Updated Classification Criteria for the Large Vessel Vasculitis...4. SBP difference >10mmHg in arms...

Updated Classification Criteria for the

Large Vessel Vasculitis

Lead Investigators

Raashid Luqmani, University of OxfordPeter A. Merkel, University of Pennsylvania

Richard Watts, University of East Anglia

• Cristina Ponte – None

• Jo Robson – None

• Peter Grayson – None

• Peter Merkel – None

• Raashid Luqmani – None

• Ravi Suppiah – None

• Richard Watts – None

Disclosures

1. Hunder GG et al. The American College of Rheumatology 1990 criteria for the

classification of giant cell arteritis. Arthritis Rheum 1990.

2. Arend WP et al. The American College of Rheumatology 1990 criteria for the

classification of Takayasu arteritis. Arthritis Rheum 1990.

3. Jennette JC et al. Nomenclature of systemic vasculitides. Proposal of an international

consensus conference. Arthritis Rheum 1994.

4. Jennette JC et al. 2012 Revised International Chapel Hill Consensus Conference

Nomenclature of Vasculitides. Arthritis Rheum 2013.

Evidence based

Prior Classification/Nomenclature Schemes in Vasculitis

Not Diagnostic

Criteria!

Until final endorsement by the ACR & EULAR, these criteria are not final, may change, and are

not appropriate for use in research or for clinical purposes

Acknowledgements

136 sites in 32 countries

Expert panel reviewers 2018

Alba, Marco Duftner, Christina Jennette, Charles Pagnoux, Christian

Barra, Lillian Emmi, Giamoco Juche, Aaron Salvarani, Carlo

Basu, Neil Faurschou, Mikkel Karadağ, Ömer Schmidt, Wolfgang

Blockmans, Daniel Flores-Suárez, Luis Felipe Kermani, Tanaz Sharma, Aman

Brouwer, Elisabeth Gatenby, Paul Khalidi, Nader Sivakumar, Rajappa

Buttgereit, Frank Geraldes, Ruth Koster, Matthew Springer, Jason

Camellino, Dario Gewurz-Singer, Ora Macieira, Carla Sunderkötter, Cord

Chrysidis, Stavros Guillevin, Loïc Matsui, Kazuo Terslev, Lene

Cid, Maria Hammam, Nevin Milchert, Marcin Tesar, Vladimir

Daikeler, Thomas Hauser, Thomas Molloy, Eamonn Tomasson, Gunnar

de Boysson, Hubert Hellmich, Bernhard Monti, Sara Vaglio, Augusto

de Miguel, Eugenio Henes, Joerg Neumann, Thomas Warrington, Kenneth

Dejaco, Christian Hinojosa, Andrea Nielsen, Berit

Diamantopoulos, Andreas Hocevar, Alojzija Novikov, Pavel

Direskeneli, Haner Holle, Julia

Acknowledgements

Andrew JudgeUniversity of Bristol

Sara KhalidUniversity of Oxford

Andrew HutchingsLondon School of Hygiene & Tropical

Medicine

Katherine (Bates) GribbonsNational Institutes of Health

Statisticians

Acknowledgements

Clinical Research FellowsAlberto FloresAna RodriguesBen Seeliger

Hannah QuerinJan Sznajd

Kasia Wawrzycka-AdamczykSara Monti

Takahiro Nunokawa

Data ManagementDavid Gray

Ann-Marie MorganIssi Platt

Database designJoe Barrett

Joe RosaNathanael Gray

Site communications & paymentsMarian Montgomery

Study ManagementAnthea Craven

Acknowledgements

DCVAS is sponsored by the University of Oxford and supported in the UK by theNIHR Clinical Research Network

DCVAS is funded by the American College of Rheumatology, the European League Against Rheumatism, and

the Vasculitis Foundation

Acknowledgements

New Classification Criteria for the Large Vessel Vasculitis (LVV)

Overview

1. Why do we need new classification criteria for LVV?

2. Methodology

3. Results

4. Draft classification criteria for GCA and TAK

5. The changing face of LVV

Why do we need new classification criteria for LVV?

