American College of Rheumatology/European League Against Rheumatism (ACR/EULAR) Diagnostic and Classification Criteria for Primary Systemic Vasculitis (DCVAS)

August 18, 2016 updated by: University of Oxford

ACR/EULAR Endorsed Study to Develop New Diagnostic and Classification Criteria for Primary Systemic Vasculitis

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.

Study Overview

Status

Unknown

Conditions

Detailed Description

The systemic vasculitides are a group of uncommon but important diseases whose prognosis has improved dramatically with the use of immunosuppressive therapy. However, long-term morbidity from recurrent disease flares, low-grade grumbling disease and/or accumulating damage from previous disease activity or drug therapy now characterise the long-term outlook for patients with vasculitis. There remains major controversy, and incompatibility between the ANCA-associated vasculitides: granulomatosis with polyangiitis (Wegener's), microscopic polyangiitis, and Churg Strauss Syndrome, as well as polyarteritis nodosa in the current classification criteria and disease definitions. Importantly, there are no diagnostic criteria for any of the primary systemic vasculitides.

We propose to improve existing classification criteria for the primary systemic vasculitides. As a starting point will include the following diseases: granulomatosis with polyangiitis (Wegener's) (GPA), microscopic polyangiitis (MPA), Churg Strauss syndrome (CSS), polyarteritis nodosa (PAN), giant cell arteritis (GCA) and Takayasu arteritis (TAK).

We propose to develop and validate classification and diagnostic criteria for primary systemic vasculitis using the guidelines suggested by the Classification and Response Criteria Subcommittee of the American College of Rheumatology Committee on Quality Measures. For all patients, a detailed medical history, physical examination, laboratory tests (including ANCA), radiology (including angiography), biopsy results, treatment, Birmingham Vasculitis Activity Score (BVAS)version 3, Vasculitis Damage Index (VDI), will be collected. The exact list of items to be recorded will be determined by the expert panel at the start of the study.

Classification criteria

We will study a minimum of 100 patients (new and existing patients) prospectively within each currently defined disease category (GPA, CSS, MPA, PAN, GCA, TAK) for the development of the classification criteria. We anticipate the need to recruit 130 patients to account for misdiagnosis and dropout to achieve the target of 100 with the confirmed reference diagnosis. This will include patients that have vasculitis which are assumed to be related to ANCA but do not fulfil the current definitions of any of the diseases, and patients with large vessel vasculitis which do not fulfil current definition for GCA or TAK. Therefore new categories of disease may be created as part of this process and some of the current disease categories may be changed to include or exclude certain patients.

The other diseases will be the controls. The same minimum number of patients will be used to validate the criteria. The 1st 100 patients with a formal reference diagnosis that are recruited for each disease will be used for development of the classification criteria; the next 100 consecutive patients recruited with a confirmed reference diagnosis for each disease will be used to validate the criteria. Again we anticipate the need to recruit 130 patients to account for misdiagnosis and dropout to achieve the 100 target. The majority of cases included will be the same as that used for the development of the diagnostic criteria.

In the absence of an established gold standard, we propose to develop a reference standard. Clinical vignettes using clustering of clinical features and investigations will be constructed from actual cases by the steering group. An expert panel will then be asked to classify each vignette. Hypothetical changes will then be made to components of each clinical vignette and the expert panel will be asked to re classify the case. This process will be repeated multiple times in an attempt to determine what key clinical feature influence the expert panel to change the diagnosis. Using this data driven process, a construct of important clinical features for each disease will be determined by the expert panel. Using this new construct, patients will be classified by the expert panel. This will form the reference standard against which the new criteria will be tested.

Diagnostic Criteria

We propose to develop and validate diagnostic criteria for primary systemic vasculitis. Based on current disease categories we will include GPA, MPA, CSS, PAN, GCA and TAK (but this may change depending on whether new categories are created or existing categories merged as part of the classification criteria component). For the development of diagnostic criteria, we will study a minimum of 100 patients (will require approx 130 patients to allow for dropout and misdiagnosis) for each disease category. Assuming 6 disease categories, the majority of these 780 patients will have already been identified from the classification criteria component of the study and will be re used for the development and validation of diagnostic criteria. However, for the diagnostic criteria to be clinically relevant we will only include patients that are seen at the time of 1st presentation, therefore not all the 780 patients recruited for the classification criteria section of the study will be suitable, and we will need to recruit additional new patients for each of the types of vasculitis being studied.

