American College of Rheumatology/European League Against Rheumatism (ACR/EULAR) Diagnostic and Classification Criteria for Primary Systemic Vasculitis
DCVAS
ACR/EULAR Endorsed Study to Develop New Diagnostic and Classification Criteria for Primary Systemic Vasculitis
1 other identifier
observational
3,588
32 countries
119
Brief Summary
Vasculitis is group of diseases where inflammation of blood vessels is the common feature. Patients typically present with fever, fatigue, weakness and muscle and joint aches. These symptoms are very common among many different diseases, not just vasculitis. A clustering of other symptoms, physical examination findings, blood tests, radiology and biopsy help make the diagnosis. There are currently no criteria to help doctors make a diagnosis of vasculitis when a patient presents with these non specific symptoms and they are reliant on previous experience and disease definitions. One of the aims of this project is to develop diagnostic criteria for the primary systemic vasculitides (granulomatosis with polyangiitis (Wegener's), microscopic polyangiitis, Churg Strauss syndrome, polyarteritis nodosa, giant cell arteritis, Takayasu arteritis). We, the investigators, will do this by studying a large group of patients with vasculitis and comparing them to a large group of patients that present in a similar way, but do not have vasculitis. By comparing the 2 groups we will create a list of items to differentiate between vasculitis and 'vasculitis mimics'. We also aim to update the current classification criteria. Classification criteria are used to group patients into different types of vasculitis, once a diagnosis of vasculitis has been made, and are useful for studying patients in clinical trials with similar or identical diseases. The current classification criteria (American college of Rheumatology 1990 criteria) were developed 20 years ago, before the availability of some important diagnostic tests (e.g. antineutrophil cytoplasmic antibodies \[ANCA\]), and are now not consistent with some of the current disease definitions. Therefore to progress future research in vasculitis, it is important that the classification criteria are updated. We will recruit 260 patients with each of the 6 types of vasculitis and compare them with 1300 controls (patients with the 5 other types of vasculitis), in order to determine the optimal combination of symptoms, signs and investigations that classify each person into the appropriate group.
Trial Health
Trial Health Score
Automated assessment based on enrollment pace, timeline, and geographic reach
participants targeted
Target at P75+ for all trials
Started Jan 2011
Longer than P75 for all trials
119 active sites
Health score is calculated from publicly available data and should be used for screening purposes only.
Trial Relationships
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Study Timeline
Key milestones and dates
First Submitted
Initial submission to the registry
February 9, 2010
CompletedFirst Posted
Study publicly available on registry
February 10, 2010
CompletedStudy Start
First participant enrolled
January 1, 2011
CompletedPrimary Completion
Last participant's last visit for primary outcome
December 1, 2017
CompletedStudy Completion
Last participant's last visit for all outcomes
December 1, 2018
CompletedAugust 19, 2016
August 1, 2016
6.9 years
February 9, 2010
August 18, 2016
Conditions
Keywords
Outcome Measures
Primary Outcomes (1)
Develop new diagnostic and classification criteria for ANCA associated vasculitis and polyarteritis nodosa
3 years
Study Arms (14)
WG classification
Patients with Wegener's granulomatosis. 1st half of these patients will be assigned to the development cohort and the second half to the validation cohort. Classification criteria.
MPA classification
Patients with microscopic polyangiitis. 1st half of these patients will be assigned to the development cohort and the second half to the validation cohort. Classification criteria.
CSS classification
Patients with Churg Strauss syndrome. 1st half of these patients will be assigned to the development cohort and the second half to the validation cohort. Classification criteria.
PAN classification
Patients with polyarteritis nodosa. 1st half of these patients will be assigned to the development cohort and the second half to the validation cohort. Classification criteria.
Control Classification
For each of the diseases being evaluated (WG, MPA, CSS, PAN, GCA, TAK), patients with the other 5 diseases will be the control group. Within these groups, 1st half of these patients will be assigned to the development cohort and the second half to the validation cohort. Classification criteria.
WG diagnostic
Patients with a new presentation of Wegener's granulomatosis. 1st half of these patients will be assigned to the development cohort and the second half to the validation cohort. Diagnostic criteria.
MPA diagnostic
Patients with a new presentation of microscopic polyangiitis. 1st half of these patients will be assigned to the development cohort and the second half to the validation cohort. Diagnostic criteria.
