Acute Acalculous Cholecystitis as Initial Presentation of Amyloidosis

Abstract

Amyloidosis is a relatively rare disease characterized by deposition of abnormal protein on any tissue or organ. It can present with a wide range of clinical manifestations depending on organ system involvement. We present here a 47-year-old Asian woman with history of hypothyroidism who came to emergency room with intermittent right upper quadrant abdominal pain for a month with worsening of symptoms for the last 3 - 4 days. Physical examination revealed mild abdomen distension, right upper quadrant tenderness, hepatomegaly and bipedal pitting edema. Her pertinent laboratory findings were serum albumin of 1.6, total bilirubin of 3.5, direct of 2.1, AST/ALT of 151/81 and alkaline phosphatase of 1,339. Her blood urea nitrogen/Cr was 20/1.6 and urinalysis was positive for proteinuria of 300 mg/dL. Ultrasound (US) of gallbladder revealed hepatomegaly, thickened gallbladder wall with 4 mm gall bladder polyps vs. non-shadowing stones concerning for acute cholecystitis. Her viral serologies were suggestive of past hepatitis B infection. Antinuclear antibody, anti-smooth muscle antibody, anti-mitochondrial antibody, alpha-1 antitrypsin, ceruloplasmin and iron profile were within normal limits. Liver biopsy was positive for amyloidosis. There are very few cases reported in the literature of amyloidosis initially presenting with features of acute cholecystitis and cholestatic pattern of liver function tests along with ascites. Our patient presented clinically as acute cholecystitis. The clinical dilemma was more when US commented on the findings as non-shadowing stones vs. polyps. Diffuse wall thickening of gall bladder as seen in US in this case might just be amyloid deposition rather than actual inflammation and the non-shadowing stones could as well be the amyloid deposition. Cholecystitis secondary to gallbladder localization of amyloidosis is very unusual. In conclusion, amyloidosis can rarely present as acute cholecystitis and there could be diagnostic dilemma because of inconclusive US findings.




J Med Cases. 2016;7(10):459-460
doi: http://dx.doi.org/10.14740/jmc2159w