Kaiser Permanente Los Angeles Medical Center Los Angeles, CA, United States
Angeline Luong, MD, Bechien Wu, MD, MPH, Elizabeth Dong, MD Kaiser Permanente Los Angeles Medical Center, Los Angeles, CA
Introduction: Familial Mediterranean fever (FMF) is a hereditary inflammatory disorder characterized by recurrent fever with pain typically in the abdomen, chest or joints due to serosal inflammation. Chronic liver disease can be seen as a result of long-term complications of recurrent inflammation. However acute hepatitis is a rare entity that has not been well-described.
Case Description/Methods: A 30-year-old male with Mediterranean ancestry presented to a gastroenterology clinic for evaluation of elevated liver enzymes. He had a history of recurrent fevers of unknown origin since childhood. With each episode, he was noted to have elevated liver enzymes. At age 16, he had recurrent fevers for 8 weeks; during this time his alanine aminotransferase (ALT) was 54, aspartate aminotransferase (AST) 36, total bilirubin 0.7, alkaline phosphatase (ALP) 96. At age 25, he had fevers for 16 days, with ALT 300, AST > 500, total bilirubin 0.6, ALP 217. At the time of his visit at the gastroenterology clinic, he had been having fevers for the past 10 days. Labs drawn prior to his visit showed ALT 343, AST 124, total bilirubin 0.5, ALP 168. He denied any medication, herbal supplements, or drug use during this time period.
He had been recently evaluated by a rheumatologist who clinically diagnosed him with FMF and started colchicine. After 1 week of colchicine, his fevers resolved. Follow-up labs (in-between episodes) showed normalization of his liver enzymes. Subsequent serologic workup did not reveal any other etiology to explain his acute hepatitis. He had no evidence of a presence of chronic liver disease through ultrasound and computed tomography scan. He has been doing well clinically since starting colchicine therapy. Genetic testing was negative for presence of a MEFV gene mutation.
Discussion: This case illustrates an atypical presentation of FMF involving acute hepatitis in the absence of chronic liver disease. It is thought that MEFV mutations may contribute to hepatic inflammation, as cases have mainly been described in patients with homozygous gene mutations. However, 10-20% of patients who meet clinical criteria for FMF are negative for any known mutation, as with this patient. As such, physicians should be aware of this when evaluating a patient with a history of FMF with acute hepatitis. In addition, as FMF may be associated with NAFLD or cirrhosis due to recurrent inflammation or secondary amyloid deposition, careful monitoring of liver enzymes during both times of attacks and remission are warranted.
Figure: AST and ALT trends over time during and in-between episodes of recurrent fevers
Angeline Luong indicated no relevant financial relationships.
Bechien Wu indicated no relevant financial relationships.
Elizabeth Dong indicated no relevant financial relationships.
Angeline Luong, MD, Bechien Wu, MD, MPH, Elizabeth Dong, MD. P1864 - Recurrent Acute Hepatitis During Attacks of Familial Mediterranean Fever, ACG 2021 Annual Scientific Meeting Abstracts. Las Vegas, Nevada: American College of Gastroenterology.