CSS diagnostic
Patients with a new presentation of Churg Strauss syndrome. 1st half of these patients will be assigned to the development cohort and the second half to the validation cohort. Diagnostic criteria.
PAN diagnostic
Patients with a new presentation of polyarteritis nodosa. 1st half of these patients will be assigned to the development cohort and the second half to the validation cohort. Diagnostic criteria.
Control diagnostic
Patients without vasculitis, but presenting with similar features to the 6 different types of vasculitis being studied. 1st half of these patients will be assigned to the development cohort and the second half to the validation cohort. Diagnostic criteria.
GCA classification
Patients with giant cell arteritis. 1st half of these patients will be assigned to the development cohort and the second half to the validation cohort. Classification criteria.
TAK classification
Patients with Takayasu arteritis. 1st half of these patients will be assigned to the development cohort and the second half to the validation cohort. Classification criteria.
GCA diagnostic
Patients with a new diagnosis of giant cell arteritis. 1st half of these patients will be assigned to the development cohort and the second half to the validation cohort. Diagnostic criteria.
TAK diagnostic
Patients with a new diagnosis of Takayasu arteritis. 1st half of these patients will be assigned to the development cohort and the second half to the validation cohort. Diagnostic criteria.
Eligibility Criteria
Patients with primary systemic vasculitis or a mimic of these diseases seen in participating secondary or tertiary care centres in Europe, USA, Mexico, Japan, Asia, Australasia.
You may qualify if:
- Adult patients aged \>18 years. There is no upper age limit.
- Ability to give informed consent. If the patient is unable to give informed consent as a result of death or physical incapacity, then informed assent from next of kin.
- Presumed diagnosis of a primary systemic vasculitis.
You may not qualify if:
- Patients \< 18 years of age.
- Inability to provide informed consent.
- Hepatitis B or C
- Co-morbidities that explain the clinical symptoms and signs on which the diagnosis of vasculitis is made. E.g. infection, tumour, other inflammatory condition, etc.
- Adult patients aged \>18 years. There is no upper age limit.
- Ability to give informed consent. If the patient is unable to give informed consent as a result of death or physical incapacity, then informed assent from next of kin.
- Suspected diagnosis of a primary systemic vasculitis
- Adult patients aged \>18 years. There is no upper age limit.
- Ability to give informed consent. If the patient is unable to give informed consent as a result of death or physical incapacity, then informed assent from next of kin.
- Patients presenting to secondary care with one of the following clinical presentations: I.Multi-system disease. Presentation of disease with at least 2 organs involved. II.Pulmonary-renal syndrome. Defined as haemoptysis / pulmonary haemorrhage with acute renal impairment. III.Acute renal failure IV.Acute respiratory distress. V.Chronic upper airways symptoms and signs. VI.Inflammatory polyarthritis. VII.Fever of unknown origin. VIII.Acute or chronic abdominal pain IX.Hypertension. X.Referred to secondary care with suspicion of vasculitis but confirmed not to have vasculitis. XII.New onset headache. XIII.Jaw or tongue pain. XIV.Sudden visual loss. XV.Limb claudication. XVI.Aortic aneurysm \>5cm.
- Patients under the age of 18
- Patient or next of kin unable or unwilling to provide informed consent or assent.
Contact the study team to confirm eligibility.
