vasculitisa case-based approach

Luc van den Hauwe1, 2

T. van der Zijden1, 2, T. Van Thielen1, 2, R. Salgado1, M. Voormolen1,S. Dekeyzer1, S. Nicolay2, E. De Smet1, S. Vanden Bossche,

J.W. Van Goethem1, P.M. Parizel1

1Antwerp University Hospital - University of Antwerp, Antwerp/BE2AZ KLINA, Brasschaat/BE

lucvdhauwe@mac.com

vasculitisa case-based approach

Luc van den Hauwe1, 2

T. van der Zijden1, 2, T. Van Thielen1, 2, R. Salgado1, M. Voormolen1,S. Dekeyzer1, S. Nicolay1, E. De Smet1, S. Vanden Bossche1,

J.W. Van Goethem1, P.M. Parizel1

1Antwerp University Hospital - University of Antwerp, Antwerp/BE2AZ KLINA, Brasschaat/BE

lucvdhauwe@mac.com

financial disclosures

no relevant disclosures

overview• background – introduction: stroke in the young• dissections: extra- and intracranial• CNS vasculitis• black-blood vessel wall imaging• take home messages

background - introduction• both cervical dissections and vasculitis are important

causes of ischemic stroke in the young(er) patient

TOAST* classification5 subtypes of ischemic stroke

• large-artery atherosclerosis (embolus/thrombosis)• cardioembolism (high-risk/medium-risk)• small-vessel occlusion (lacune)• stroke of other determined etiology • stroke of undetermined etiology

*Trial of ORG 10172 in Acute Stroke Treatment

TOAST* classification5 subtypes of ischemic stroke

• large-artery atherosclerosis (embolus/thrombosis)• cardioembolism (high-risk/medium-risk)• small-vessel occlusion (lacune)• stroke of other determined etiology • stroke of undetermined etiology

*Trial of ORG 10172 in Acute Stroke Treatment

4. stroke of other determined etiology • cervical artery dissection: up to 25% of cases• fibromuscular dysplasia• moyamoya disease: 6%-15% of nonatheroslerotic vasculopathies• migraine• rare genetic and hereditary diseases:

– Fabry disease– CADASIL– MELAS– …

• vasculitis• …

4. stroke of other determined etiology • cervical artery dissection: up to 25% of cases• fibromuscular dysplasia• moyamoya disease: 6%-15% of nonatheroslerotic vasculopathies• migraine• rare genetic and hereditary diseases:

– Fabry disease– CADASIL– MELAS– …

• vasculitis• …

• ER: acute stroke– right hemiparesis

• CT: not available (maintainance)• MRI

– conventional sequences– perfusion (DSC-PWI)

a 42-year-old woman

• ER: acute stroke– right hemiparesis

• CT: not available (maintainance)• MRI

– conventional sequences– perfusion (DSC-PWI)

a 42-year-old woman

FLAIR

DWI

a 42-year-old woman

MR perfusion

rCBF rCBV MTT TTP

a 42-year-old woman

MR perfusion

rCBF rCBV T1-Gd TTP‘luxuary perfusion’

a 42-year-old woman

eccentric true lumen‘crescent sign’: submural hematoma

T2-wiFLAIR

a 42-year-old woman

T1-Gd

CTAvessel wall enhancement: inflammation?

inflammation

Pfefferkorn T et al. Stroke 2011;42:1563-1568

cerebral vasculitis

cerebral vasculitis• vasculitis: definition

• vasculitis: classification

• diagnosis - how to image vasculitis?

vasculitis vasculopathy• heterogeneous group of (multisystemic) disorders:

– degenerative– metabolic– inflammatory– embolic– coagulative– functional– systemic lupus erythematosus (SLE), moyamoya disease, sickle

cell disease, (PRES), hypertensive encephalopathy, preeclampsia/eclampsia, immunosuppressive drug-induced vasculopathy, …

inflammation of the vessel wall=

vasculitis

vasc

ulop

athi

es

cerebral vasculitis• vasculitis: definition

• vasculitis: classification

• diagnosis - how to image vasculitis?

cerebral vasculitisdefinition • group of diseases defined by the presence of inflammation and

necrosis of blood vessel wall, involving arteries and veins• CNS vasculitis is rare!!!• M=F, but:

– SLE and Sjögren: F– Behçet: M

• age:– more often in adults– children: Kawasaki disease, IgA vasculitis, vasculitis related to infection (e.g.

