Antineutrophil cytoplasmic antibodies (ANCA) constitute a family of auto-antibodies directed against various components of the neutrophil cytoplasm. The indirect immunofluorecence assays detected three fluorescent staining patterns: cANCA--cytoplasmic; pANCA--perinuclear and aANCA--atypical. Occurence ANCA is mainly associated with Wegener's granulomatosis and vasculitis, but they are also detected in autoimmune diseases (eg. in systemic lupus erythematosus, in rheumathoid arthritis, Sjögren's syndrome, in dermatomyositis) and in inflamatory bowel diseases (Crohn disease, colitis ulcerosa). Presence of ANCA was found also in primary sclerosing cholangitis, in chronic infections and in person using some kinds of drugs. The aim of the study was to review recent investigations concerning prevalence of ANCA and their diagnostic value not only for vasculitis but also for the other disease in which they are detected.