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

Launched by UNIVERSITY OF OXFORD · Feb 9, 2010

Keywords

ClinConnect Summary

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 po...

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ALL

Eligibility criteria

• 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.