Sponsors & Collaborators
- University of Oxfordlead
- American College of Rheumatologycollaborator
- The European League Against Rheumatism (EULAR)collaborator
- The Vasculitis foundationcollaborator
Study Sites (120)
University of Alabama at Birmingham
Birmingham, Alabama, 35233, United States
Cedars-Sinai Medical Center, LA
Los Angeles, California, 90048, United States
University of California, San Francisco
San Francisco, California, 94143-0500, United States
University of Maryland
Baltimore, Maryland, 21201, United States
Vasculitis Center, Boston University School of Medicine
Boston, Massachusetts, 02118, United States
University of Michigan, Internal Medicine
Ann Arbor, Michigan, 48109, United States
Mayo Clinic
Rochester, Minnesota, 55905, United States
Dartmouth-Hitchcock Medical Centre, Lebanon, NH
Lebanon, New Hampshire, 03756, United States
New York University Langone Medical Centre
New York, New York, 10016, United States
University of North Carolina
Chapel Hill, North Carolina, 27599-7525, United States
Cleveland Clinic
Cleveland, Ohio, 44195, United States
University of Pennsylvania
Philadelphia, Pennsylvania, 19104, United States
University of Pittsburgh
Pittsburgh, Pennsylvania, 15261, United States
University Medical Center
Salt Lake City, Utah, 84132-0002, United States
Hospital Interzonal San Juan Bautista
Catamarca, Catamarca Province, Argentina
Ramos Mejia Hospital, University of Buenos Aires
Buenos Aires, C1221ADC, Argentina
ANU Medical Centre
Canberra, Australian Capital Territory, Australia
Royal Brisbane and Women's Hospital
Herston, Queensland, 4029, Australia
Medical University Innsbruck
Innsbruck, Austria
University Hospitals Leuven
Leuven, Belgium
University of Manitoba
Winnipeg, Manitoba, R3A 1M4, Canada
St Joseph's Healthcare
Hamilton, Ontario, Canada
University of Ottawa
Ottawa, Ontario, K1N 6N5, Canada
Mount Sinai Hospital, Toronto
Toronto, Ontario, ON M5T 2S8, Canada
McGill University
Montreal, Quebec, H3A 0G4, Canada
Sherbrooke University Hospital Centre
Sherbrooke, Quebec, J1H 5N4, Canada
University of Calgary
Calgary, Canada
St Joseph's Healthcare London, Ontario
Ontario, Canada
Peking Union Medical College Hospital, Beijing
Beijing, 100032, China
General University Hospital, Prague
Prague, 128 08, Czechia
General University Hospital
Prague, Czechia
Rigshospitalet
Copenhagen, Denmark
Assiut University, Assiut University Hospitals
Asyut, 71516, Egypt
Cairo University, Kasr El Ainy Hospital
Cairo, Egypt
Helsinki University Central Hospital
Helsinki, Finland
Cochin Hospital, Université Paris-descartes
Paris, 75679, France
Universitätsklinikum Münster
Münster, Münster, 48149, Germany
Universitätsklinikum Jena
Jena, 07743 Jena, Germany
University of Schleswig-Holstein
Lübeck, Germany
Kreiskliniken Esslingen
Plochingen, 73207, Germany
University Hospital Tübingen
Tübingen, D-72076, Germany
University of Debrecen Medical and Health Science Center
Debrecen, 4032, Hungary
Chatrapathi Shahuji Maharaj Medical Center, Lucknow (IProcess)
Lucknow, Uttar Pradesh, 226003, India
Postgraduate Institute of Medical Education and Research, Chandigarh
Chandigarh, Pin- 160 012, India
Nizam's Institute of Medical Sciences, Hyderabad
Hyderabad, 500082, India
Medanta, Delhi
New Delhi, 110024, India
Christian Medical College & Hospital, Vellore
Vellore, 632 004, India
Cork University Hospital
Cork, Ireland
St. Vincent's University Hospital, Dublin
Dublin, Ireland
University of Parma
Parma, Italy
Santa Maria Nuova Hospital, Reggio Emilia
Reggio Emilia, 42123, Italy
Arcispedale Santa Maria Nuova
Reggio Emilia, Italy
Kameda Medical Centre, Kamogawa
Kamogawa, Chiba, 296-8602, Japan
Tsukuba University Hospital
Tsukuba, Ibaraki, 305-8576, Japan
Miyazaki University Hospital
Miyazaki, Miyazaki, 889-1692, Japan
Saitama Medical University
Kawagoe, Saitama, 350-8550, Japan
Kyorin University Hospital
Mitaka, Tokyo, 181-8611, Japan
Chiba University
Chiba, 260-8670, Japan
Kagawa University Hospital
Kagawa, 761-0793, Japan
St. Marianna University Hospital
Kanagawa, 216-8511, Japan
Kanazawa University Hospital
Kanazawa, 920-8641, Japan
Okayama University Hospital
Okayama, 700-8558, Japan
Kitano Hospital
Osaka, Japan
Juntendo University Koshigaya Hospital
