Pilot Study of Short-Course Glucocorticoids and Rituximab for Treatment of ANCA-Associated Vasculitis

The primary aim of this pilot study is to examine whether an 8 week course of
glucocorticoids, in combination with rituximab, is effective in inducing and maintaining
disease remission for up to 6 months in a subset of patients with ANCA-associated vasculitis
(AAV) who have a more favorable prognosis.

This pilot study will enroll 20 patients with active AAV. Close patient follow-up will insure
that any patients who require courses of glucocorticoids longer than two months will receive
longer therapy, if appropriate for their well-being.

Inclusion Criteria:

- Patients ages 18-85 years old

- Diagnosis of GPA or MPA according to the definitions of the Chapel Hill Consensus
Conference

- New diagnosis or disease flare with a Birmingham Vasculitis Activity Score/Wegener's
granulomatosis (BVAS/WG) of > 3

Exclusion Criteria:

- Renal disease in patients with PR3-ANCA as defined by any of the following:

- Urinary red blood cell casts

- Biopsy-proven glomerulonephritis

- Increase in serum creatinine of >30% over baseline

- Severe renal disease in patients with MPO-ANCA as defined by both of the following:

- Urinary red blood cell casts or biopsy-proven glomerulonephritis

- Estimated glomerular filtration rate < 30 ml/min/1.73m2

- Diffuse alveolar hemorrhage requiring ventilatory support

- GC treatment for longer than 14 days prior to enrollment unless patient has been on a
stable maintenance dose of prednisone at the time of the flare

- Daily oral cyclophosphamide within 1 month prior to enrollment

- Completed a remission induction course of cyclophosphamide or rituximab within 4
months of enrollment

- Hepatitis B infection

- HIV infection

- History of anti-GBM disease

- Other uncontrolled disease, including drug and alcohol abuse, that may interfere with
the study

- Pregnancy or breastfeeding

- History of severe allergic reactions to human or chimeric monoclonal antibodies