A therapeutic dilemma on lupus enteritis with bowel ischemia presenting as small intestinal obstruction in a Filipino female

A Case Report

Abstract

Significance: Lupus enteritis is rare and life-threatening. Lupus enteritis, as the main manifestation of SLE, is underreported in the Philippines. To our knowledge, this study is the first local report of this case. A high index of suspicion and immediate treatment are necessary due to its high mortality rate and poor prognosis.

Clinical Presentation: A 57-year-old Filipino female from Leyte, Philippines, with a history of recurrent pregnancy loss, chronic left leg swelling, and significant family history of thrombosis presented with a one-month history of diffuse, crampy, intermittent, and progressive abdominal pain, graded 3/10, non-radiating, aggravated by food intake, and associated with bloatedness and vomiting. The severity of abdominal pain did not correlate with the abdominal physical examination. Workups showed thrombocytopenia, positive antinuclear antibody (ANA), low complement component 3 (C3), borderline positive anti-double-stranded deoxyribonucleic acid (anti-dsDNA) antibody, and negative anti-Smith antibody. Whole abdomen computed tomography (CT) scan with triple contrast revealed distal jejunum wall thickening. CT angiography of the abdominal aorta showed portal vein thrombosis. Venous duplex study revealed left leg deep-venous thrombosis (DVT). Antiphospholipid syndrome (APS) was highly suspected despite having negative lupus anticoagulant diluted Russell viper venom time (dRVVT), anticardiolipin antibodies (ACA) IgG and IgM, and beta-2 glycoprotein I (β2GPI) antibodies IgG and IgM. The patient was advised to repeat dRVVT, ACA, β2GPI antibodies, and kaolin clotting time (KCT) after 12 weeks, for further evaluation. She was diagnosed with partial small intestinal obstruction secondary to lupus enteritis, bowel ischemia, and portal vein thrombosis, with probable APS.

Management: Worsening symptoms of intestinal obstruction prompted exploratory laparotomy, segmental resection, and anastomosis of the jejunum under general endotracheal anesthesia. Lupus flare was postoperatively confirmed. Hydrocortisone was started despite the risks of anastomotic failure. Mycophenolate mofetil, hydroxychloroquine, and trimethoprim-sulfamethoxazole were added. After the platelet counts stabilized, fondaparinux was started and later overlapped with warfarin. The patient was discharged, improved and stable. Due to the distance of her province and the limitations imposed by the pandemic, most of the patient’s follow-up was conducted online. On follow-up one month after discharge, the patient remained well and stable. Workups were unremarkable. The patient was unable to undergo the follow-up tests for APS (dRVVT, ACA, β2GPI antibodies, and KCT) due to their limited availability in her province. Warfarin, hydroxychloroquine, mycophenolate mofetil, atorvastatin, ezetimibe, fish oil, and linagliptin were continued. Prednisone was tapered until discontinued.

Recommendations: Management of lupus enteritis involves a multidisciplinary and individualized approach. A comprehensive history and physical examination, along with careful assessment of an intervention’s risks and benefits, are crucial.

Keywords: Antiphospholipid syndrome, Case report, Lupus enteritis, Mesenteric ischemia, Philippines, Small intestinal obstruction, Systemic lupus erythematosus