title: Initial diagnosis of Wegener's granulomatosis mimicking severe ulcerative colitis: a case report.
abstract: BACKGROUND
  We describe the case of a woman with an unusual presentation of Wegener's granulomatosis.
  
  
  METHODS
  A 20-year old Caucasian woman presented with the principal feature of a pancolonic, superficial microulceration mimicking severe ulcerative colitis. Our patient was refractory to therapy and had persisting signs of septic shock as well as being at risk of perforation, so we performed a subtotal colectomy and a cholecystectomy due to the incipient necrosis of her gallbladder. Histologic analysis of her colon showed multiple superficial microulcera of the mucosa, lamina propria mucosae and, to a lesser extent, the lamina submucosa. The medium-sized arteries and arterioles of her entire colon, appendix and gallbladder showed acute vasculitic changes with fibrinoid necrosis of the walls and diffuse infiltration with neutrophil granulocytes, accompanied by a strong perivascular histiocyte-rich and partially granulomatous reaction. These findings strongly suggested an autoimmune multisystem disease like Wegener's granulomatosis or microscopic polyangiitis. A diagnosis of Wegener's granulomatosis was confirmed by the results of serologic antibody tests: her cytoplasmic antineutrophil cytoplasmic antibody titer was considerably elevated at 1:2560 specific for subclass proteinase 3 (>200kU/L). After the histopathological diagnosis and serological tests, immunosuppression with high doses of corticosteroids and plasmapheresis was started.
  
  
  CONCLUSIONS
  In critically ill patients with severe, therapy-refractory ulcerative colitis, Wegener´s granulomatosis should be considered and serologic antibody testing should be performed.
authors.affiliation: Department of General, Visceral and Transplantation Surgery, University Hospital of the RWTH Aachen, Aachen, Germany. mbinneboesel@ukaachen.de.
authors.forename: Sonja
authors.initials: S
authors.lastname: Timmermann
authors.forename: Alberto
authors.initials: A
authors.lastname: Perez Bouza
authors.forename: Karsten
authors.initials: K
authors.lastname: Junge
authors.forename: Ulf P
authors.initials: UP
authors.lastname: Neumann
authors.forename: Marcel
authors.initials: M
authors.lastname: Binnebösel