We will use a minimum of 400 context specific controls (patients that don't have vasculitis) for AAV and PAN that will cover the spectrum of different disease presentations and severity. In addition, we will recruit a minimum of 100 context specific controls for GCA and a similar number for TAK. Different control populations are needed for AAV, GCA and TAK as they have significantly different clinical presentations. In a similar manner to cases, we will recruit 30% more patients than the minimum required to account for misdiagnosis and drop out. The same minimum number of cases and controls will be needed to validate the criteria. The first half of the patients recruited would be used to develop the criteria, and the 2nd half to validate the criteria. We will allow inclusion of patients from previously studied prospective cohorts that meet all the appropriate inclusion / exclusion criteria and have had all the appropriate clinical information and mandatory investigation (to be defined later) recorded at time of their first presentation. This is to facilitate the recruitment of sufficient patients with PAN, CSS and TAK which are rare conditions.

Statistical analysis

We will follow the ACR recommended statistical methods for creating the classification criteria. Patients will have been classified into the different types of vasculitis according to the proposed EULAR/ACR schema by the expert panel or as a vasculitis mimic. The outcomes of interest are binary variables indicating whether or not a patient has been classified as having a particular type of vasculitis, such as GPA, MPA, etc. For each outcome, multivariable logistic regression modeling will be used to identify predictors of outcome based on the list of potential predictor variables described earlier. We will also explore the use of Classification And Regression Tree (CART) analysis. This is a tree-building technique ideally suited to the generation of clinical decision rules. Unlike conventional regression methods, patients are partitioned ("split") into different groups based on an exhaustive search of all possible predictor variables. The advantage of CART analysis over conventional methods is that it is non-parametric, so no assumptions are made about the underlying distribution of predictor variables. CART can handle many hundreds of possible predictor variables and can uncover complex interactions between predictors which may be difficult or impossible to uncover using traditional multivariate techniques that can suffer from model over fitting. In addition, clinicians generally do not think in terms of probability but rather in terms of categories, such as low versus high risk. Clinical decision rules generated using CART analysis are more likely to make clinical sense, and hence more likely to be followed in clinical practice.

Once the best items are identified, the expert panel will decide on the best short list of items to be included in each criteria and also choose the most appropriate decision tree. This will provide the best content validity.

The statistical methods to be used for diagnostic criteria will be very similar to that used for the classification criteria. The binary outcome for analysis is whether the person is a case or control (without vasculitis). We repeat the analyses for each of each type of vasculitis e.g. WG versus controls, then CSS versus controls etc

Study Type

Observational

Enrollment (Anticipated)

3588

Contacts and Locations

This section provides the contact details for those conducting the study, and information on where this study is being conducted.