Presenter

Ravi Suppiah, Auckland District Health Board

Why do we need new classification criteria?

• 1990 ACR criteria

Arend WP et al. Arthritis Rheum 1990

Giant cell arteritis Takayasu’s arteritis

1. Age ≥50 at onset2. New headache3. TA tenderness or decreased

pulsation4. ESR ≥ 50 mm/hr5. Artery biopsy consistent with

vasculitis

1. Age at onset ≤ 40 years2. Claudication of extremities3. Decreased brachial artery pulse4. SBP difference >10mmHg in arms5. Bruit over subclavian or abd aorta6. Arteriographic narrowing or

occlusion of the aorta and its primary branches

3/5 needed for classification 3/6 needed for classification

Hunder GG et al. Arthritis Rheum 1990


• Reduced sensitivity in modern cohorts

GCA TAK

Sensitivity Specificity Sensitivity Specificity

Original ACR 1990 cohort

93.5% 91.2% 90.5% 97.8%

Preliminary DCVAS cohort (N=1005)

81.1% 94.9% 73.6% 98.3%

Seeliger et al. Rheumatology 2017


• Modern imaging modalities – MRI / MRA


• Growth in availability of MRI scanners

Cheung et al. Agency for Healthcare Research and Quality (US) 2011

ACR 1990 criteria

DCVAS


• Modern imaging modalities – FDG-PET CT


• Modern imaging modalities – Ultrasound

Temporal artery

Axillary artery – Longitudinal and transverse view


• ACR 1990 Criteria not suitable for modern trials

GIACTATrial

(1) Age ≥50 years(2) History of ESR ≥50 mm/hour(3) And at least one of the following:

(a) Unequivocal cranial symptoms of GCA (new onset localized headache, scalp or temporal artery tenderness, ischemia-related vision loss, or otherwise unexplained mouth or jaw pain upon mastication)

(b) Unequivocal symptoms of PMR, defined as shoulder and/or hip girdle pain associated with inflammatory stiffness

(4) And at least one of the following:(a) Temporal artery biopsy revealing features of GCA(b) Evidence of large-vessel vasculitis by angiography or cross-sectional

imaging study such as MRA, CTA, or PET-CT

Unizony et al. Int J of Rheumatology 2013

TIME FOR AN UPDATE

Methodology for developing the LVV classification criteria

Presenter

Peter Grayson, United States National Institutes of Health

Three phases

Methodology

Case selection

• Clinical vignette expert panel review

• Avoid circularity of applying “gold standard”

• Quality control

Item selection

• Data-driven item reduction

• Clinical consensus item reduction

Draft criteria

• Combined data-driven and clinical expert review

• Specific type of analysis - Lasso logistic regression

• Model performance: face validity, discrimination

Clinical vignette expert panel reviewCase selection

Methodology

Case selection

Methodology

Web-based review system

Vasculitis?Vessel size?

Large vessel vasculitis?

Subtype?

Expert reviewers were asked:

• Level of certainty: very certain, moderately certain, uncertain, or very uncertain

• Match on subtype (≥ moderate certainty) - Case passed

• No match - Second review by DCVAS committee member

Methodology

Data-driven and expert consensusItem selection

>8000 DCVAS items (including combinations)

Retained important items for regression analysis(as individual or combined items)

Assessed for frequency and clinical relevance

Vascular Exam

Clinical Symptoms & Labs

Biopsy

Vascular Imaging

Data Reduction

• Bruit

• Blood pressure differences

• Pulse abnormality

Upper-limb

Lower-limb

• Territory specific exam abnormality

Carotid

Temporal artery

Vascular Exam: Data Reduction

Arterial Territories:

Vascular Exam Categories:

111 Vascular Exam Combinations

• Temporal artery• Carotid• Subclavian• Axillary• Brachial• Radial

• Femoral• Popiteal• Posterior tibia• Dorsalis pedis• Abdominal aorta• Renal

• Tenderness• ’Cord-like’

