Case of acute, resistant fulminant Wegener's granulomatosis successfully treated by rituximab

Background:Wegener's granulomatosis is generally a chronic, indolent, inflammatory condition, treated with cytotoxics (cyclophosphamide) and corticosteroids.Objective:This paper reports an unusual case of acute fulminant Wegener's granulomatosis that failed to respond to conventional treatment, but showed a dramatic response to rituximab, which is a relatively new form of treatment for resistant cases.Method:As well as describing the case (with photographic illustrations), the current paper provides a review of the literature, focusing on acute Wegener's granulomatosis and frequency of resistance to ‘conventional’ forms of treatment. There is also an evaluation of the evidence for the effectiveness of rituximab in resistant Wegener's granulomatosis.Results:The patient responded remarkably well to rituximab and had no disease recurrence at 24 months' follow up.Conclusion:Clinicians should be aware of the acute fulminant form of Wegener's granulomatosis, as a delay in diagnosis and treatment may have fatal consequences. The paper also highlights the dramatic response to rituximab experienced by the patient.