Chiou, Yee-Hsuan

Feng, Wen-Bin

Huang, I-Fei

Wang, Ying-Shu

Elsevier - Publisher Connector 

Kaohsiung Journal of Medical Sciences (2015) 31, 440e441Available online at www.sciencedirect.com
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journal homepage: http: / /www.kjms-onl ine.comLETTER TO THE EDITORRecurrent lupus mesenteric vasculitis leading
to gastrointestinal perforation and sepsisTo the Editor,
Systemic lupus erythematosus (SLE) is an autoimmune dis-
ease affecting multiple systems. Gastrointestinal (GI)
manifestations are not included in the diagnostic criteria of
SLE, but frequently affect SLE patients. There are various
GI manifestations, including anorexia, nausea, and vomit-
ing. The etiologies vary, such as lupus mesenteric vasculitis
(LMV) and serositis secondary to lupus itself, side effects of
SLE medication, or coincidence of GI diseases like appen-
dicitis, pancreatitis, or acute viral or bacterial enteroco-
litis. Diagnosing the cause of GI symptoms in patients with
SLE is a challenge for clinicians. However, LMV should be
considered if all other etiologies have been excluded. Here
is a case of a patient with SLE presenting as recurrent
abdominal pain and with poor prognosis.
A 15-year-old girl had a history of SLE with class IV lupus
nephritis and lupus nephritis-related secondary hyperten-
sion. On the first admission, she had abdominal pain for 2
weeks with fever, tarry stool, and vomiting for 4e5 days.
Physical examination showed diffuse tenderness over the
abdominal wall, without rebound tenderness or muscle
guarding. Abdominal computed tomography (CT) revealed
segmental mural thickening of the proximal jejunum. Acute
segmental obstructive enteritis (ASOE), mesenteric vascu-
litis related, was highly suspected. As an SLE flare-up was
favored, intravenous methylprednisolone (500 mg/d) was
administered for 3 days. She was discharged in a stable
condition, with oral prednisolone (1 mg/kg/d) as a main-
tenance treatment. However, she was re-admitted after 3
days with similar conditions, which were resolved with
intravenous steroids.
Unfortunately, the patient had abdominal pain with
vomiting, and watery diarrhea with blood-tinged stool 4
months later. Laboratory examination revealed a white cell
count of 8180/uL (segment/lymphocyte: 97%/1%), platelet
73,000/mm3, C-reactive protein 3.2 mg/dL, amylase/lipaseConflicts of interest: All authors declare no conflicts of interest.
http://dx.doi.org/10.1016/j.kjms.2015.04.007
1607-551X/Copyright ª 2015, Kaohsiung Medical University. Published65/206 U/L, and SGOT/SGPT 53/45 U/L. Intravenous
methylprednisolone (500 mg/d) was administered for 3
days, but the abdominal pain recurred. Abdominal X-ray of
the kidneys, ureter, and bladder (KUB) demonstrated small
intestine obstruction with proximal dilatation. Emergency
abdominal CT (Fig. 1) revealed free air and fluid collection
in the peritoneal cavity. Hollow organ perforation was
highly suspected. There was also segmental wall thickening
of the small bowel loop, as well as splenic infarction.
A pediatric surgeon immediately performed an explor-
atory laparotomy. Jejunal perforation was noted, so
segmental resection of the jejunum with end-to-end anas-
tomosis was performed. Histopathology report of the
resected jejunum showed a picture of vasculitis of the
small arteries, with fibrinoid necrosis of the vessel wall.
Ulcer with perforation and acute inflammatory exudate in
the serosal layer were observed. A broad spectrum antibi-
otic was administered due to the suspicion of sepsis. An
antifungal agent was also administered because an ascites
culture showed Candida albicans.
However, the abdominal pain recurred after the opera-
tion. Abdominal CT revealed focal abscess collection with
peritonitis over the left abdomen. Surgical intervention was
scheduled, but the patient had septic shock with con-
sciousness change the next morning. Her consciousness
remained confused even after drainage of the abscess.
Brain CT revealed symmetric calcification over the bilateral
basal ganglion, probably chronic SLE-related rather than
acute change. The patient was intubated due to declining