Temporal arteries

• Diminished pulse• Absent pulse• Bruit

Definite Vasculitis (n = 427):

• Giant cells

• Granuloma

• Mononuclear infiltrate

• Necrotizing arteritis with necrosis

Possible Vasculitis (n = 115):

• Vascular thrombosis

• Fragmentation internal elastic membrane

• Adventitial/vaso vasorum inflammation

• Intimal hyperplasia

Temporal Artery Biopsy: Data Reduction

• Extravascular granulomatous inflammation

• Extravascular eosinophil-predominant inflammation

• Extravascular non-granulomatous acute or chronic inflammation

• Calcification

Extravascular Findings:

44 Diagnostic FindingsSpecific Findings:• Giant cells• Granuloma• Necrotizing

arteritis/arteriolitis/venulitis• Perivascular inflammation • Immune complex deposits• Prominent neutrophils

• Prominent eosinophils • Mononuclear leukocytes • Vascular scarring • Fragmentation of the

internal elastic lamina• Intimal thickening• Vascular thrombosis

Vascular Imaging: Data Reduction

• Vessel narrowing• Vessel occlusion• Wall thickening• Delayed Contrast

enhancement• Enhanced FDG uptake

• Aneurysm• Dissection• Beading• Calcification of vessel• Thrombus present• Halo sign positive

Arterial Involvement:

1,962 Vascular Imaging Combinations

• Ascending aorta• Thoracic aorta• Abdominal aorta• Axillary• Carotid

• Iliofemoral• Mesenteric• Ophthalmic• Pulmonary• Renal

• Subclavian• Temporal• Vertebral• Distal leg• Distal arm

Arterial Territories:

• CT• CT angio• MRI

• MR angio• PET/ PET CT• Fluorescein angio

• Ultrasound• Catheter-based

dye angio

Imaging Modalities:

• Stenosis• Occlusion• Arterial FDG

uptake

• Aneurysm• Halo sign positive

Definition of Involvement:

• Ultrasound

• PET

• Angiogram (MR, CT, catheter)

Modalities of Interest:

• Thoracic aorta• Abdominal aorta• Axillary• Carotid• Iliofemoral

• Mesenteric• Renal• Subclavian• Temporal• Vertebral

Territories of interest:

GCA Specific PatternsTAK Specific Patterns

Angiogram and Ultrasound PET

Arterial Patterns Specific for GCA or TAK

Methodology

Statistical methodsDraft criteria

• Disease specific comparators (other LVV, PAN, PACNS, mimics)

• Development (70%) and validation (30%) datasets

• Too many predictors for standard regression analysis, even after

data reduction

• Specific type of analysis - Lasso logistic regression

Menelaos Pavlou et al. BMJ Research Methods and Reporting 2015

General results for developing the LVV classification criteria

ResultsClinical vignette expert panel review 2018 Case

selection

DCVAS cases with submitting physician diagnosis high/ moderate confidence (N=2131)

56 external expert reviewers

7 DCVAS committee reviewers

No agreement REJECTED N= 436

Committee agrees(expert OR DCVAS)

AgreementPASSED N= 1695 (80% all cases)

Expert does not agree N= 867 (41%) Expert agrees N=1264 (59%)

TOTAL CASES AVAILABLE N= 2068(included 373 cases passing review 2016)

Data-driven and expert consensusItem selection

>8000 DCVAS items (including combinations)

89 items retained for lasso regression analysis

Results

Draft criteria

*Behçet’s disease, PACNS, isolated aortitis, LVV cannot subtype, relapsing polychondritis

