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Published ahead of print on October 13, 2005, doi:10.1164/rccm.200507-1144OC

Am. J. Respir. Crit. Care Med., Volume 173, Number 2, January 2006, 180-187

A more recent version of this article appeared on January 15, 2006
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Submitted on July 25, 2005
Accepted on October 7, 2005

Rituximab for Refractory Wegener's Granulomatosis: Report of A Prospective, Open-Label Pilot Trial

Karina A Keogh1, Steven R Ytterberg2, Fernando C Fervenza3, Kimberly A Carlson1, Darrell R Schroeder4, and Ulrich Specks1*

1 Division of Pulmonary and Critical Care Medicine, Mayo Clinic, Rochester, MN, USA, 2 Division of Rheumatology, Mayo Clinic, Rochester, MN, USA, 3 Department of Medicine, Division of Nephrology and Hypertension, Mayo Clinic, Rochester, MN, USA, 4 Division of Biostatistics, Mayo Clinic, Rochester, MN, USA

* To whom correspondence should be addressed. E-mail: specks.ulrich{at}mayo.edu.

Rationale: Standard therapy for Wegener's granulomatosis is fraught with substantial toxicity and not always effective. B lymphocytes have been implicated in the pathogenesis of Wegener's granulomatosis. Their depletion has been proposed as salvage therapy for refractory disease. Earlier encouraging reports are confounded by concomitant immunosuppressive medications and include only limited available biomarker data. Objectives: To evaluate efficacy and safety of rituximab for remission induction in refractory Wegener's granulomatosis. Methods: Prospective open-label pilot trial in 10 patients followed for one year. Included were patients with active severe ANCA-associated vasculitis, ANCA-positivity, resistance to - or intolerance of cyclophosphamide. The remission induction regimen consisted of oral prednisone (1 mg/kg/day) and four weekly infusions of rituximab (375mg/m2). Prednisone was tapered and discontinued over 5 months. Failure to achieve remission, a clinical flare in the absence of B lymphocytes, or inability to complete the glucocorticoid taper, were considered treatment failures. Main Results: Three women and 7 men, median age of 57 (25-72), were enrolled. All had ANCA reacting with proteinase 3. The median activity score at enrollment was 6 (5-10). All patients tolerated rituximab well, achieved swift B lymphocyte depletion and complete clinical remission (activity score=0) by 3 months, and were tapered off glucocorticoids by 6 months. Five patients were retreated with rituximab alone for recurring/rising ANCA titers according to protocol. One patient experienced a clinical flare after B lymphocyte reconstitution. Conclusions: In this cohort, rituximab was a well-tolerated and effective remission induction agent for severe refractory Wegener's granulomatosis.
Key words: Wegener granulomatosis, ANCA, rituximab, treatment




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