respiratory condition, but her condition worsened with
multiple organ failure until she finally died.
A case report from Taiwan in 2011 involved a patient
with severe, recurrent lupus enteritis and severe hypo-
complementemia as the initial and only presentation of
SLE. The patient died of sepsis [1]. This patient did not
suffer from bowel perforation, but had total intestinal
obstruction with obstructive jaundice resulting from
obstruction of the ampulla of Vater, all of which were
suspected to be secondary to severe duodenal wall swellingby Elsevier Taiwan LLC. All rights reserved.
Figure 1. Abdominal computed tomography of the patient, showing free air and fluid collection in the peritoneal cavity, and
hollow organ perforation was suspected.
Letter to the Editor 441and edema. This case and the present case report both had
recurrent lupus enteritis that caused the poor prognosis. It
remains unclear as to what risk factor of mesenteric
vasculitis was related to poor prognosis.
There are several reports on recurrent lupus mesenteric
enteritis. Kwok et al. [2] mentioned that SLE patients with
pre-existing antiphospholipid antibody syndrome have a
tendency for more frequent recurrences. Kim et al. [3]
noted that the cumulative dose of prednisolone and the
duration of prednisolone treatment are significantly higher
in patients with nonrecurrent lupus enteritis than in those
with recurrent lupus enteritis. The wall thickness of the
most thickened bowel in patients with recurrent lupus en-
teritis is greater than that in patients with nonrecurrent
lupus enteritis, although the difference is not significant
[3]. Lian et al. [4] have found that patients treated with
cyclophosphamide have less recurrent GI diseases.
The present case report had recurrent LMV for three
times within half a year, despite antiphospholipid antibody
titers within normal limits. She received steroid control for
6 years and intermittent intravenous cyclophosphamide
treatment nine times, but she had poor medication
compliance with only intermittent outpatient department
visits. Thus, despite immunosuppressants and normal anti-
phospholipid antibody titers, poor drug adherence with
lower cumulative oral prednisolone, and shorter treatment
duration may have led to recurrent LMV. The report from
Kim et al. [3] has 16 patients, including seven with recur-
rent LMV, but without any mortality. More clinical experi-
ence is needed to investigate risk factors of recurrent LMV
and gastrointestinal perforation.
References
[1] Lin HP, Wang YM, Huo AP. Severe, recurrent lupus enteritis as
the initial and only presentation of systemic lupuserythematosus in a middle-aged woman. J Microbiol Immunol
Infect 2011;44:152e5.
[2] Kwok SK, Seo SH, Ju JH, Park KS, Yoon CH, Kim WU. Lupus
enteritis: clinical characteristic, risk factor for relapse and
association with anti-endothelial cell antibody. Lupus 2007;16:
803e9.
[3] Kim YG, Ha HK, Nah SS, Lee CK, Moon HB, Yoo B. Acute
abdominal pain in systemic lupus erythematosus: factors
contributing to recurrence of lupus enteritis. Ann Rheum Dis
2006;65:1537e8.
[4] Lian TY, Edwards CJ, Chan SP, Chng HH. Reversible acute
gastrointestinal syndrome associated with active systemic
lupus erythematosus in patients admitted to hospital. Lupus
2003;12:612e6.
Ying-Shu Wang
Department of Pediatrics, Kaohsiung Veterans General
Hospital, Kaohsiung, Taiwan
I-Fei Huang
Department of Pediatrics, Kaohsiung Veterans General
Hospital, Kaohsiung, Taiwan
School of Medicine, National Yang-Ming University, Taipei,
Taiwan
Wen-Bin Feng
Yee-Hsuan Chiou*
Department of Pediatrics, Kaohsiung Veterans General
Hospital, Kaohsiung, Taiwan
*Corresponding author. Department of Pediatrics,
Kaohsiung Veterans General Hospital, Number 386, Dazhong
1st Road, Zuoying District, Kaohsiung City 813, Taiwan,
ROC.
E-mail address: yhchiou@vghks.gov.tw (Y.-H. Chiou)


Recurrent lupus mesenteric vasculitis leading to gastrointestinal perforation and sepsis 

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Recurrent lupus mesenteric vasculitis leading to gastrointestinal perforation and sepsis

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