Varicella Zoster)

cerebral vasculitisdefinition • group of diseases defined by the presence of inflammation and

necrosis of blood vessel wall, involving arteries and veins• CNS vasculitis is rare!!!• M=F, but:

– SLE and Sjögren: F– Behçet: M

• age:– more often in adults– children: Kawasaki disease, IgA vasculitis, vasculitis related to infection (e.g.

Varicella Zoster)

is it??? Underestimated!!!

to discuss with M. Edjlali, Paris/FR

cerebral vasculitisdefinition • group of diseases defined by the presence of inflammation and

necrosis of blood vessel wall, involving arteries and veins• CNS vasculitis is rare!!!• M=F, but:

– SLE and Sjögren: F– Behçet: M

• age:– more often in adults– children: Kawasaki disease, IgA vasculitis, vasculitis related to infection (e.g.

Varicella Zoster)

cerebral vasculitis• cerebral ischemia with possible focal infarction is the major

neurological manifestation

• clinical manifestations:– headache– altered mental status: encephalopathy– seizures– cranial nerve palsies– focal neurological deficits

cerebral vasculitisclassification • according to etiology

– primary isolated CNS vasculitis, aka primary angiitis of the CNS (PACNS)– primary systemic vasculitis with CNS involvement– secondary vasculitis as a complication of systemic disorders

• according to the size of vessels involved– large vessels– medium vessels– small vessels

a 65-year-old woman• general deterioration, incontinence, weight loss• desoriented in time, bradyfrenia• laboratory findings:

– signs of inflammation: CRP, sedimentation– ANF, ANCA negative– infectious screening (toxo, CMV, EBV, Borrelia): negative– lupus anticoagulans: negative– cultures (urine and blood): negative

• cardiac investigation: -

PACNS

cerebral vasculitisclassification • according to etiology

– primary isolated CNS vasculitis, aka primary angiitis of the CNS (PACNS)– primary systemic vasculitis with CNS involvement– secondary vasculitis as a complication of systemic disorders

• according to the size of vessels involved– large vessels– medium vessels– small vessels VU University Medical Centre, Amsterdam/NL

VU University Medical Centre, Amsterdam/NL

aorta & main branches

ECA, ICA & VBAA1-M1-P1

cerebral arteries distal to the bifurcation of the MCA

ACom & PCom Aa

imaging features of cerebral vasculitis• vasculitis: definition

• vasculitis: classification

• diagnosis - how to image vasculitis? • laboratory findings• imaging• brain biopsy

imaging features of cerebral vasculitis• vasculitis: definition

• vasculitis: classification

• diagnosis - how to image vasculitis? • laboratory findings• imaging• brain biopsy

• DSA catheter angiography• MRI – MRA• CT – CTA

y findings

psy••

That’s it?

That’s the best you got?

Lin et al. 2017Lin et al. 2017

Lin A et al. Neurosurgery 2017 Epub

cerebrovascular imagingimaging the vessel lumen: luminal imaging• DSA catheter angiography• CTA• MRA angiography

• limitations: – only depicts the arterial lumen, not the culprit

vessel wall itself– technical pitfalls

Lin A et al. Neurosurgery 2017 Epub Egar Moniz 1927

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luminal narrowing

cerebrovascular imagingimaging the vessel lumen: luminal imaging• DSA catheter angiography• CTA• MRA angiography

• limitations: – only depicts the arterial lumen, not the culprit

vessel wall itself– technical pitfalls

Lin A et al. Neurosurgery 2017 Epub

pixel: 0.2 x 0.2 mm2

voxel: 0.5 x 0.5 x 0.5 mm3

voxel: 0.36 x 0.36 x 0.5 mm3

voxel: 0.2 x 0.2 x 0.2 mm3

@ 7T

3D TOF @7T

reconstructed voxel size: 0.2 x 0.2 x 0.2 mm

From: De Cocker LJ et al. NeuroImage 2016 Epub

cerebrovascular imagingimaging the vessel lumen: luminal imaging

– DSA catheter angiography– CTA angiography– MRA angiography

imaging the vessel wall: vessel wall imaging (VWI) – high-resolution– black-blood– 3T (& 7T)

Lin A et al. Neurosurgery 2017 Epub Egar Moniz 1927

WI))

PETblack-blood T1 MRblack-blood T1 MR

vasculitis

PETblack-blood (bb) T1 MRblack-blood (bb) T1 MR

vasculitis

black-blood (bb) PD MR

intracranial vessel wall imaging• still in its developmental stage

• growing number of research groups and publications

• excellent review papers!!!– Lindenholz A et al. Radiology 2018;286:12-28– Mandell DM et al. AJNR Am J Neuroradiol 2017;38:218-229– Bhogal P et al. Clin Radiol 2016;71:293-303

???

