Abstract
A 24-year-old woman with congenital adrenal hyperplasia (CAH) was referred for evaluation
of elevated liver enzyme activities over the preceding 6 months. The patient was diagnosed
with CAH at the age 12 when she presented with irregular menses and hirsutism. Since
then, she had been on dexamethasone to maintain a normal menstrual cycle and prevent
hirsutism and acne. She had no history of chronic liver disease and drank alcohol
socially. An extensive workup for other treatable causes of liver disease was unrevealing.
Therefore, a liver biopsy was performed, which revealed extensive ballooned degenerative
hepatocytes containing Mallory-Denk hyalines. The ballooned hepatocytes were located
predominantly in centrilobular areas and without any accompanying steatosis. Even
though the histopathologic features are most compatible with alcoholic and/or nonalcoholic
steatohepatitis, it was not supported by the patient's medical history and clinical
presentation. The patient had a normal body mass index and only occasional alcohol
use. Based on the biopsy finding and clinical presentation, we postulated that the
abnormal liver enzyme and pathological features seen on the liver biopsy were secondary
to CAH and long-term use of glucocorticoid. A few studies have shown that patients
with CAH often develop metabolic abnormalities and insulin resistance, particularly
women treated with glucocorticoid for several years. To our knowledge, this is the
first report describing steatohepatitis secondary to CAH and prolonged glucocorticoid
treatment. It is important to be aware that steatohepatitis can develop in these patients
due to long-term glucocorticoid use and potentially lead to progressive liver damage.
Furthermore, in patients with CAH who develop abnormal liver enzyme activities a liver
biopsy is warranted to assess for steatohepatitis and any associated fibrosis. If
indeed fibrosis is already present, a consultation with the endocrinologist should
be undertaken in an effort to lower the dose of the glucocorticoids as much as possible
while still controlling the symptoms of the disease.
Keywords
steatohepatitis - congenital adrenal hyperplasia - glucocorticoid
