ANCA-Associated Vasculitis

Posted by • July 16th, 2010

Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis, including Wegener’s granulomatosis and microscopic polyangiitis, is a multisystem autoimmune syndrome characterized by vasculitis predominantly affecting microscopic vessels and circulating autoantibodies to neutrophil cytoplasmic antigens.

Jones et al. report that in a randomized trial, standard glucocorticoid regimen plus rituximab was not superior to standard intravenous cyclophosphamide as induction therapy in patients with newly diagnosed ANCA-associated vasculitis and renal involvement. Rates of sustained remission were high in both groups.

Clinical Pearls

What is the renal involvement in patients with ANCA-associated vasculitis?

Renal involvement occurs in 70% of affected patients and is manifested as rapidly progressive glomerulonephritis with pauci-immune necrotizing, crescentic glomerulonephritis on biopsy.

What is the current standard of care for ANCA-associated vasculitis?

The current standard of care for ANCA-associated vasculitis is cyclophosphamide with high-dose glucocorticoids; this regimen is associated with leukopenia, severe infections, cancer, and ovarian failure. Mortality at 1 year exceeds 15%; infection and active vasculitis are the predominant causes of early death. The partial efficacy and severe toxicity of this regimen indicate a need for better induction therapy in ANCA-associated vasculitis.

Morning Report Questions

Q: What is the mechanism of action of rituximab?

A: Rituximab is a B-cell-depleting anti-CD20 monoclonal antibody that has been approved by the European Medicines Agency and the U.S. Food and Drug Administration for the treatment of non-Hodgkin’s lymphoma and rheumatoid arthritis.

Q: What was the comparative efficacy of cyclophosphamide- and rituximab-based regimens to treat ANCA-associated vasculitis?

A: A rituximab-based regimen was not superior to standard intravenous cyclophosphamide for severe ANCA-associated vasculitis. Sustained-remission rates were high in both groups, and the rituximab-based regimen was not associated with reductions in early severe adverse events.

Figure 2. Cumulative Incidence of Remission and Cumulative Proportion of Patients with a Severe Adverse Event.

One Response to “ANCA-Associated Vasculitis”

  1. drwael says:

    what is the mechanism of Nephrotic syndrome with vasculitis