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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.