After expert reviewer process

Cases and comparators used for analysis

Results

TAK462

PAN77

Other vasculitis*

198 LVV vasculitis mimics

342GCA942

GCA vs TAK Age at Diagnosis

≥ 60 years of age40-60 years of age≤ 40 years of age

Draft criteria

Results

Age as almost a perfect predictor

Takayasu’s arteritis

Giant cell arteritis

Age at diagnosis TAK GCA Total

< 40 354 3 357

40 to 60 105 74 179> 60 3 865 868Total 462 942 1,404

Draft criteria

Results

Age as almost a perfect predictor

Takayasu’s arteritis

Giant cell arteritis

Age at diagnosis TAK GCA Total

< 40 354 3 357

40 to 60 105 74 179> 60 3 865 868Total 462 942 1,404Absolute inclusion criteria

TAK: ≤ 60 years of age at diagnosis

GCA: ≥ 40 years of age at time of diagnosis

Draft classification criteria for Giant Cell Arteritis and Takayasu’s Arteritis

Presenter

Cristina Ponte, Hospital de Santa Maria, Lisbon

Newly-derived classification criteria

Draft classification criteria

Giant Cell Arteritis

Takayasu’s Arteritis

Draft criteria

Development set Validation set Total

GCA 518 238 756

Comparators 536 213 749

Total 1054 451 1505

Draft classification criteria for GCA

Data split of development and validation sets (70% : 30%)

Description Odds Ratio (95%CI) P-valueDefinite vasculitis on TAB 222.2 (50.4, 979.4)

GCA vs TAK Temporal Artery Biopsy

Definite VasculitisProbable VasculitisNot Vasculitis, No Biopsy

Halo SignGCA vs TAK

Halo Sign Positive

Halo Sign Negative or No Ultrasound

Risk factor at baseline Risk scoreDefinite vasculitis on TAB +5Halo of the temporal arteries on ultrasound +5FDG-PET activity throughout aorta +3Maximum ESR ≥50 mm/hour or CRP ≥10 mg/L +3Bilateral axillary involvement on imaging +3Morning stiffness in shoulders or neck +2Possible vasculitis on TAB +2Sudden visual loss +2New temporal headache +2Scalp tenderness +2Jaw or tongue claudication +2Temporal artery abnormality on vascular examination +1

Draft classification criteria for GCAPoints based risk score


DevelopmentN=1054 (70%)

Cut-off ≥ 6 points

SensitivitySpecificity

89.96%93.84%

AUC (95%CI)

0.97 (0.96 to 0.98)

Model Performance characteristics

Validation N=451 (30%)



89.08%91.55%

AUC (95%CI)

0.96 (0.95 to 0.98)


Performance Characteristics by GCA Subgroup

Patient Subgroup N AUC (95%CI) Sensitivity Specificity

All GCA 1,505 0.97 (0.96 to 0.98) 89.68% 93.19%

Biopsy-proven GCA 1192 0.99 (0.99 to 1.00) 98.87% 93.19%

Large-vessel GCA 860 0.89 (0.86 to 0.93) 66.67% 93.19%

Inclusion Criteria: The following must be met to be considered for classification

Draft Giant Cell Arteritis Classification Criteria

Criteria: ≥ 6 points meets threshold for classification

Clinical FeaturesMorning stiffness in shoulders or neck

Sudden visual loss

Jaw or tongue claudication

New temporal headache

Scalp tenderness

+2

+2

+2

+2

+2

Clinical Features

Definite vasculitis

Possible vasculitis+5

+2

Temporal Artery Biopsy (select one)

Temporal artery halo sign (US)

Bilateral axillary involvement

FDG-PET activity throughout aorta

+5

+3

+3

Imaging Findings

LaboratoryESR ≥ 50mm/hour or CRP ≥10mg/L +3

Laboratory Findings

Vascular ExamReduced pulse, ‘cord-like’, or tenderness +1

Temporal Artery Exam Findings

Diagnosis of vasculitis ≥ 40 years of age at time of diagnosis

Newly-derived classification criteria

Draft classification criteria

Giant Cell Arteritis

Takayasu’s Arteritis

Draft criteria

Development set Validation set Total

TAK 316 146 462

Comparators 323 127 450

Total 639 273 912

Data split of development and validation sets (70% : 30%)

Draft classification criteria for Takayasu’s arteritis

Description Odds Ratio (95%CI) P-valueAbd. aorta, and renal or mesenteric arteries on imaging 15.5 (4.7, 51.5)