Study Contact

Study Contact Backup

Study Locations

  • Argentina
      • Buenos Aires, Argentina, C1221ADC
        • Recruiting
        • Ramos Mejia Hospital, University of Buenos Aires
        • Contact:
        • Principal Investigator:
          • Eduardo Kerzberg
    • Catamarca
      • San Fernando del Valle de Catamarca, Catamarca, Argentina
        • Recruiting
        • Hospital Interzonal San Juan Bautista
        • Contact:
        • Principal Investigator:
          • Sergio Toloza
  • Australia
    • Australian Capital Territory
      • Canberra, Australian Capital Territory, Australia
        • Recruiting
        • ANU Medical Centre
        • Contact:
        • Principal Investigator:
          • Paul Gatenby
    • Queensland
      • Herston, Queensland, Australia, 4029
  • Austria
      • Innsbruck, Austria
  • Belgium
  • Canada
      • Calgary, Canada
        • Not yet recruiting
        • University of Calgary
        • Contact:
        • Principal Investigator:
          • Michael Walsh
      • Ontario, Canada
        • Recruiting
        • St Joseph's Healthcare London, Ontario
        • Contact:
          • Lillian Barra
        • Principal Investigator:
          • Lillian Barra
    • Manitoba
      • Winnipeg, Manitoba, Canada, R3A 1M4
        • Recruiting
        • University of Manitoba
        • Contact:
        • Principal Investigator:
          • Navjot Dhindsa
    • Ontario
      • Hamilton, Ontario, Canada
        • Recruiting
        • St Joseph's Healthcare
        • Contact:
        • Principal Investigator:
          • Nader Khalidi
      • Ottawa, Ontario, Canada, K1N 6N5
      • Toronto, Ontario, Canada, ON M5T 2S8
        • Recruiting
        • Mount Sinai Hospital, Toronto
        • Contact:
        • Principal Investigator:
          • Simon Carette
    • Quebec
      • Montreal, Quebec, Canada, H3A 0G4
        • Recruiting
        • McGill University
        • Contact:
        • Principal Investigator:
          • Christian Pineau
      • Sherbrooke, Quebec, Canada, J1H 5N4
        • Recruiting
        • Sherbrooke University Hospital Centre
        • Contact:
        • Principal Investigator:
          • Patrick Liang
  • China
      • Beijing, China, 100032
        • Recruiting
        • Peking Union Medical College Hospital, Beijing
        • Contact:
        • Principal Investigator:
          • Xinping Tian
  • Czech Republic
      • Prague, Czech Republic, 128 08
      • Prague, Czech Republic
        • Recruiting
        • General University Hospital
        • Contact:
          • Vladamir Tesar
        • Principal Investigator:
          • Vladamir Tesar
  • Denmark
      • Copenhagen, Denmark
        • Recruiting
        • Rigshospitalet
        • Contact:
        • Principal Investigator:
          • Niels Rasmussen
        • Principal Investigator:
          • Bo Baslund
  • Egypt
      • Assiut, Egypt, 71516
        • Recruiting
        • Assiut University, Assiut University Hospitals
        • Contact:
        • Principal Investigator:
          • Nevin Hammam
      • Cairo, Egypt
        • Recruiting
        • Cairo University, Kasr El Ainy Hospital
        • Contact:
        • Principal Investigator:
          • Amira Shahin
  • Finland
      • Helsinki, Finland
        • Recruiting
        • Helsinki University Central Hospital
        • Contact:
        • Principal Investigator:
          • Jukka Putaala
  • France
      • Paris, France, 75679
        • Not yet recruiting
        • Cochin Hospital, Université Paris-descartes
        • Contact:
        • Principal Investigator:
          • Loic Guillevin
  • Germany
      • Jena, Germany, 07743 Jena
      • Luebeck, Germany
        • Recruiting
        • University of Schleswig-Holstein
        • Contact:
        • Principal Investigator:
          • Julia Holle
      • Münster, Germany, 48149
      • Plochingen, Germany, 73207
        • Recruiting
        • Kreiskliniken Esslingen
        • Contact:
        • Principal Investigator:
          • Bernhard Hellmich
      • Tübingen, Germany, D-72076
  • Hungary
      • Debrecen, Hungary, 4032
        • Recruiting
        • University of Debrecen Medical and Health Science Center
        • Contact:
        • Principal Investigator:
          • Zoltan Szekanecz
  • India
