Case Report
Copyright ©The Author(s) 2019. Published by Baishideng Publishing Group Inc. All rights reserved.
World J Clin Cases. Jun 6, 2019; 7(11): 1315-1322
Published online Jun 6, 2019. doi: 10.12998/wjcc.v7.i11.1315
Lupus enteritis as the only active manifestation of systemic lupus erythematosus: A case report
Adalberto Gonzalez, Vaibhav Wadhwa, Fayssa Salomon, Jeevna Kaur, Fernando J Castro
Adalberto Gonzalez, Fayssa Salomon, Jeevna Kaur, Department of Internal Medicine, Cleveland Clinic Florida, Weston, FL 33331, United States
Vaibhav Wadhwa, Fernando J Castro, Department of Gastroenterology and Hepatology, Cleveland Clinic Florida, Weston, FL 33331, United States
Author contributions: Gonzalez A and Salomon F were the patient’s Internal Medicine physicians and contributed to manuscript drafting. Wadhwa V was one of the patient’s gastroenterologists, reviewed the literature, and contributed to manuscript writing. Kaur J reviewed the literature and contributed to manuscript writing. Castro FJ was the patient’s gastroenterologist and is responsible for the revision of the manuscript. All authors issued final approval for the version to be submitted.
Informed consent statement: Informed written consent was obtained from the patient for publication of this report and any accompanying images.
Conflict-of-interest statement: The authors declare that they have no conflict of interest.
CARE Checklist (2016) statement: The authors have read the CARE Checklist (2016), and the manuscript was prepared and revised according to the CARE Checklist (2016).
Open-Access: This article is an open-access article which was selected by an in-house editor and fully peer-reviewed by external reviewers. It is distributed in accordance with the Creative Commons Attribution Non Commercial (CC BY-NC 4.0) license, which permits others to distribute, remix, adapt, build upon this work non-commercially, and license their derivative works on different terms, provided the original work is properly cited and the use is non-commercial. See: http://creativecommons.org/licenses/by-nc/4.0/
Corresponding author: Fernando Castro, MD, AGAF, Staff Physician, Department of Gastroenterology and Hepatology, Cleveland Clinic Florida, 2950 Cleveland Clinic Boulevard, Weston, FL 33331, United States. castrof@ccf.org
Telephone: +1-954-6895646
Received: February 12, 2019
Peer-review started: February 15, 2019
First decision: March 14, 2019
Revised: March 27, 2019
Accepted: April 18, 2019
Article in press: April 19, 2019
Published online: June 6, 2019
Processing time: 114 Days and 14.3 Hours
Abstract
BACKGROUND

Lupus enteritis is a rare manifestation of systemic lupus erythematosus (SLE). Diagnosis of this condition is difficult, especially in the absence of other symptoms related to active SLE. We present the case of a 25-year-old female with lupus enteritis as the sole initial manifestation of active SLE.

CASE SUMMARY

A 25-year-old African American female presented to the Emergency Department complaining of diffuse abdominal pain, diarrhea, nausea, and vomiting for 2 days. Her past medical history was significant for seasonal allergies and family history was pertinent for discoid lupus in her father and SLE in a cousin. The patient’s vital signs on presentation were normal. Her physical exam was remarkable for significant lower abdominal tenderness without guarding or rigidity. A computed tomography of the abdomen and pelvis revealed marked circumferential wall thickening and edema of the proximal and mid small bowel predominantly involving the submucosa. Our main differential diagnoses were intestinal angioedema and mesenteric vein thrombosis. However, mesenteric vessels were patent, and laboratory testing for hereditary angioedema showed a normal C1 Esterase Inhibitor level and low C3 and C4 levels. Infectious work-up was negative. Autoimmune tests showed elevated anti-nuclear antibodies (ANA) (13.6), anti-Smith antibody, and anti-ribonucleoprotein (anti-RNP) antibody. The patient was diagnosed with SLE enteritis. She was maintained on bowel rest, given intravenous hydration, and started on methylprednisolone 60 mg IV daily. She had significant improvement in her abdominal pain, diarrhea, and emesis after 2 days of treatment. Steroids were tapered and maintained on Hydroxychloroquine with no relapses one year after presentation.

CONCLUSION

This case of lupus enteritis represents a rare manifestation of SLE. Diagnosis requires clinical suspicion, characteristic imaging and laboratory tests. Endoscopic appearance and biopsies usually yield non-specific findings. High dose steroids are the preferred treatment modality for moderate and severe cases.

Keywords: Lupus enteritis, Systemic lupus erythematous, Abdominal pain, Hereditary angioedema, Case report

Core tip: Lupus enteritis is a rare manifestation of systemic lupus erythematosus (SLE). It is a difficult diagnosis, especially in the absence of other symptoms related to active SLE. We present the case of a 25-year-old female with lupus enteritis as the sole initial manifestation of active SLE. The diagnosis can be made with history, physical exam, laboratory testing, and imaging. Endoscopy is not required nor recommended to make the diagnosis. Treatment depends on the severity. In this patient with moderate severity lupus enteritis, high dose steroids were an efficient initial treatment. Hydroxychloroquine was used to maintain remission.