Two TerritoriesOne TerritoryNo large-vessel involvement

Three or More Territories

GCA vs TAK Territories Involved

Risk factor at baseline Risk scoreAbd. aorta, and renal or mesenteric arteries on imaging +3Number of affected arteries on imaging - three or more +3Angina or ischemic cardiac pain attributable to vasculitis +2Reduced pulse in upper extremity +2Arm or leg claudication +2Carotid – reduced pulse or tenderness +2Arterial bruit +2

Number of affected arteries on imaging - two +2SBP difference in arms ≥ 20mmHg +1Vasculitis affecting paired branch arteries on imaging +1Female sex +1Number of affected arteries on imaging - one +1

Points based risk score



DevelopmentN=639 (70%)



90.82%92.26%

AUC (95%CI)

0.96 (0.95 to 0.98)

Model Performance characteristics

Validation N=273 (30%)



94.52%93.70%

AUC (95%CI)

0.98 (0.97 to 1.00)


Draft Takayasu’s Arteritis Classification Criteria


Clinical Features:Female sex

Angina or ischemic cardiac pain attributable to vasculitis

Arm or leg claudication

+1

+2. .

+2

Angiography and Ultrasound

Number of affected arteries (select one):

One arteryTwo arteries Three or more arteries

Vasculitis affecting paired branch arteries

Abdominal aorta involvement with renal or mesenteric involvement

A

A

+1+2+3

+1.

+3

Vascular Exam:Arterial bruit

Reduced pulse in upper extremity

Carotid – reduced pulse or tenderness

SBP difference in arms ≥ 20mmHg

+2

+2 +2

+1

Clinical Features

Vascular Exam Findings

Angiographic and Ultrasound Findings

Diagnosis of vasculitis ≤ 60 years of age at diagnosis Evidence of vasculitis on imaging

Draft classification criteria for TAK and GCA

Age in the new classification criteria – the 40-60 years interval

The Changing Face of Large-Vessel Vasculitis

Presenter

Ravi Suppiah, Auckland District Health Board

• Largest study to date in vasculitis

• Reflects how the international community currently thinks about large vessel vasculitis

• Different regions of the world represented– Important because of different clinical phenotypes in different regions

– Different clinical practices by region


• International effort

All patients from North America

(including Mexico)

TAK = 63 patientsGCA = 214 patients


• ACR 1990 Cohort

Arend WP et al. Arthritis Rheum 1990Hunder GG et al. Arthritis Rheum 1990

More Global Population

DCVASTAK = 462GCA = 942

ACR 1990TAK = 63 patientsGCA = 214 patients


• DCVAS Cohort

India North America and Japan


• Different Patterns of Disease in TAK

Yajima et al. Jpn Circ J. 1994; Moriwaki et al. Angiology 1997; Jain et al. Int J Cardiol 2000

• 14 year old female• Presented with:

– Fatigue– Significant weight loss– Acute-onset, left-sided vision loss– Hypertension

• Angiography (MRA):– Moderate stenosis of carotids– Severe bilateral renal stenosis– Severe abdominal aorta stenosis

Stenotic

abdominal aorta

Occluded right

renal artery

Total of 5 vessels


• Case example

• 1990 ACR criteria for Takayasu’s arteritis


1. Age at onset ≤ 40 years2. Claudication of extremities3. Decreased brachial artery pulse (1 or both)4. >10mmHg systolic BP difference in arms5. Bruit over subclavian or abdominal aorta6. Arteriographic narrowing or occlusion of the aorta, primary

branches, or large vessels in extremities

3/6 needed for classification of TAK








+1

+2. .

+2






A

A

+1+2+3

+1.

+3





+2

+2 +2

+1

Clinical Features



✔

8 points

Diagnosis of vasculitis ≤ 60 years of age at diagnosis Evidence of vasculitis on imaging✔✔

• Expanding Clinical Phenotype

• Improved sensitivity of new criteria

• What does the case highlight?


• If biopsy positive, why do you need other features?

• If positive halo on US, why do you need other features?


