Amyloidosis and inflammatory bowel disease: fact or myth?

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Emanuele Sinagra
Marco Ciofalo
Giovanni Tomasello
Francesco Cappello
Gaetano Cristian Morreale
Georgios Amvrosiadis
Provvidenza Damiani
Francesco Damiani
Giancarlo Pompei
Aroldo Gabriele Rizzo
Carmelina Canale
Giuseppe Mastrocinque
Francesco Carini
Dario Raimondo

Keywords

Amyloidosis, Crohn’s disease, Ulcerative colitis, Inflammatory Bowel Disease

Abstract

Inflammatory Bowel Disease (IBD), which includes both Crohn’s Disease (CD) and Ulcerative Colitis (UC), is a chronic idiopathic inflammatory disorder affecting the gastrointestinal tract. Extraintestinal manifestations (EIMs) are common in patients with IBD, and occur in 6%-47% of patients with CD or UC. EIMs can involve organs other than the gastrointestinal tract such as skin, eyes, joints, biliary tract, and kidneys. Renal and urinary involvement particularly occurs in 4-23% of patients with IBD. Among the renal complications of IBD, secondary amyloidosis (AA-type, AAA) is a rare but serious complication. Renal amyloidosis has been proven to be the most common lethal manifestation of IBD-associated amyloidosis, since renal involvement rapidly leads to end-stage renal failure. A few studies suggest that AAA is more prevalent in CD than in UC, mainly occurring in male patients with an extensive, long-lasting, and penetrating disease pattern. The therapeutic approaches of IBD-associated AAA are based both on control of the chronic inflammatory process that causes the production and storage of serum amyloid A (SAA), which is a precursor of the amyloid, as well as on destabilizing amyloid fibrils so that they can no longer maintain their β-pleated sheet configuration; however, in patients with end-stage renal disease, the only therapeutic options still available are hemodyalisis and renal transplantation. Whether effective treatment exists for AAA remains controversial.
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