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

Sponsor:	University of Oxford
Collaborators:	American College of Rheumatology The European League Against Rheumatism (EULAR) The Vasculitis foundation
Information provided by (Responsible Party):	University of Oxford
ClinicalTrials.gov Identifier:	NCT01066208

Purpose

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.

Condition
Wegener's Granulomatosis Microscopic Polyangiitis Churg Strauss Syndrome Polyarteritis Nodosa Giant Cell Arteritis Takayasu Arteritis

Study Type:	Observational
Study Design:	Observational Model: Case Control Time Perspective: Prospective
Official Title:	ACR/EULAR Endorsed Study to Develop New Diagnostic and Classification Criteria for Primary Systemic Vasculitis

Resource links provided by NLM:

MedlinePlus related topics: Giant Cell Arteritis Polymyalgia Rheumatica Vasculitis Wegener's Granulomatosis

U.S. FDA Resources

Further study details as provided by University of Oxford:

Primary Outcome Measures:

Develop new diagnostic and classification criteria for ANCA associated vasculitis and polyarteritis nodosa [ Time Frame: 3 years ] [ Designated as safety issue: No ]

Biospecimen Retention: Samples With DNA

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.

Estimated Enrollment:	3588
Study Start Date:	January 2011
Estimated Study Completion Date:	December 2013
Estimated Primary Completion Date:	June 2013 (Final data collection date for primary outcome measure)

Groups/Cohorts
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.

Show Detailed Description

Eligibility

Ages Eligible for Study:	18 Years and older
Genders Eligible for Study:	Both
Accepts Healthy Volunteers:	No
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.

Criteria

Inclusion Criteria for Classification criteria:

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.

Exclusion criteria for classification criteria:

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.

Inclusion criteria for diagnostic criteria:

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

Inclusion criteria for controls group for diagnostic criteria:

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.

Exclusion Criteria for diagnostic criteria:

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

Contacts and Locations

Please refer to this study by its ClinicalTrials.gov identifier: NCT01066208

Contacts

Contact: Raashid A Luqmani	+44 1865 738106	raashid.luqmani@ndorms.ox.ac.uk
Contact: Anthea Craven		anthea.craven@ndorms.ox.ac.uk

Show 47 Study Locations

Sponsors and Collaborators

University of Oxford

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	Boston University Vasculitis Center
Principal Investigator:	Richard Watts, DM, FRCP	University of East Anglia

More Information

Additional Information:

Website for European Vasculitis Study Group (EUVAS) This link exits the ClinicalTrials.gov site

Publications:

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.

Rao 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.

Hunder 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. No abstract available.

Fries 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. No abstract available.

Jennette 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. Review.

Sørensen 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.

Felson DT, Anderson JJ. Methodological and statistical approaches to criteria development in rheumatic diseases. Baillieres Clin Rheumatol. 1995 May;9(2):253-66. Review.

Responsible Party:	University of Oxford
ClinicalTrials.gov Identifier:	NCT01066208 History of Changes
Other Study ID Numbers:	ACREULAR001
Study First Received:	February 9, 2010
Last Updated:	January 6, 2012
Health Authority:	United Kingdom: Research Ethics Committee

Keywords provided by University of Oxford:

Classification criteria
Diagnostic criteria

Additional relevant MeSH terms:

Arteritis
Churg-Strauss Syndrome
Polyarteritis Nodosa
Takayasu Arteritis
Aortic Arch Syndromes
Giant Cell Arteritis
Polymyalgia Rheumatica
Vasculitis
Wegener Granulomatosis
Systemic Vasculitis
Microscopic Polyangiitis
Vascular Diseases
Cardiovascular Diseases
Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis
Granuloma

Lymphoproliferative Disorders
Lymphatic Diseases
Autoimmune Diseases
Immune System Diseases
Skin Diseases, Vascular
Skin Diseases
Aortic Diseases
Vasculitis, Central Nervous System
Autoimmune Diseases of the Nervous System
Nervous System Diseases
Cerebrovascular Disorders
Brain Diseases
Central Nervous System Diseases
Muscular Diseases
Musculoskeletal Diseases

ClinicalTrials.gov processed this record on May 23, 2012