• Inter-observer agreement for evaluation of TAB and US

Luqmani et al. Health Technology Assessment 2016

0

1

2

3

4

5

6

7

8

9

10

11

12

fre

qu

en

cy

GCA-negative GCA-positive

0

1

2

3

4

5

6

7

8

9

10

11

12

13

14

fre

qu

en

cy

GCA-negative GCA-positive

Moderate agreement between 14 pathologists (kappa 0.62) assessing 30 biopsyimages and 12 sonographers (kappa 0.61) assessing 30 ultrasound videos

Biopsy Ultrasound


Data driven exercise to define

• Definitions for TAB Interpretation into classification


Possible Vasculitis (n = 115):

• Vascular thrombosis

• Fragmentation internal elastic membrane

• Adventitial/vaso vasorum inflammation

• Intimal hyperplasia

Definite Vasculitis (n = 427):

• Giant cells

• Granuloma

• Mononuclear infiltrate

• Necrotizing arteritis with necrosis

• 70 year old woman

• Temporal headache

• Arm claudication

• CRP 40 mg/dL

• TA biopsy – ‘Possible vasculitis’

• PET CT – FDG uptake


• Case example 2





Sudden visual loss



Scalp tenderness

+2

+2

+2

+2

+2

Clinical Features

Definite vasculitis


+2





+5

+3

+3

Imaging Findings


Laboratory Findings



✔

13 points

Diagnosis of vasculitis ≥ 40 years of age at time of diagnosis✔

• 49 year old, Caucasian, male

• Profound fatigue

• Background of Psoriatic arthritis, in remission off therapy


• Case example 3

• CRP 52 mg/L (

Narrowing and halo of the BOTH axillary arteries

Ultrasound


• Case example 3

Do you agree that he has a LVV?


• Case example 3

How would you classify this patient?

A. Takayasu’s arteritis

B. Giant cell arteritis

C. Other form of LVV

• 1990 ACR criteria


Giant cell arteritis Takayasu’s arteritis

1. Age ≥50 at onset2. New headache3. TA tenderness or decreased

pulsation4. ESR ≥ 50 mm/hr5. Artery biopsy consistent with

vasculitis

1. Age at onset ≤ 40 years2. Claudication of extremities3. Decreased brachial artery pulse4. SBP difference >10mmHg in arms5. Bruit over subclavian or abd aorta6. Arteriographic narrowing or

occlusion of the aorta and its primary branches

3/5 needed for classification 3/6 needed for classification

Hunder GG et al. Arthritis Rheum 1990


GCA Specific PatternsTAK Specific Patterns

Angiogram and Ultrasound PET

Arterial Patterns Specific for GCA or TAK





Sudden visual loss



Scalp tenderness

+2

+2

+2

+2

+2

Clinical Features

Definite vasculitis


+2





+5

+3

+3

Imaging Findings


Laboratory Findings



✔

6 points

Diagnosis of vasculitis ≥ 40 years of age at time of diagnosis✔







+1

+2. .

+2






A

A

+1+2+3

+1.

+3





+2

+2 +2

+1

Clinical Features



✔

3 points


MRA showed narrowing of :- left subclavian artery , right distal

subclavian- bilateral axillary and brachial artery - vertebral artery origins - prox. right common carotid artery


• Case example 3 – what if the patient also had an MRA?

Went back and examined patient:- Reduced arm pulses


• Case example 3 – with new findings

How would you classify this patient?

A. Takayasu’s arteritis

B. Giant cell arteritis

C. Other form of LVV







+1

+2. .

+2






A

A

+1+2+3

+1.

+3





+2

+2 +2

+1

Clinical Features



✔

6 points


What does the case highlight?


• Age as a classifier – Prior criteria did not include patients between 40 to 50 years of age

– Benefit of inclusive criteria approach BUT has limitations

• Use of modern imaging – Halo sign on ultrasound

– Patterns of vessel involvement

• Examination or Investigation omission – e.g. pulses not checked or MRA not done may bias reference diagnosis and

weighting of items in new criteria

• Gold standard (there is none)– Used an expert panel review process, but imperfect

– Imaging or biopsy alone also imperfect

• Omission of items may artificially skew weighting in criteria– E.g. Halo sign. Is it a near perfect predictor simply because it wasn’t done in TAK

patients?

