From the Guidelines
An uncommon cause of fatty liver in young women is Wilson's disease, a rare genetic disorder affecting copper metabolism. This condition leads to copper accumulation in the liver, brain, and other organs, causing liver damage that can present as fatty liver 1. According to the American Association for the Study of Liver Diseases, Wilson's disease is a known cause of macrovesicular steatosis, a type of fatty liver disease 1. Patients typically need lifelong treatment with copper chelating agents like D-penicillamine or trientine, along with zinc supplements to block copper absorption.
Some key points to consider in the diagnosis and management of Wilson's disease include:
- Blood tests showing low ceruloplasmin levels and elevated 24-hour urinary copper excretion
- Liver biopsy with copper quantification may be necessary for diagnosis
- Treatment with copper chelating agents and zinc supplements can help manage the disease
- Early diagnosis is crucial as Wilson's disease is treatable but can lead to cirrhosis or liver failure if untreated
Other uncommon causes of fatty liver in young women include:
- Glycogen storage diseases
- Lipodystrophy
- Medications like tamoxifen or valproate, which can cause microvesicular or macrovesicular steatosis 1 Young women with unexplained fatty liver, especially with neurological symptoms or Kayser-Fleischer rings (copper deposits in the cornea), should be evaluated for these rare conditions even if they lack traditional risk factors like obesity or alcohol use.
From the Research
Uncommon Reasons for Fatty Liver in Young Women
- An uncommon reason for fatty liver in a young woman is the use of tamoxifen, a medication commonly used to treat breast cancer 2.
- Long-term use of tamoxifen has been shown to significantly aggravate fatty liver status compared to other treatment modalities in breast cancer patients 2.
- The development of fatty liver due to tamoxifen use is a concern, as it can lead to worsening of liver function and potentially increase the risk of other metabolic diseases.
Polycystic Ovary Syndrome (PCOS) and Fatty Liver
- PCOS is a common endocrine disorder in reproductive-aged women, and it has been linked to an increased risk of nonalcoholic fatty liver disease (NAFLD) 3, 4, 5, 6.
- Insulin resistance and hyperandrogenemia are potential links between PCOS and NAFLD 5, 6.
- Women with PCOS should be screened for NAFLD, particularly those with other metabolic risk factors, and lifestyle interventions and weight loss are recommended as first-line treatment 5, 6.