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• W4387248877 abstract "SESSION TITLE: Critical Care Case Report Posters 67 SESSION TYPE: Case Report Posters PRESENTED ON: 10/11/2023 12:00 pm - 12:45 pm INTRODUCTION: Antineutrophil cytoplasmic autoantibody (ANCA)- associated vasculitidies are rare and life-threatening conditions. Pathologic data obtained from a biopsy is an additional option for diagnosis but not always available. Patients typically present with pulmonary or renal manifestations. CASE PRESENTATION: The patient is a 41-year-old female with no past medical history that presented with dyspnea, abdominal pain and lethargy. The patient was found to have severe lactic acidosis of 14.8 mmol/L on admission. CT scan of the abdomen revealed Inflammation in the cecum and the terminal ileum and a minimally elevated serum creatinine 1.30mg/dL. Her creatinine normalized with fluid resuscitation. She was admitted to ICU where she developed respiratory failure and septic shock due to Enterobacter peritonitis. An emergent exploratory laparotomy revealed ischemic bowel. Pathology showed mucosal ulceration and necrosis with acute serositis, with 114 cm of small bowel surgically removed and 20 cm of right colon removed. Hypercoagulable work up was negative. Antineutrophil cytoplasmic antibody (C-ANCA) testing was performed early into her admission and titers were initially equivocal at 1:20. Myeloperoxidase (MPO) antibody was negative and proteinase-3 (PR-3) antibody was significantly elevated on admission but later improved and attributed to false positive testing given the presence of an extended beta-lactamase (ESBL) producing Enterobacter infection. After a 46-day hospital stay, the patient was moved to an inpatient rehabilitation unit. After 21 days there the patient developed acute hypoxic respiratory failure with bilateral pulmonary infiltrates. She was empirically started on 1g of methylprednisolone daily and bronchoscopy revealed diffuse alveolar hemorrhage. PR-3 antibody was critical elevated at >800.0 units but C-ANCA titers were only mildly elevated at 1:40. Once infectious etiologies were ruled out, the patient was treated with rituximab. After 9 days in the hospital, she returned to inpatient rehabilitation, from where she was successfully discharged home and continues to follow with rheumatology. DISCUSSION: Our case highlights a unique and important presentation of ANCA associated vasculitidies. Our literature review yielded only ten instances of intestinal ischemia or necrosis related to an underlying vasculitis process, none of which had bowel ischemia as the presenting issue. However, Chetty & Serra note in their 2017 review in the Journal of Clinical Pathology that gastrointestinal manifestation of a systemic vasculitis process is possible as a sole manifestation of disease and recognize that is likely under diagnosed. It also notes the pertinence of sampling of the mesentery and additional vascular structure of the necrotic bowel to confirm underlying vascular process. This was a limitation in our case as pathology reports only minimal adipose tissue sent with bowel specimens. Additional limitations to diagnosis are the lack of specificity with conventional radiography and even endoscopy. Extra-intestinal causes are often helpful in diagnosis, but as in this case not consistently present. CONCLUSIONS: If this rare manifestation were more recognized, her initial elevation in PR-3 testing may not have been falsely attributed to her infection and prevention of morbidity could have happened with early intervention for her vasculitis disease. Adequate mesenteric sampling is recommended with submission of ischemic bowel for proper evaluation for underlying vasculitis. REFERENCE #1: Chetty R, Serra S.A pragmatic approach to vasculitis in the gastrointestinal tract.Journal of Clinical Pathology 2017;70:470-475. REFERENCE #2: Nakazawa D, Masuda S, Tomaru U, Ishizu A. Pathogenesis and therapeutic interventions for ANCA-associated vasculitis. Nat Rev Rheumatol. 2019 Feb;15(2):91-101. doi: 10.1038/s41584-018-0145-y. Erratum in: Nat Rev Rheumatol. 2019 Jan 17;: PMID: 30542206. REFERENCE #3: Shirai T, Fujii H, Saito S, Ishii T, Yamaya H, Miyagi S, Sekiguchi S, Kawagishi N, Nose M, Harigae H. Polyarteritis nodosa clinically mimicking nonocclusive mesenteric ischemia. World J Gastroenterol. 2013 Jun 21;19(23):3693-8. doi: 10.3748/wjg.v19.i23.3693. PMID: 23801874; PMCID: PMC3691024. DISCLOSURES: No relevant relationships by Rhiannon Holden No relevant relationships by Alexandra Vatzakas" @default.
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• W4387248877 title "RARE PRESENTATION OF VASCULITIS: ACUTE ISCHEMIC BOWEL" @default.
• W4387248877 doi "https://doi.org/10.1016/j.chest.2023.07.1338" @default.
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