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Successful use of TNFα blockade in a severe case of idiopathic non-granulomatous ulcerative jejunoileitis associated with thrombotic thrombocytopenic purpura
  1. Fabian Braun1,2,
  2. Victor Suarez1,
  3. Johanna Dinter3,
  4. Stefan Haneder4,
  5. Alexander Quaas5,
  6. Thomas Benzing1,
  7. Dirk Nierhoff3,
  8. Roman-Ulrich Müller1
  1. 1 Department II of Internal Medicine and Center for Molecular Medicine Cologne, University of Cologne, Faculty of Medicine and University Hospital Cologne, Cologne, Germany
  2. 2 III. Department of Medicine, University Medical Center Hamburg-Eppendorf, Hamburg, Germany
  3. 3 Department of Gastroenterology and Hepatology, University of Cologne, Cologne, Germany
  4. 4 Institute of Diagnostic and Interventional Radiology, University of Cologne, Cologne, Germany
  5. 5 Department of Pathology, University of Cologne, Cologne, Germany
  1. Correspondence to Dr Roman-Ulrich Müller, Department II of Internal Medicine and Center for Molecular Medicine Cologne, University of Cologne, Faculty of Medicine and University Hospital Cologne, Cologne, Cologne, Germany; roman-ulrich.mueller{at}uk-koeln.de; Dr Dirk Nierhoff, ; dirk.nierhoff{at}uk-koeln.de

Abstract

We describe the case of 50-year-old female patient who presented with severe gastrointestinal symptoms and progressive weight loss of unknown origin. Shortly after admission, she developed an acute flare of thrombotic thrombocytopaenic purpura (TTP) that had to be treated by plasma exchange therapy and rituximab administration. While the signs of TTP subsided, the gastrointestinal symptoms worsened with abdominal cramps, massive gastric retention, malnourishment and a stenosis due to extensive inflammation and wall thickening of the small bowel. Extensive diagnostic efforts yielded no specific cause, so the patient—based on the histopathological findings—was diagnosed with idiopathic non-granulomatous ulcerative jejunoileitis. Following a highly complicated clinical course over several months, successful remission of the inflammatory activity and recovery of the patient could be obtained by TNF-alpha blockade.

  • inflammatory bowel disorders
  • tnf-alpha
  • infliximab

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Footnotes

  • DN and R-UM are joint senior authors.

  • FB and VS contributed equally.

  • Contributors FB, VS and JD were the patient’s primary physicians. SH and AQ interpreted the histological and radiological diagnostics, prepared and provided figures. TB, R-UM and DN supervised the treatment. FB and VS wrote the manuscript. All authors revised the manuscript.

  • Competing interests None declared.

  • Patient consent for publication Obtained.

  • Provenance and peer review Not commissioned; externally peer reviewed.

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