      • Chandigarh, India, Pin- 160 012
        • Recruiting
        • Postgraduate Institute of Medical Education and Research, Chandigarh
        • Contact:
        • Principal Investigator:
          • Aman Sharma
      • Hyderabad, India, 500082
        • Recruiting
        • Nizam's Institute of Medical Sciences, Hyderabad
        • Contact:
        • Principal Investigator:
          • Liza Rajasekhar
      • New Delhi, India, 110024
        • Recruiting
        • Medanta, Delhi
        • Contact:
        • Principal Investigator:
          • Rajiva Gupta
      • Vellore, India, 632 004
        • Recruiting
        • Christian Medical College & Hospital, Vellore
        • Contact:
        • Principal Investigator:
          • Debashish Danda
    • Uttar Pradesh
      • Lucknow, Uttar Pradesh, India, 226003
        • Recruiting
        • Chatrapathi Shahuji Maharaj Medical Center, Lucknow (IProcess)
        • Contact:
          • Siddhart Das
        • Principal Investigator:
          • Siddhart Das
  • Ireland
      • Cork, Ireland
        • Recruiting
        • Cork University Hospital
        • Contact:
        • Principal Investigator:
          • Michael Clarkson
      • Dublin 4, Ireland
        • Recruiting
        • St. Vincent's University Hospital, Dublin
        • Contact:
        • Principal Investigator:
          • Eamonn Molloy
  • Italy
      • Parma, Italy
      • Reggio Emilia, Italy, 42123
        • Recruiting
        • Santa Maria Nuova Hospital, Reggio Emilia
        • Contact:
        • Principal Investigator:
          • Carlo Salvarani
      • Reggio Emilia, Italy
        • Recruiting
        • Arcispedale Santa Maria Nuova
        • Contact:
        • Principal Investigator:
          • Carlo Salvarani
  • Japan
      • Chiba, Japan, 260-8670
      • Kagawa, Japan, 761-0793
        • Recruiting
        • Kagawa University Hospital
        • Contact:
          • Hiroaki Dobashi
        • Principal Investigator:
          • Hiroaki Dobashi
      • Kanagawa, Japan, 216-8511
        • Recruiting
        • St. Marianna University Hospital
        • Contact:
        • Principal Investigator:
          • Hidehiro Yamada
      • Kanazawa, Japan, 920-8641
      • Okayama, Japan, 700-8558
        • Recruiting
        • Okayama University Hospital
        • Contact:
          • Hirofumi Makino
        • Principal Investigator:
          • Hirofumi Makino
      • Osaka, Japan
        • Recruiting
        • Kitano Hospital
        • Contact:
          • Eri Muso
        • Principal Investigator:
          • Eri Muso
      • Saitama, Japan, 343-0032
        • Recruiting
        • Juntendo University Koshigaya Hospital
        • Contact:
          • Shigeto Kobayashi
        • Principal Investigator:
          • Shigeto Kobayashi
      • Tochigi-ken, Japan, 3311-1 Yakushiji
        • Recruiting
        • Jichi Medical University Hospital
        • Contact:
          • Wako Yumura
        • Principal Investigator:
          • Wako Yumura
      • Tokyo, Japan, 113-8655
        • Recruiting
        • University Tokyo Hospital
        • Contact:
        • Principal Investigator:
          • Junichi Hirahashi
    • Chiba prefecture
      • Kamogawa City, Chiba prefecture, Japan, 296-8602
        • Recruiting
        • Kameda Medical Centre, Kamogawa
        • Contact:
        • Principal Investigator:
          • Kazuo Matsui
    • Ibaraki Prefecture
      • Tsukuba, Ibaraki Prefecture, Japan, 305-8576
        • Recruiting
        • Tsukuba University Hospital
        • Contact:
          • Kunihiro Yamagata
        • Principal Investigator:
          • Kunihiro Yamagata
    • Miyazaki Prefecture
      • Miyazaki City, Miyazaki Prefecture, Japan, 889-1692
        • Recruiting
        • Miyazaki University Hospital
        • Contact:
          • Shouichi Fujimoto
        • Principal Investigator:
          • Shouichi Fujimoto
    • Saitama Prefecture
      • Kawagoe, Saitama Prefecture, Japan, 350-8550
        • Recruiting
        • Saitama Medical University
        • Contact:
        • Principal Investigator:
          • Koichi Amano
    • Tokyo Prefecture
      • Mitaka, Tokyo Prefecture, Japan, 181-8611
        • Recruiting
        • Kyorin University Hospital
        • Contact:
          • Yoshihiro Arimura
        • Principal Investigator:
          • Yoshihiro Arimura
  • Korea, Republic of
      • Seoul, Korea, Republic of, 110-744
        • Recruiting
        • Seoul National University Hospital
        • Contact:
        • Principal Investigator:
          • Yeong-Wook Song
  • Mexico
      • Mexico City, Mexico, 14000
        • Recruiting
        • Instituto Nacional de Enfermedades Respiratorias
        • Contact:
        • Principal Investigator:
          • Luis Felipe Flores-Suárez
      • Mexico City, Mexico
        • Recruiting
        • Instituto Nacional de Ciencias Medicas y Nutricion Salvador Zubiran
        • Contact:
          • Andrea Hinojosa
        • Principal Investigator:
          • Andrea Hinojosa
  • Netherlands
      • Amsterdam, Netherlands, 6Z 165
        • Not yet recruiting
        • VU University Medical Center
        • Contact:
        • Principal Investigator:
          • Maarten Boers
      • Groningen, Netherlands, 30.001
        • Recruiting
        • University Medical Center Groningen
        • Contact:
        • Principal Investigator:
          • Abraham Rutgers
  • New Zealand
      • Auckland, New Zealand
        • Recruiting
        • Auckland District Health Board
        • Contact:
          • Ravi Suppiah
        • Principal Investigator:
          • Ravi Suppiah
      • Auckland, New Zealand
        • Not yet recruiting
        • Waitemata District Health Board, North Shore Hospital
        • Contact:
        • Principal Investigator:
          • Janak de Zoysa
      • Hamilton, New Zealand, 3240
    • Canterbury
      • Christchurch, Canterbury, New Zealand, 8011
        • Recruiting
        • University of Otago, Christchurch
        • Contact:
        • Principal Investigator:
          • Lisa Stamp
  • Norway
      • Kristiansand, Norway, Post box 416, 4605
        • Recruiting
        • Hospital of southern Norway
        • Contact:
        • Principal Investigator:
          • Andreas Diamantopoulos
      • Tromsø, Norway, 9038
        • Recruiting
        • The University Hospital of Northern Norway, Tromsø
        • Contact:
        • Principal Investigator:
          • Emilio Besada
  • Poland
      • Kraków, Poland, 31-007
        • Recruiting
        • University of Jagiellonian
        • Contact:
        • Principal Investigator:
          • Jan Sznajd
  • Portugal
      • Almada, Portugal
        • Recruiting
        • Hospital Garcia de Orta, Almada
        • Contact:
        • Principal Investigator:
          • Miguel Rodrigues
      • Lisbon, Portugal, 1649-035
        • Recruiting
        • Santa Maria Hospital, Lisbon
        • Contact:
        • Principal Investigator:
          • Ruth Geraldes
      • Porto, Portugal, 4099 - 001
        • Recruiting
        • Hospital Santo Antonio, Porto
        • Contact:
        • Principal Investigator:
          • Ernestina Santos
  • Russian Federation
      • Moscow, Russian Federation, 119991
        • Recruiting
        • First Moscow State Medical University
        • Contact:
        • Principal Investigator:
          • Sergey Moiseev
        • Sub-Investigator:
          • Pavel Novikov
  • Slovenia
      • Ljubljana, Slovenia, 1000
        • Recruiting
        • University Medical Centre Ljubljana
        • Contact:
        • Principal Investigator:
          • Alojzija Hočevar
  • Spain
    • Catalonia
      • Barcelona, Catalonia, Spain
        • Recruiting
        • Clinic Barcelona Hospital Universitari
        • Contact:
        • Principal Investigator:
          • Maria Cid
  • Sri Lanka
      • Columbo 8, Sri Lanka
        • Recruiting
        • University of Colombo
        • Contact:
        • Principal Investigator:
          • Inoshi Atukorala
  • Sweden
      • Lund, Sweden, SE-221 85
      • Stockholm, Sweden, 141 86
        • Not yet recruiting
        • Karolinska Institute, Stockholm
        • Contact:
        • Principal Investigator:
          • Johan Bratt
      • Stockholm, Sweden, 581 83
        • Recruiting
        • Linköping University
        • Principal Investigator:
          • Marten Segelmark
        • Contact:
      • Umeå, Sweden, 901 85
        • Not yet recruiting
        • Umea University
        • Contact:
        • Principal Investigator:
          • Ewa Berglin
      • Uppsala, Sweden, 751 85
  • Switzerland
