Spyridon Zouridis, MD, Omar Tageldin, MD, Cassidy Alexandre, MD, Joseph Choma, MD Albany Medical Center, Albany, NY
Introduction: It is well known that cystic fibrosis (CF) patients are more susceptible to hepatobiliary disorders. CF related liver disease (CFLD) is the most common spectrum in these patients including focal biliary cirrhosis, multilobular cirrhosis, non-cirrhotic portal hypertension, hepatic steatosis, neonatal cirrhosis. However, CF has been linked to other entities affecting the gallbladder and biliary ductal system. We report an atypical presentation of a case with ascending cholangitis with normal liver function tests in a patient with CF.
Case Description/Methods: A 60-year-old female with history of CF since childhood requiring lung transplant, pancreatic insufficiency, diabetes mellitus and cholecystectomy presented with two days of sharp, constant epigastric pain radiating to her back associated with nausea, vomiting and a 101 fever. She reported similar recurrent episodes during the last 6 months which resolved without hospitalization. Her exam was significant for 101.4 temperature, mild epigastric and RUQ abdominal tenderness. Beside leukopenia, all her labs were within normal limits including LFTs and lipase. CT scan showed no pneumoperitoneum or obstruction with mild bowel wall thickening suspicious for colitis. Blood, urine and stool cultures were obtained, and patient was started on Ciprofloxacin and Flagyl. Her blood cultures returned positive for E. faecalis within 24h. MRCP revealed marked dilatation of intrahepatic ducts with visible multifocal strictures suggestive of primary sclerosing cholangitis and CBD choledocholithiasis. Subsequently, ERCP was performed and showed cholangitis with purulent drainage. CBD stent was placed, and patient's clinical condition improved.
Discussion: Cystic Fibrosis has been linked with intrahepatic biliary ductal disease including ascending cholangitis. Ascending cholangitis is a severe condition with reported mortality rates up to 50% in advanced disease. Modern therapeutic approaches allowed to reduce mortality and are now at physicians’ disposal. Charcot’s triad is considered the classic presentation of cholangitis. However, clinicians should bear in mind that simultaneous presence of fever, abdominal pain and jaundice is a non-sensitive criterion with only 50-75% of patients presenting with cholangitis triad symptoms. As such work-up with abdominal imaging, MRCP and urgent GI consult to evaluate for possible ERCP should be timely initiated in high-risk patients or when cholangitis is clinically suspected.
Figure: ERCP images
Disclosures: Spyridon Zouridis indicated no relevant financial relationships. Omar Tageldin indicated no relevant financial relationships. Cassidy Alexandre indicated no relevant financial relationships. Joseph Choma indicated no relevant financial relationships.
Spyridon Zouridis, MD, Omar Tageldin, MD, Cassidy Alexandre, MD, Joseph Choma, MD. P1158 - Atypical Presentation of Ascending Cholangitis in a Patient with Cystic Fibrosis, ACG 2021 Annual Scientific Meeting Abstracts. Las Vegas, Nevada: American College of Gastroenterology.