound

MRI: Aortic wall edema

Arrows outline regions of aortic wall edema on T2-weighted STIR images

Narvaez et alNarvaez et al

CT: Aneurysm and occlusion

Arrow identifies a thoracic aortic aneurysm on non-contrast CT

Arrows outline SMA wall thickening and occlusion on IV contrast CT

Hunder #4

Lockhart et al

Angiography: Subclavian stenosis

Hunder Up-to-date #4

Box highlights an extended region of subclavian stenosis. Note the extensive collaterals.

PET and US: Investigational approaches

Arrows show uptake of FDG in inflamed subclavian arteries

Hypoechoic halo around a temporal artery (above) and SMA (below) on color Duplex sonography. This represents mural edema.Seo et al

Seo et al

Lockhart et al

Patient 2: History

39 year-old male with one year of left arm and leg pain/numbness now presents with:1) Abdominal pain2) Unexplained 20 pound weight loss3) New renal insufficiency4) New HTN5) Bilateral LE sensory neuropathy on EMG6) Serologic evidence of HepB infection7) ESR 42

Patient 2: CT nephrogram

Note the many wedge- shaped defects that give the kidneys a patchy appearance.

CT nephrogram = CT scan of kidneys after IV contrast has opacified the renal parenchyma (vessels and tubules)

CAS/MGH

Patient 2: SMA angiography

Courtesy of Dr. Rabkin Courtesy of Dr. Rabkin

Late after contrast administration, aneurysms stand out because they retain contrast.

Note the multiple aneurysms (black dots) present in the SMA branches.

Renal vasculature

http://people.eku.edu/ritchisong/RITCHISO//kidneyarteries.jpg

Courtesy of Dr. Rabkin

Normal renal angiogram

Patient 2: Renal angiography

CAS/MGH CAS/MGH

Diffuse loss of perforating and interlobular arteries bilaterally, referred to as “pruning.”

Patient 2: Renal angiography late

CAS/MGH CAS/MGH

Marked perfusion defects are present bilaterally, resulting in “patchy” kidneys.

Patient 2: Diagnosis

The combination of SMA aneurysms and renal artery occlusions indicate a necrotizing vasculitis.

Diagnosis: Polyarteritis nodosa (PAN)

Note: The diagnosis was confirmed on renal biopsy, but angiography was sufficient to prompt initiation of treatment.

Differentials for aneurysms and pruning

Medium-sized artery aneurysms:Necrotizing vasculitisInfection (mycotic aneurysm)Intravenous drug abuse (e.g. heroin)TraumaFibromuscular dysplasia

Pruning of renal vasculature (i.e. multiple small vessel occlusions): Necrotizing vasculitisHypertensive nephrosclerosisScleroderma

PAN: Clinical features

Rare (prevalence 2-33/million)Most cases thought to be idiopathic, but some are associated with hepatitis B and hairy cell leukemiaPathogenesis: Vessel wall inflammation (trigger unknown) resultsin aneurysms from breakdown of elastic lamina and tissue infarction from luminal narrowing/occlusionPathology: Segmental transmural infiltration of muscular arteries by PMNs and mononuclear cells, resulting in fibrinoid necrosis. Distribution: Medium-sized arteries anywhere in the body. Spares arterioles, capillaries, and venules.

PAN: Symptoms

1) Constitutional – Fever, fatigue, weight loss2) Renal – Renal insufficiency, renal infarction, perirenal

hematomas, renin/angiotensin mediated hypertension3) Gastrointestinal – Mesenteric ischemia, bowel

infarction/perforation (generally small intestine)4) Neurologic – Mononeuropathy multiplex5) Coronary – Myocardial ischemia, ischemic cardiomyopathy6) Dermatologic – Ulcers, gangrene, vesicles, palpable purpura,

erythematous nodules, livedo reticularis7) Musculoskeletal – Claudication, weakness, arthralgia

Prognosis: 50% 1-year and 13% 5-year survival if untreated; 80% 5-year survival if treatedTreatment: Glucocorticoids, cyclophosphamide