      • Basel, Switzerland, 4031
        • Recruiting
        • University Hospital Basel
        • Contact:
        • Principal Investigator:
          • Thomas Daikeler
      • Zurich, Switzerland
        • Not yet recruiting
        • Immunologie-Zentrum Zurich
        • Contact:
        • Principal Investigator:
          • Thomas Hauser
  • Turkey
      • Ankara, Turkey
        • Recruiting
        • Hacettepe University
        • Contact:
        • Principal Investigator:
          • Omer Karadag
      • Istanbul, Turkey, 34098
        • Recruiting
        • Istanbul University, Cerrahpasa Medical School
        • Contact:
        • Principal Investigator:
          • Gulen Hatemi
      • Istanbul, Turkey, 34668
        • Recruiting
        • Marmara University Medical School
        • Contact:
        • Principal Investigator:
          • Haner Direskeneli
      • Istanbul, Turkey, 34844
        • Recruiting
        • Fatih University Medical Faculty
        • Contact:
        • Principal Investigator:
          • Seval Pehlivan
      • Istanbul, Turkey
        • Not yet recruiting
        • Haydarpasa Education and Research Hospital
        • Contact:
        • Principal Investigator:
          • Hasan Yazici
      • Istanbul, Turkey
        • Recruiting
        • Istanbul University, Istanbul Medical School
        • Contact:
        • Principal Investigator:
          • Sevil Kamali
  • United Kingdom
      • Birmingham, United Kingdom
        • Recruiting
        • University of Birmingham
        • Contact:
        • Principal Investigator:
          • Caroline Savage
      • Cambridge, United Kingdom
      • Dudley, United Kingdom, DY1 2HQ
        • Recruiting
        • Dudley Group of Hospitals, NHS FT
        • Contact:
          • Rainer Kloche
          • Phone Number: 0044 1384 244807
        • Principal Investigator:
          • Rainer Kloche
      • London, United Kingdom, W12 0HS
        • Recruiting
        • Imperial College Healthcare NHS Trust, Hammersmith Hospital
        • Contact:
        • Principal Investigator:
          • Justin Mason
      • Manchester, United Kingdom, M13 9WL
        • Recruiting
        • University of Manchester, Manchester Royal Infirmary
        • Contact:
        • Principal Investigator:
          • Ian Bruce
      • Norwich, United Kingdom, NR4 7UY
        • Recruiting
        • Norfolk and Norwich University Hospital
        • Contact:
        • Principal Investigator:
          • Chetan Mukhtyar
      • Nottingham, United Kingdom, NG7 2UH
        • Recruiting
        • Nottingham University Hospitals NHS Trust (QMC)
        • Contact:
        • Principal Investigator:
          • Peter Lanyon
      • Oxford, United Kingdom, OX3 7LD
        • Recruiting
        • Nuffield Orthopaedic Centre
        • Contact:
        • Principal Investigator:
          • Raashid Luqmani
        • Sub-Investigator:
          • Joanna Robson
      • Oxford, United Kingdom, OX3 7LE
        • Recruiting
        • Oxford University Hospitals NHS Trust (The Churchill Hospital)
        • Contact:
        • Principal Investigator:
          • Rachel Hoyles
      • Reading, United Kingdom, RG1 5AN
      • Slough, United Kingdom, SL2 4HL
        • Recruiting
        • Heatherwood & Wexham Park Hospitals NHS Foundation Trust
        • Contact:
        • Principal Investigator:
          • Matthew Adler
      • Southampton, United Kingdom, SO16 6YD
        • Withdrawn
        • Southampton University Hospitals NHS Trust
      • York, United Kingdom, YO31 8HE
        • Recruiting
        • York Hospital NHS Foundation Trust
        • Contact:
        • Principal Investigator:
          • Colin Jones
    • Cumbria
      • Carlisle, Cumbria, United Kingdom, CA2 7HY
        • Recruiting
        • North Cumbria University Hospitals, The Cumberland Infirmary
        • Contact:
        • Principal Investigator:
          • Alaa Hassan
    • Essex
      • Basildon, Essex, United Kingdom, SS16 5NL
        • Recruiting
        • Basildon and Thurrock University Hospitals NHS Foundation Trust
        • Contact:
          • Nagui Gendi
        • Principal Investigator:
          • Nagui Gendi
      • Romford, Essex, United Kingdom, RM7 0AG
        • Recruiting
        • Queen's Hospital
        • Contact:
