Introduction. We describe the case of a woman with an unusual presentation of Wegener's granulomatosis. Case presentation. 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. Conclusion: In critically ill patients with severe, therapy-refractory ulcerative colitis, Wegeneŕs granulomatosis should be considered and serologic antibody testing should be performed. © 2013 Timmermann et al.; licensee BioMed Central Ltd.
CITATION STYLE
Timmermann, S., Perez Bouza, A., Junge, K., Neumann, U. P., & Binnebösel, M. (2013). Initial diagnosis of Wegener’s granulomatosis mimicking severe ulcerative colitis: A case report. Journal of Medical Case Reports, 7. https://doi.org/10.1186/1752-1947-7-141
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