Undetectable caeruloplasmin values in a patient with autoimmune chronic active hepatitis

Area of Science:

• Biochemistry
• Hepatology
• Internal Medicine

Background:

• Caeruloplasmin, an alpha 2 glycoprotein synthesized by the liver, plays a crucial role in copper transport.
• Elevated caeruloplasmin levels are typical in chronic liver diseases like chronic active hepatitis.
• Conversely, significantly low caeruloplasmin concentrations are a hallmark of Wilson's disease.

Observation:

• A case study involving a 17-year-old female patient presented with markedly diminished serum caeruloplasmin levels.
• The patient was simultaneously diagnosed with chronic active hepatitis of the lupoid type.
• This presentation posed a diagnostic challenge, differentiating between Wilson's disease and other hepatic conditions.

Findings:

• The patient exhibited very low caeruloplasmin values, atypical for chronic active hepatitis alone.
• Despite the presence of chronic active hepatitis, the low caeruloplasmin levels initially suggested Wilson's disease.
• Treatment with corticosteroids led to a notable increase in serum caeruloplasmin concentration.

Implications:

• This case highlights that very low caeruloplasmin levels are not exclusive to Wilson's disease and can be observed in certain chronic liver conditions.
• The findings suggest that steroid therapy may positively influence caeruloplasmin levels in patients with autoimmune hepatitis.
• Effective management of the underlying liver disease can potentially restore normal or near-normal caeruloplasmin concentrations.