– E.g. Arterial bruits. Do we listen to all arteries in our GCA patients?

(dataset reflects current practice – so does it matter?)

• 40-60 age group is a difficult area to separate LV GCA from TAK

Limitations of the study

New draft criteria

Modern imaging

ACR 1990 classification criteria

Clinical assessment Biopsy and lab

• Allows more discriminant items to factor more heavily in disease classification

– Biopsy

– Halo on ultrasound

– Pattern of angiography

– Diffuse descending aorta involvement on PET


New criteria are weighted

New draft classification criteria for GCA and TAK

Summary

• Extensive set of data collected through an incredibly large world-

wide effort

• Advanced data-driven and expert consensus methodology

• Reflects current practice by incorporating advanced imaging

• Improved sensitivity by expanding clinical phenotype of disease

• Optimize point based system after comprehensive review ofcases who fail to meet either criteria or who meet bothcriteria for GCA and TAK simultaneously

• Definitions of TAK vs GCA specific imaging territories

• Test in datasets where there is standardized acquisition ofclinical and imaging data amongst patients with GCA and TAK

• Obtain and review feedback from YOU

Next steps

Until final endorsement by the ACR & EULAR, these criteria are not final, may change, and are

not appropriate for use in research or for clinical purposes

Acknowledgements

136 sites in 32 countries

Expert panel reviewers 2018

Alba, Marco Duftner, Christina Jennette, Charles Pagnoux, Christian

Barra, Lillian Emmi, Giamoco Juche, Aaron Salvarani, Carlo

Basu, Neil Faurschou, Mikkel Karadağ, Ömer Schmidt, Wolfgang

Blockmans, Daniel Flores-Suárez, Luis Felipe Kermani, Tanaz Sharma, Aman

Brouwer, Elisabeth Gatenby, Paul Khalidi, Nader Sivakumar, Rajappa

Buttgereit, Frank Geraldes, Ruth Koster, Matthew Springer, Jason

Camellino, Dario Gewurz-Singer, Ora Macieira, Carla Sunderkötter, Cord

Chrysidis, Stavros Guillevin, Loïc Matsui, Kazuo Terslev, Lene

Cid, Maria Hammam, Nevin Milchert, Marcin Tesar, Vladimir

Daikeler, Thomas Hauser, Thomas Molloy, Eamonn Tomasson, Gunnar

de Boysson, Hubert Hellmich, Bernhard Monti, Sara Vaglio, Augusto

de Miguel, Eugenio Henes, Joerg Neumann, Thomas Warrington, Kenneth

Dejaco, Christian Hinojosa, Andrea Nielsen, Berit

Diamantopoulos, Andreas Hocevar, Alojzija Novikov, Pavel

Direskeneli, Haner Holle, Julia

Acknowledgements

Andrew JudgeUniversity of Bristol

Sara KhalidUniversity of Oxford

Andrew HutchingsLondon School of Hygiene & Tropical

Medicine

Katherine (Bates) GribbonsNational Institutes of Health

Statisticians

Acknowledgements

Clinical Research FellowsAlberto FloresAna RodriguesBen Seeliger

Hannah QuerinJan Sznajd

Kasia Wawrzycka-AdamczykSara Monti

Takahiro Nunokawa

Data ManagementDavid Gray

Ann-Marie MorganIssi Platt

Database designJoe Barrett

Joe RosaNathanael Gray

Site communications & paymentsMarian Montgomery

Study ManagementAnthea Craven

Acknowledgements

DCVAS is sponsored by the University of Oxford and supported in the UK by theNIHR Clinical Research Network

DCVAS is funded by the American College of Rheumatology, the European League Against Rheumatism, and

the Vasculitis Foundation

Acknowledgements

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Updated Classification Criteria for the Large Vessel Vasculitis...4. SBP difference >10mmHg in arms...

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