Saitama, 343-0032, Japan
Jichi Medical University Hospital
Tochigi-ken, 3311-1 Yakushiji, Japan
University Tokyo Hospital
Tokyo, 113-8655, Japan
Instituto Nacional de Enfermedades Respiratorias
Mexico City, 14000, Mexico
Instituto Nacional de Ciencias Medicas y Nutricion Salvador Zubiran
Mexico City, Mexico
VU University Medical Center
Amsterdam, 6Z 165, Netherlands
University Medical Center Groningen
Groningen, 30.001, Netherlands
University of Otago, Christchurch
Christchurch, Canterbury, 8011, New Zealand
Auckland District Health Board
Auckland, New Zealand
Waitemata District Health Board, North Shore Hospital
Auckland, New Zealand
Waikato District Health Board
Hamilton, 3240, New Zealand
Hospital of Southern Norway
Kristiansand, Post box 416, 4605, Norway
The University Hospital of Northern Norway, Tromsø
Tromsø, 9038, Norway
University of Jagiellonian
Krakow, 31-007, Poland
Hospital Garcia de Orta, Almada
Almada, Portugal
Santa Maria Hospital, Lisbon
Lisbon, 1649-035, Portugal
Hospital Santo Antonio, Porto
Porto, 4099 - 001, Portugal
First Moscow State Medical University
Moscow, 119991, Russia
University Medical Centre Ljubljana
Ljubljana, 1000, Slovenia
Seoul National University Hospital
Seoul, 110-744, South Korea
Clinic Barcelona Hospital Universitari
Barcelona, Catalonia, Spain
University of Colombo
Colombo, Sri Lanka
Lund University
Lund, Lund, SE-221 85, Sweden
Karolinska Institute, Stockholm
Stockholm, 141 86, Sweden
Linköping University
Stockholm, 581 83, Sweden
Umeå University
Umeå, 901 85, Sweden
Uppsala University Hospital
Uppsala, 751 85, Sweden
University Hospital Basel
Basel, 4031, Switzerland
Immunologie-Zentrum Zurich
Zurich, Switzerland
Hacettepe University
Ankara, Turkey (Türkiye)
Istanbul University, Cerrahpasa Medical School
Istanbul, 34098, Turkey (Türkiye)
Marmara University Medical School
Istanbul, 34668, Turkey (Türkiye)
Fatih University Medical Faculty
Istanbul, 34844, Turkey (Türkiye)
Haydarpasa Education and Research Hospital
Istanbul, Turkey (Türkiye)
Istanbul University, Istanbul Medical School
Istanbul, Turkey (Türkiye)
North Cumbria University Hospitals, The Cumberland Infirmary
Carlisle, Cumbria, CA2 7HY, United Kingdom
Basildon and Thurrock University Hospitals NHS Foundation Trust
Basildon, Essex, SS16 5NL, United Kingdom
Queen's Hospital
Romford, Essex, RM7 0AG, United Kingdom
Southend University Hospital NHS Trust
Westcliff-on-Sea, Essex, SS0 0RY, United Kingdom
NHS Fife, Whyteman's Brae Hospital, Windygates
Kirkcaldy, Fife, KY8 5PR, United Kingdom
Norfolk and Norwich University Hospital
Norwich, Norwich, NR4 7UY, United Kingdom
Nuffield Orthopaedic Centre
Oxford, Oxford, OX3 7LD, United Kingdom
Aberdeen Royal Infirmary
Aberdeen, Scotland, AB25 2ZN, United Kingdom
NHS Greater Glasgow & Clyde, Gartnavel Hospital
Glasgow, Scotland, G12 8TA, United Kingdom
Ipswich Hospital NHS Trust
Ipswich, Suffolk, United Kingdom
Epsom and St Helier University Hospitals NHS Trust
Carshalton, Surrey, SM5 1AA, United Kingdom
University of Birmingham
Birmingham, United Kingdom
Addenbrooke's Hospital
Cambridge, United Kingdom
Dudley Group of Hospitals, NHS FT
Dudley, DY1 2HQ, United Kingdom
Imperial College Healthcare NHS Trust, Hammersmith Hospital
London, W12 0HS, United Kingdom
University of Manchester, Manchester Royal Infirmary
Manchester, M13 9WL, United Kingdom
Nottingham University Hospitals NHS Trust (QMC)
Nottingham, NG7 2UH, United Kingdom
Oxford University Hospitals NHS Trust (The Churchill Hospital)
Oxford, OX3 7LE, United Kingdom
Royal Berkshire NHS Trust
Reading, RG1 5AN, United Kingdom
Heatherwood & Wexham Park Hospitals NHS Foundation Trust
Slough, SL2 4HL, United Kingdom
Southampton University Hospitals NHS Trust
Southampton, SO16 6YD, United Kingdom
York Hospital NHS Foundation Trust
York, YO31 8HE, United Kingdom
Related Publications (8)
Singh JA, Solomon DH, Dougados M, Felson D, Hawker G, Katz P, Paulus H, Wallace C; Classification and Response Criteria Subcommittee of the Committee on Quality Measures, American College of Rheumatology. Development of classification and response criteria for rheumatic diseases. Arthritis Rheum. 2006 Jun 15;55(3):348-52. doi: 10.1002/art.22003.
