Atypical Large Vessel Vasculitis Presenting With Cholestatic Liver Abnormalities: Case-Based Review

Area of Science:

• Rheumatology
• Vascular Medicine
• Hepatology

Background:

• Giant cell arteritis (GCA) typically presents with cranial symptoms, but atypical large vessel vasculitis (LVV) can manifest with a broader range of signs.
• LVV can affect various blood vessels beyond the temporal arteries, including coronary, aortic, intracranial, and hepatic arteries.
• Constitutional symptoms like fever of unknown origin in LVV can mimic neoplastic or infectious diseases, complicating diagnosis.

Purpose of the Study:

• To highlight the diagnostic challenges of atypical large vessel vasculitis (LVV).
• To underscore the significance of liver enzyme abnormalities in the presentation of LVV.
• To illustrate the effectiveness of immunosuppressive therapy in managing LVV-associated hepatic manifestations.

Main Methods:

• Review of three patients with eventually diagnosed atypical LVV.
• Diagnostic confirmation through temporal artery biopsy in two patients.
• Positron emission tomography/computed tomography (PET/CT) used for diagnosis in one patient.

Main Results:

• Atypical LVV cases presented with varied symptoms beyond typical cranial manifestations.
• Elevated alkaline phosphatase levels were a common hepatic abnormality observed in all reviewed patients.
• Alkaline phosphatase elevations demonstrated a rapid positive response to immunosuppressive treatment initiation.

Conclusions:

• Atypical LVV requires a high index of suspicion, especially when presenting with non-specific symptoms and liver enzyme abnormalities.
• Elevated alkaline phosphatase is a key indicator of hepatic involvement in LVV and responds well to treatment.
• Early diagnosis and initiation of immunosuppressive therapy are crucial for managing LVV and its diverse clinical manifestations.