Clinical diagnosis and management of large vessel vasculitis: giant cell arteritis

Area of Science:

• Rheumatology
• Vascular Medicine

Background:

• Large vessel vasculitis (LVV) encompasses primary inflammatory conditions of the aorta and its branches.
• Giant cell arteritis (GCA) and Takayasu arteritis (TA) are the predominant subtypes of LVV.
• Clinical symptoms arise from vascular stenosis, occlusion, dilation, and potential complications like aneurysm rupture or dissection.

Purpose of the Study:

• To provide a comprehensive overview of GCA and isolated aortitis in the first of a two-part series.
• To discuss the clinical manifestations, diagnostic challenges, and therapeutic strategies for LVV.

Main Methods:

• Review of current literature and clinical practices regarding GCA and isolated aortitis.
• Discussion of diagnostic limitations, including unreliable clinical evaluation and imperfect imaging modalities.
• Analysis of therapeutic approaches, including glucocorticoids, steroid-sparing agents, and surgical interventions.

Main Results:

• Clinical evaluation for LVV activity is often unreliable.
• Diagnostic tools, including acute phase reactants and imaging, have limitations in assessing inflammation.
• Glucocorticoids remain the primary treatment, but long-term use leads to adverse effects.

Conclusions:

• LVV management requires a multi-faceted approach due to diagnostic and therapeutic challenges.
• Adverse effects of long-term glucocorticoid therapy necessitate the exploration of steroid-sparing agents.
• Surgical and endovascular interventions are sometimes required but associated with common relapses.