Neutrophil perinuclear antibodies generally refer to anti-neutrophil perinuclear antibodies, which are a hallmark antibody of autoimmune diseases. Anti-neutrophil cytoplasmic antibodies are categorized into cytoplasmic and perinuclear types. Neutrophil cytoplasmic antibodies, also known as cANCA, and neutrophil perinuclear antibodies, also known as pANCA, are generally detected by indirect immunofluorescence. pANCA shows a fluorescent pattern of perinuclear staining on ethanol-fixed slices of neutrophil substrate, with fluorescence-positive cytoplasmic concentration around the nucleus of the schizont. Anti-neutrophil cytoplasmic antibody has been shown to be the serum signature antibody for systemic vasculitis and is of paramount importance in the diagnosis, classification and prognosis of vasculitis. A positive pANCA indicates a high probability of the presence of a primary vasculitis such as microscopic polyangiitis and eosinophilic granulomatous polyangiitis. It is recommended that patients with positive neutrophil perinuclear antibody test should visit the hospital in time to find out the cause and avoid delay.