Top 10 Topics in Vasculitis from a Rheumatologist’s Perspective

Arthur Kavanaugh, MD

Dr Kavanaugh, a leading Rheumatologist, provides us with the top ten topics in vasculitis from his perspective.

  1. There has been substantial change in the nomenclature for systemic vasculitides. Wegener’s granulomatosis is now being called GPA (granulomatosis with polyangiitis) and Churg Strauss syndrome is now being called EGPA (eosinophilic granulomatosis with polyangiitis). The 3rd similar systemic vasculitis is microscopic polyangiitis (MPA), which is distinct from polyarteritis nodosa (PAN).
  2. GPA, EGPA and PAN are sometimes known as ANCA associated vasculitides because of their potential association with anti-neutrophil cytoplasmic antibodies (ANCA).
  3. In addition to vessel size and ANCA, other factors relevant for vasculitides include the extent of involvement (limited vs diffuse) and the response to treatment.
  4. Systemic Vasculitis can have diverse manifestations. The skin is involved in approximately 40-65% of patients with GPA, EGPA, MPA and PAN.
  5. Internal organ involvement can be severe and serious and affect multiple organs. However, most areas of involvement will be apparent with only a directed history and physical examination, and a few straightforward laboratory tests and imaging procedures.
  6. ANCA testing can be of value, but results MUST be interpreted in the correct clinical context. The quality of the lab is very important.
  7. ANCA testing should not be relied upon as a predictor of disease activity for individual patients.
  8. Treatment options for systemic vasculitides include methotrexate for many more limited disease manifestations, and cyclophosphamide for more severe disease. Treatment often must be continued to prevent relapses.
  9. Rituximab has gained increasing attention as an effective therapy for severe systemic vasculitis.
  10. There are mimics of vasculitis that clinicians should be aware of.