ABSTRACT

AA amyloidosis occurs in association with chronic inflammatory disorders that give rise to an acute phase response, notably including rheumatoid arthritis, ankylosing spondylitis, inflammatory bowel disease, familial Mediterranean fever, and chronic infections. No specific treatment is currently available for this life-threatening disease and its management has traditionally focused on control of the underlying chronic inflammatory disease. Fibrillex is a novel therapeutic agent under investigation for the treatment of AA amyloidosis. Fibrillexis the first member of a new therapeutic classs being tested in the largest ever study in patients with AA amyloidosis. This international, multicenter trial provides valuable data that allows to examine and compare the natural history of AA amyloidosis with respect to character and extent of organ system involvement in many countries.