Lindenholz A et al. Radiology 2018;286:12-28

vessel wall imaging

ask the experts!!!

principal technical requirements• high signal-to-noise ratio (SNR)• high contrast-to-noise ratio (CNR)• high spatial resolution• reasonable scan time• PD, T2-w, T1-w (before and/or after Gd)• challenges:

– adequate suppression of signal from luminal blood and CSF– full brain coverage

3T 7T1.5T

high-resolution (HR) black-blood (bb) vessel wall imaging:

principal technical requirements• high signal-to-noise ratio (SNR)• high contrast-to-noise ratio (CNR)• high spatial resolution• reasonable scan time• PD, T2-w, T1-w (before and/or after Gd)• challenges:

– adequate suppression of signal from luminal blood and CSF– full brain coverage

3T 7T1.5T

high-resolution (HR) black-blood (bb) vessel wall imaging: vwi @3T

Lindenholz A et al. Radiology 2018;286:12-28

compressed sensing

cerebrovascular imaging: MRI

Egar Moniz 1927

complementary techniques!!!

imaging the ‘vessel lumen’: luminal imaging: 3D TOF– DSA catheter angiography– CTA angiography– MRA angiography

imaging the ‘vessel wall’: vessel wall imaging– pattern: concentric, eccentric, focal, nodular– distribution: solitary vs multifocal– vessel wall enhancement!

HR-VWI & intracranial vasculopathies• intracranial atherosclerosis• moyamoya disease• intracranial vasculitis• RCVS• aneurysms

submillimeter evaluationof the vessel walls

identify potential causes of luminal narrowing

imaging features of cerebral vasculitis• more than two stenoses in at least two separate vascular

distributions • ‘string-of-beads’ appearance

• multifocal areas of marked vessel wall enhancement• concentric >> eccentric

‘string-of-beads’ appearance

multifocal areas of marked vessel wall enhancemeconcenentrtricic >> eccentricc

Eur Radiol 2004;14:1005-1012

a 47-year-old man• clinically proven

PACNS• biopsy: inconclusive• segmental

narrowing on 3D TOF

• concentric VW enhancement ICA A1-M1 bifurcation

case courtesy: A. van der Kolk & J. Hendrikse, Utrecht/NLFrom: Lindenholz. et al. Radiology 2018; 286:12-28

FLAIR 3D TOF HR bb T1-Gd

HR-VWI & intracranial vasculopathies• intracranial atherosclerosis• moyamoya disease• intracranial vasculitis• RCVS• aneurysms

case courtesy: A. van der Kolk & J. Hendrikse, Utrecht/NL slide courtesy: M. Edjlali, Paris/FR

modified TOAST• cardioembolism• small-vessel occlusion • stroke of other determined etiology • stroke of undetermined etiology• large-artery atherosclerosis of the cervical arteries• intracranial atherosclerosis• intracranial artery dissection• vasculitis• reversible cerebral vasoconstriction syndrome• intracranial arteriopathy not otherwise specified (NOS))

• altered the etiologic classification in 55% of patients

• the proportion of patients classified as havingintracranial arteriopathy not otherwise specifieddecreased from 31% to 4%

• proportion classified as having intracranialatherosclerotic disease increased from 23% to 57%

• wall thickening– concentrically– eccentricaly

• enhancement– yes– no

some more (final) examples

vasculitis - cocaine

Case courtesy: Timo Krings, Toronto, CanadaFrom Geibprasert et al. AJNR 2010

Case courtesy: Timo Krings, Toronto, CanadaFrom Geibprasert et al. AJNR 2010

take home messages• stroke in the young

– dissection– vasculitis

• donut/bagel concept– ‘lumenography’– vessel wall imaging

• ‘push your technique’: compressed sensing

thank you for your attention!!!

lucvdhauwe@mac.com