Detectable anti-proteinase-3 antibodies precede clinical manifestations in a case of anti-neutrophil cytoplasmic antibody-associated vasculitis

Anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitides (AAVs) are devastating clinical syndromes characterized by small vessel vasculitis occasioning multiorgan dysfunction, such as renal and pulmonic involvement, and ANCAs. These antibodies are chiefly directed against proteinase-3 (PR3) and myeloperoxidase (MPO) antigens. We describe a rare case of AAV where anti-PR3 antibodies were detected in the serum months before the onset of the clinical syndrome. We also discuss the emerging concept of ‘pre-disease’ status with rheumatic diseases.