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Test Category: Immunology

Investigation Name:Proteinase 3 antibody (PR3)

Alias or Abbreviation:PR3, ANCA

Intro:

Indication: Granulomatosis with polyangiitis (GPA, formerly called Wegener's granulomatosis) Further information: In accordance with the revised 2017 international consensus on testing of ANCAs, ANCA requests should be confined to patients with a high pre-test probability of small vessel vasculitis, if misleading 'false positives' in a range of non-vasculitic disorders are to be avoided. Indications are: • Glomerulonephritis, especially rapidly progressing glomerulonephritis • Pulmonary haemorrhage, especially pulmonary renal syndrome • Acute pulmonary-renal syndrome • Cutaneous vasculitis with systemic features • Multiple lung nodules • Chronic destructive disease of the upper airways • Long-standing sinusitis or otitis • Granulomatous nasopharyngeal lesions • Subglottic tracheal stenoses • Mononeuritis multiplex, or other peripheral neuropathy • Retro-orbital mass • Scleritis Antibodies to PR3 are highly sensitive and specific for GPA. The sensitivity is dependent on the phase and on the activity of the disease. PR3 antibodies may also be seen in MPA and EGPA. PR3 antibodies may also occur in 20% to 30% of patients with necrotizing glomerulonephritis with no obvious extrarenal manifestations of small vessel vasculitis. NOTE: Results >2.0 IU/mL are reflexed for ANCA test on new patients only For urgent requests, contact the Laboratory directly

Requestable Seperately?No

Minimum Sample Volume:1mL

Expected Turnaround Time:Frequency: Daily Target: 3 days

Test Code:ANCA

Sample Type:

Serum Gel Tube (Gold top)/ Plasma

Complex Reference Range:<2 IU/mL = Negative

Collection Conditions / Other Information:1st class post or Transport

Referred to Another Laboratory?No

Storage Requirements:Ambient temperature

NPEX / PDF Reporting Available:Yes

EQA Scheme:Yes

Further information:Orderable in panel with MPO

Lead Contact Details:rwh-tr.bcpsimmunologyclinicalqueries@nhs.net

Email Address For Chasing Results:rwh-tr.ImmunologyLab@nhs.net

Methodology:Fluoroenzymeimmunoassay

UKAS Reference Number:9580 (recommended)

Site Sample Tested:Immunology, New Cross Hospital, Wolverhampton