Giant Cell Hepatitis - A Rare Association with Connective Tissue Disease

Area of Science:

• Hepatology and Rheumatology
• Autoimmune Diseases
• Connective Tissue Disorders

Background:

• Giant cell hepatitis (GCH) is a rare liver condition.
• Undifferentiated connective tissue disease (UCTD) involves overlapping autoimmune symptoms.
• Antiphospholipid syndrome (APS) is associated with autoimmune conditions and thrombosis.

Observation:

• A 68-year-old male presented with asymptomatic, persistently abnormal liver function tests.
• Liver biopsy confirmed giant cell hepatitis (GCH).
• The patient also exhibited interstitial lung disease (ILD) and features of UCTD, including Raynaud's phenomenon and capillaritis.
• Extensive antibody testing revealed positive antinuclear antibody (ANA), anti-PM-SCL, and triple-positive antiphospholipid antibodies (aPL).

Findings:

• The patient was diagnosed with GCH in the context of UCTD and secondary antiphospholipid syndrome.
• This represents a unique presentation of GCH co-occurring with UCTD.
• Treatment with mycophenolate mofetil (MMF) and hydroxychloroquine led to symptom resolution.

Implications:

• This case underscores the importance of considering autoimmune etiologies in unexplained liver disease.
• It highlights the potential for overlap between GCH, UCTD, and APS.
• Mycophenolate mofetil (MMF) demonstrates efficacy in managing this complex autoimmune presentation.