PMID: 16739201BACKGROUNDRao JK, Allen NB, Pincus T. Limitations of the 1990 American College of Rheumatology classification criteria in the diagnosis of vasculitis. Ann Intern Med. 1998 Sep 1;129(5):345-52. doi: 10.7326/0003-4819-129-5-199809010-00001.
PMID: 9735061BACKGROUNDHunder GG, Arend WP, Bloch DA, Calabrese LH, Fauci AS, Fries JF, Leavitt RY, Lie JT, Lightfoot RW Jr, Masi AT, et al. The American College of Rheumatology 1990 criteria for the classification of vasculitis. Introduction. Arthritis Rheum. 1990 Aug;33(8):1065-7. doi: 10.1002/art.1780330802. No abstract available.
PMID: 2390119BACKGROUNDFries JF, Hunder GG, Bloch DA, Michel BA, Arend WP, Calabrese LH, Fauci AS, Leavitt RY, Lie JT, Lightfoot RW Jr, et al. The American College of Rheumatology 1990 criteria for the classification of vasculitis. Summary. Arthritis Rheum. 1990 Aug;33(8):1135-6. doi: 10.1002/art.1780330812. No abstract available.
PMID: 2202312BACKGROUNDJennette JC, Falk RJ, Andrassy K, Bacon PA, Churg J, Gross WL, Hagen EC, Hoffman GS, Hunder GG, Kallenberg CG, et al. Nomenclature of systemic vasculitides. Proposal of an international consensus conference. Arthritis Rheum. 1994 Feb;37(2):187-92. doi: 10.1002/art.1780370206.
PMID: 8129773BACKGROUNDSorensen SF, Slot O, Tvede N, Petersen J. A prospective study of vasculitis patients collected in a five year period: evaluation of the Chapel Hill nomenclature. Ann Rheum Dis. 2000 Jun;59(6):478-82. doi: 10.1136/ard.59.6.478.
PMID: 10834866BACKGROUNDFelson DT, Anderson JJ. Methodological and statistical approaches to criteria development in rheumatic diseases. Baillieres Clin Rheumatol. 1995 May;9(2):253-66. doi: 10.1016/s0950-3579(05)80189-x.
PMID: 7656339BACKGROUNDYates M, MacGregor AJ, Robson J, Craven A, Merkel PA, Luqmani RA, Watts RA. The association of vascular risk factors with visual loss in giant cell arteritis. Rheumatology (Oxford). 2017 Apr 1;56(4):524-528. doi: 10.1093/rheumatology/kew397.
PMID: 27940595DERIVED
Related Links
Biospecimen
Patients may be given the option of having blood, urine or other tissue obtained as part of their routine care retained for use in future ethically approved studies. They may also be asked to provide additional blood to be stored for this purpose. This component is an optional extra, and does not form part of the main study.
MeSH Terms
Conditions
Condition Hierarchy (Ancestors)
Study Officials
- PRINCIPAL INVESTIGATOR
Raashid A Luqmani, DM, FRCP(E)
University of Oxford, United Kingdom
- PRINCIPAL INVESTIGATOR
Peter Merkel, MD, MPH
University of Pennsylvania
- PRINCIPAL INVESTIGATOR
Richard Watts, DM, FRCP
University of East Anglia
Central Study Contacts
Study Design
- Study Type
- observational
- Observational Model
- CASE CONTROL
- Time Perspective
- PROSPECTIVE
- Sponsor Type
- OTHER
- Responsible Party
- SPONSOR
Study Record Dates
First Submitted
February 9, 2010
First Posted
February 10, 2010
Study Start
January 1, 2011
Primary Completion
December 1, 2017
Study Completion
December 1, 2018
Last Updated
August 19, 2016
Record last verified: 2016-08