Chronic intestinal pseudo-obstruction due to ß2microglobulin-amyloidosis in a patient on high-flux haemodialysis.

ABSTRACT

Dialysis-related amyloidosis (DRA) or ß2microglobulin (ß2m)-amyloidosis is a disorder caused by the inability to clear a protein called ß2m in patients with chronic kidney disease. It results in deposition of ß2m as amyloid fibrils, most commonly in bones and joints. Infrequently, visceral organs may be involved. With modern high-flux haemodialysis, DRA has become a rare disease, yet it may occur. We present a case of DRA in an 86-year-old woman. This case is particularly notable for its rare presentation as chronic intestinal pseudo-obstruction. It is of paramount importance to recognise this entity in order to reduce delay in treatment and avoid patients being frustrated not getting a diagnosis.