Feature Article

Systemic vasculitides. Part 1: Large, medium and variable vessel diseases



© zephyr/science photo library
© zephyr/science photo library


Vasculitis, or inflammation of vessel walls, is common and consists of a diverse array of disease entities encompassing virtually all organ systems. As onset is typically subacute and nonspecific, affected patients usually first seek attention from primary care providers. Size and distribution of the affected vasculature dictate end-organ manifestations and preliminary screening tests. Complications may carry high morbidity and mortality; therefore, prompt recognition, specialist referral and institution of immunosuppressive therapy are crucial to obviate potentially poor outcomes.

Key Points

  • Systemic vasculitides are a group of important diseases caused by vessel wall inflammation and its end-organ effects. They are classified by the size of the vessels affected.
  • Although many cases are idiopathic, important secondary causes of vasculitis include connective tissue diseases, haematological malignancies and chronic infections. These conditions must be considered when investigating patients with vasculitis.
  • Lethal and highly morbid complications of vasculitis including visual compromise, renal dysfunction, haemoptysis, vascular aneurysms and occlusive vascular disease must be considered when diagnosing vasculitis. Early specialist involvement is crucial if these complications are present.
  • Management involves the use of corticosteroids and immunosuppressant medications, thus chronic complications such as osteoporosis, hypertension, diabetes mellitus and risk of opportunistic infections and secondary malignancies must be addressed in the long-term management of patients with vasculitis.