        • Principal Investigator:
          • Kuntal Chakravarty
      • Westcliff-on-Sea, Essex, United Kingdom, SS0 0RY
    • Fife
      • Kirkcaldy, Fife, United Kingdom, KY8 5PR
        • Recruiting
        • NHS Fife, Whyteman's Brae Hospital, Windygates
        • Contact:
        • Principal Investigator:
          • John Mclaren
    • Scotland
      • Aberdeen, Scotland, United Kingdom, AB25 2ZN
        • Recruiting
        • Aberdeen Royal Infirmary
        • Contact:
        • Principal Investigator:
          • Neil Basu
      • Glasgow, Scotland, United Kingdom, G12 8TA
        • Recruiting
        • NHS Greater Glasgow & Clyde, Gartnavel Hospital
        • Contact:
        • Principal Investigator:
          • Iain McInnes
    • Suffolk
      • Ipswich, Suffolk, United Kingdom
        • Recruiting
        • Ipswich Hospital NHS Trust
        • Contact:
        • Principal Investigator:
          • Professor Richard Watts
    • Surrey
      • Carshalton, Surrey, United Kingdom, SM5 1AA
        • Recruiting
        • Epsom and St Helier University Hospitals NHS Trust
        • Contact:
          • Sanjeev Patel
        • Contact:
        • Principal Investigator:
          • Sanjeev Patel
  • United States
    • Alabama
      • Birmingham, Alabama, United States, 35233
        • Recruiting
        • University of Alabama at Birmingham
        • Contact:
        • Principal Investigator:
          • Angelo Gaffo
    • California
      • Los Angeles, California, United States, 90048
        • Recruiting
        • Cedars-Sinai Medical Center, LA
        • Contact:
        • Principal Investigator:
          • Michael Weisman
      • San Francisco, California, United States, 94143-0500
        • Recruiting
        • University of California, San Francisco
        • Contact:
        • Principal Investigator:
          • Sharon Chung
    • Maryland
      • Baltimore, Maryland, United States, 21201
        • Recruiting
        • University of Maryland
        • Contact:
        • Principal Investigator:
          • Jamal Mikdashi
    • Massachusetts
      • Boston, Massachusetts, United States, 02118
        • Recruiting
        • Vasculitis Center, Boston University School of Medicine
        • Contact:
        • Principal Investigator:
          • Peter Grayson
    • Michigan
      • Ann Arbor, Michigan, United States, 48109
        • Recruiting
        • University of Michigan, Internal Medicine
        • Contact:
        • Contact:
          • Ora Gewurz-Singer
        • Principal Investigator:
          • Professor William J McCune
    • Minnesota
      • Rochester, Minnesota, United States, 55905
        • Recruiting
        • Mayo Clinic
        • Contact:
        • Principal Investigator:
          • Eric Matteson
    • New Hampshire
      • Lebanon, New Hampshire, United States, 03756
        • Recruiting
        • Dartmouth-Hitchcock Medical Centre, Lebanon, NH
        • Contact:
        • Principal Investigator:
          • Daniel A Albert
    • New York
      • New York, New York, United States, 10016
        • Not yet recruiting
        • New York University Langone Medical Centre
        • Contact:
        • Principal Investigator:
          • Yusuf Yazici
    • North Carolina
      • Chapel Hill, North Carolina, United States, 27599-7525
        • Not yet recruiting
        • University of North Carolina
        • Contact:
        • Principal Investigator:
          • Charles Jennette
        • Principal Investigator:
          • Ronald Falk
    • Ohio
      • Cleveland, Ohio, United States, 44195
        • Recruiting
        • Cleveland Clinic
        • Contact:
        • Principal Investigator:
          • Leonard Calabrese
    • Pennsylvania
      • Philadelphia, Pennsylvania, United States, 19104
        • Recruiting
        • University of Pennsylvania
        • Contact:
        • Principal Investigator:
          • Peter A Merkel
      • Pittsburgh, Pennsylvania, United States, 15261
        • Recruiting
        • University of Pittsburgh
        • Contact:
        • Principal Investigator:
          • Kim Liang
    • Utah
      • Salt Lake City, Utah, United States, 84132-0002
        • Not yet recruiting
        • University Medical Center
        • Contact:
        • Principal Investigator:
          • Curry Koening

