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- W2979152034 abstract "Granulomatosis with Polyangiitis (GPA), is an ANCA-positive vasculitis that affects the small and medium size vessels of various organs, including those of respiratory tract, lungs, and the kidneys. Gastrointestinal manifestation of GPA is rare, with an estimated prevalence of 5 to 11%. Furthermore, severe gastrointestinal involvement such as bowel perforation or hemorrhage, are even rarer manifestations of GPA. Although the use steroids and cyclophosphamide has become the standard of practice for treating GPA, there is insufficient evidence on the treatment of patients with severe gastrointestinal involvement. We report a case of a 50 year old previously healthy man who presented to our hospital with a 3 month history of migratory polyarthritis and new onset abdominal pain, bloody diarrhea, and oral ulcers after returning from a trip to French Polynesia. Based on laboratory, serologic, radiographic (Figure 1), and endoscopic findings (Figure 2), he was diagnosed with GPA. Soon after the diagnosis, he suffered a colonic perforation, requiring an exploratory laparotomy (Figure 3). He was treated with Prednisone and Rituximab, which proved to be effective therapies in inducing and maintaining clinical remission. The patient currently remains 6 months out from his hospitalization, and has maintained clinical remission on rituximab and a steroid taper. He has continued to do well with no clinical evidence of disease activity, and normalization of his laboratory markers.Figure 1Figure 2Figure 3Prompt diagnosis of GPA is important, as expeditious initiation of therapy is often necessary to reduce mortality and morbidity of multiorgan involvement. Gastrointestinal involvement is an uncommon presentation of GPA. Although local necrotizing arteriolitis of the intestine has been seen in 24% of GPA cases at autopsy, clinically apparent gastrointestinal manifestations of GPA have been reported only at a rate of 5-11%. Moreover, early intestinal perforation or rectal bleeding are even rarer manifestations of the disease, and have only been reported in a small number of cases. Given its rarity, there is limited data concerning the management of severe intestinal involvement in GPA. There have been series of reports that have shown successful use of Rituximab in patients with intestinal perforation secondary to GPA. In our patient's case, early Rituximab therapy in combination with high dose steroids proved to be a safe and effective treatment of his severe gastrointestinal involvement. In conclusion, the presented case report illustrates that severe complications such as intestinal perforation and hemorrhage can be seen in early stage of GPA despite the rarity of intestinal involvement. In addition to high-dose steroids, anti-CD20 antibody Rituximab, was able to achieve successful induction and maintenance of remission." @default.
- W2979152034 created "2019-10-10" @default.
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- W2979152034 date "2015-10-01" @default.
- W2979152034 modified "2023-10-14" @default.
- W2979152034 title "In the Knick of Time: A Case of Bowel Perforation in Granulomatosis With Polyangiitis and the Use of Rituximab" @default.
- W2979152034 doi "https://doi.org/10.14309/00000434-201510001-00408" @default.
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