Primary Amyloidosis Presenting as Common Bile Duct Obstruction With Cholangitis

Abstract

A 61-year-old woman presented with features of acute cholangitis and distal common bile duct obstruction. Histopathology from ampulla of Vater biopsy demonstrated extensive local amyloid deposition. Amyloidomas can cause local obstructive effects and have been described in the small intestine, stomach, and gallbladder. This is the second case of a discrete amyloid deposit causing extrahepatic biliary obstruction and cholangitis.

Acute cholangitis - an update

“…The biliary obstruction is most commonly caused by choledocholithiasis. Other causes of obstruction include benign or malignant stricture of the bile duct or hepatic ducts, pancreatic cancer, ampullary adenoma or cancer, porta hepatis tumor or metastasis, biliary stent obstruction (due to microbial biofilm formation, biliary sludge deposition and duodenal reflux of food content), primary sclerosing cholangitis, amyloid deposition in the biliary system[ 3 ], Mirizzi syndrome (gallstone impacted in cystic duct or neck of the gall bladder causing compression on common bile duct or common hepatic duct), Lemmel’s syndrome (peri-ampullary diverticulum causing distal biliary obstruction), round worm ( Ascaris lumbricoides ) or tapeworm ( Taenia saginata ) infestation of the bile duct[ 4 ], acquired immunodeficiency syndrome (commonly known as AIDS) cholangiopathy and strictured bilioenteric anastomoses[ 5 ]. Choledochocele and narrow-caliber bile duct are other risk factors for acute cholangitis.…”

Diagnosis of AL‐type amyloidosis from bile duct wall thickening

“…Cases of amyloid deposition in the CBD with jaundice as an initial symptom are rare 1‐3 . In this case, EUS findings of irregular CBD wall thickening and preservation of the luminal hyperechoic layer suggested amyloid deposition in the submucosal layer of the bile duct, and were characteristic of bile duct amyloidosis.…”

“…Cases of amyloid deposition in the CBD with jaundice as an initial symptom are rare. [1][2][3] In this case, EUS findings of irregular CBD wall thickening and preservation of the luminal hyperechoic layer suggested amyloid deposition in the submucosal layer of the bile duct, and were characteristic of bile duct amyloidosis. These findings are atypical of cholangiocarcinoma, in which the luminal hyperechoic layer of the lesion disappears; primary sclerosing cholangitis, in which an intrahepatic bile duct stricture is observed; and IgG4-related sclerosing cholangitis, which presents with uniform wall thickening and preserved luminal hyperechoic layers throughout the CBD.…”

Kolangitis Akut dengan Komplikasi Perdarahan Saluran Cerna Bagian Atas dan Peningkatan Ca 19-9: Laporan Kasus

“…Patofisiologi kolangitis akut pada obstruksi bilier ialah empedu menjadi stagnan dalam sistem bilier, tekanan intraduktal meningkat, tight junction antara kolangiosit melebar, malfungsi sel Kupffer, dan produksi IgA menurun. 7 Tekanan koledokal berperan penting dalam patogenesis kolangitis akut. Tekanan duktus biliaris normal ialah 7-14 cm H 2 O. Ketika tekanan intraduktal melebihi 25 cm H 2 O, dapat terjadi refluks kolangiovenous dan kolangiolimfatik, yang menyebabkan bakteriemia dan endotoksinemia.…”