Participation Criteria

Researchers look for people who fit a certain description, called eligibility criteria. Some examples of these criteria are a person's general health condition or prior treatments.

Eligibility Criteria

Ages Eligible for Study

18 years and older (Adult, Older Adult)

Accepts Healthy Volunteers

No

Genders Eligible for Study

All

Sampling Method

Non-Probability Sample

Study Population

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.

Description

Inclusion Criteria for Classification criteria:

  1. Adult patients aged >18 years. There is no upper age limit.
  2. 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.
  3. Presumed diagnosis of a primary systemic vasculitis.

Exclusion criteria for classification criteria:

  1. Patients < 18 years of age.
  2. Inability to provide informed consent.
  3. Hepatitis B or C
  4. 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.

Inclusion criteria for diagnostic criteria:

  1. Adult patients aged >18 years. There is no upper age limit.
  2. 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.
  3. Suspected diagnosis of a primary systemic vasculitis

Inclusion criteria for controls group for diagnostic criteria:

  1. Adult patients aged >18 years. There is no upper age limit.
  2. 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.
  3. 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.

Exclusion Criteria for diagnostic criteria:

  1. Patients under the age of 18
  2. Patient or next of kin unable or unwilling to provide informed consent or assent.

Study Plan

This section provides details of the study plan, including how the study is designed and what the study is measuring.

How is the study designed?

Design Details

  • Observational Models: Case-Control
  • Time Perspectives: Prospective

Cohorts and Interventions

Group / Cohort
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.

What is the study measuring?

Primary Outcome Measures

Outcome Measure
Time Frame
Develop new diagnostic and classification criteria for ANCA associated vasculitis and polyarteritis nodosa
Time Frame: 3 years
3 years

Collaborators and Investigators

This is where you will find people and organizations involved with this study.

Sponsor

University of Oxford

Collaborators

American College of Rheumatology
The European League Against Rheumatism (EULAR)
The Vasculitis foundation

Investigators

  • 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

Publications and helpful links

The person responsible for entering information about the study voluntarily provides these publications. These may be about anything related to the study.

General Publications

Helpful Links

Study record dates

These dates track the progress of study record and summary results submissions to ClinicalTrials.gov. Study records and reported results are reviewed by the National Library of Medicine (NLM) to make sure they meet specific quality control standards before being posted on the public website.

Study Major Dates

Study Start

January 1, 2011

Primary Completion (Anticipated)

December 1, 2017

Study Completion (Anticipated)

December 1, 2018

Study Registration Dates

First Submitted

February 9, 2010

First Submitted That Met QC Criteria

February 9, 2010

First Posted (Estimate)

February 10, 2010

Study Record Updates

Last Update Posted (Estimate)

August 19, 2016

Last Update Submitted That Met QC Criteria

August 18, 2016

Last Verified

August 1, 2016

More Information

Terms related to this study

Keywords

Additional Relevant MeSH Terms

Other Study ID Numbers

  • ACREULAR001

This information was retrieved directly from the website clinicaltrials.gov without any changes. If you have any requests to change, remove or update your study details, please contact register@clinicaltrials.gov. As soon as a change is implemented on clinicaltrials.gov, this will be updated automatically on our website as well.

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