title: Case of acute, resistant fulminant Wegener's granulomatosis successfully treated by rituximab.
abstract: 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.
  
  
  METHODS
  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.
  
  
  CONCLUSIONS
  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.
authors.affiliation: Division of Otorhinolaryngology, Faculty of Health Sciences, University of Stellenbosch/Tygerberg Hospital, Cape Town, South Africa. akebrahim@gmail.com
authors.forename: A K
authors.initials: AK
authors.lastname: Ebrahim
authors.forename: J W
authors.initials: JW
authors.lastname: Loock
mesh_terms: D000208:Acute Disease; D000328:Adult; D058846:Antibodies, Monoclonal, Murine-Derived / Q000627:therapeutic use*; D003520:Cyclophosphamide / Q000009:adverse effects / Q000627:therapeutic use; D003937:Diagnosis, Differential; D014890:Granulomatosis with Polyangiitis / Q000175:diagnosis* / Q000188:drug therapy*; D006801:Humans; D007155:Immunologic Factors / Q000627:therapeutic use*; D007166:Immunosuppressive Agents / Q000009:adverse effects / Q000627:therapeutic use; D008297:Male; D000069283:Rituximab
chemical_list: D058846:Antibodies, Monoclonal, Murine-Derived; D007155:Immunologic Factors; D007166:Immunosuppressive Agents; D000069283:Rituximab; D003520:Cyclophosphamide