Proteinase 3 (PR3) is the main antigen for cANCA, anti-neutrophil cytoplasmic antibodies directed against the cytoplasm. PR3 antibodies have a strong connection to granulomatosis with polyangiitis (GPA), formerly known as Wegener’s granulomatosis. Up to 80% of patients – and possibly more of those with active disease – have these antibodies.
Granulomatosis with polyangiitis is a type of vasculitis, an autoimmune inflammation of the blood vessels, which typically affects the sinuses, nose, and throat; the lungs; and the kidneys.
Besides indirect immunofluorescence assays, immunoblots and ELISA tests for ANCA and anti-PR3, a biopsy of the affected organ is often performed to confirm the diagnosis.
ANCA tests offered by ORGENTEC Diagnostika
ANCA-3-Line (ORG 789)