Diagnostic criteria for PAN1990 American College of Rheumatology criteria (3/10 required):

1) Unexplained weight loss greater than 4 kg2) Livedo reticularis3) Testicular pain or tenderness4) Myalgia (excluding hip and shoulder), muscle weakness, muscle

tenderness5) Mononeuropathy or polyneuropathy6) New onset diastolic blood pressure greater than 90 mmHg7) Elevated BUN (>40 mg/dL) or creatinine (>1.5 mg/dL)8) Hepatitis B infection9) Characteristic angiographic appearance10) Biopsy of small or medium-sized artery demonstrating infiltrating PMNs

ACR criteria has sensitivity and specificity of 82% and 87%

PAN: Menu of tests

1) Conventional angiography – Gold standard, sensitivity and specificity of 89% and 90%

2) CT and CTA3) MR and MRA

PAN: Radiographic features

1) Microaneurysms at vessel bifurcations (60% of patients have aneurysms)

2) Focal short segments of arterial occlusion (occlusions found in nearly every PAN patient)

3) Multiple small wedge-shaped renal infarcts (90% of patients have renal involvement)

Angiography: Renal aneurysms

Savage et al

The arrows mark prominent capsular arteries supplying the renal cortex.

Helenon et al

Angiography: Occlusions

Small vessel occlusions give the kidney a “pruned” appearance.

The left arrow shows occlusion of a hepatic artery branch, the right arrow points to an aneurysm, and the arrowhead points to a region of luminal irregularity.

Stanson et alStanson et al

MR: Digital artery occlusion

Long arrows identify digital artery occlusions and short arrows identify aneurysms on this MIP image.

Conventional angiography of the same hand for comparison

Connell et al

Connell et al

CT: SMA thickening and branch occlusion

Kato et al for all three images

Arrow in A points to SMA wall thickening shown on IV contrast CT. Arrowheads in B point to a SMA branch occlusion shown on MIP. The same occlusions on angiography are shown for comparison.

CT: Perirenal hematoma

Kawashima et al

Aneurysm rupture can cause intrarenal, subcapsular, or perinephric hematoma (shown here on IV contrast CT).

Note the lobulated appearance of the kidneys. This is due to scarring secondary to multiple past infarcts.

Patient 3: History

66 year-old woman without history of autoimmunity who presents with:1) One year of intermittent right arm and leg myalgia2) Unexplained ten pound weight loss3) ESR 72

Patient 3: Abdominal MRA

Box highlights enhancement of the abdominal aortic wall

PACS/BIDMC

Patient 3: Abdominal MR

Box highlights mural edema of abdominal aorta on T2-weighted image

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Patient 3: MIP

Box highlights region of marked luminal irregularity of infrarenal aorta

PACS/BIDMC

Patient 3: Diagnosis

Giant cell arteritis!

Note: No temporal artery biopsies were attempted as imaging was adequate for a clinical diagnosis.

Patient 4: History

53 year-old male with PEG tube presents with two weeks of:1) Abdominal pain2) Coffee grounds in the PEG (EGD negative)3) BRBPR

Patient 4: Abdominal CT with IV contrast

Box highlights splenic artery constriction

PACS/BIDMC

Patient 4: Abdominal CTA further view

Box 1 = Marked wall thickening around hepatic artery

Box 2 = Splenic artery aneurysm

Box 3 = Common hepatic artery aneurysm

PACS/BIDMC

Patient 4: Coronal MIP

Box #1 = Proper hepatic artery aneurysm

Box #2 = Splenic artery aneurysm

PACS/BIDMC

Patient 4: 3D volume rendering

3D volume rendering depicts the surface of vessels from CTA, highlighting the patient’s multiple aneurysms

PACS/BIDMC

Patient 4: Diagnosis

Polyarteritis nodosa!

Note: CTA findings led to a diagnosis and initiation of steroid treatment. No further diagnostic studies were done.

Acknowledgements

Dr. Gillian LiebermanMaria LevantakisDr. Andrew BennettDr. Dmitry RabkinFellow students

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