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Lupus Enteritis Earlydiagnosis
Lupus Enteritis Earlydiagnosis
Lupus Enteritis Earlydiagnosis
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Luís M1, Brites AL1, Duarte AC2, Teixeira V3, Freitas R2, Oliveira-Ramos F3, Macieira C3, Santos MJ2, Inês L1
results
1. Reumatologia, Centro Hospitalar e Universitário de Coimbra
2. Reumatologia, Hospital Garcia de Orta
3. Reumatologia, Centro Hospitalar Universitário Lisboa Norte Seven cases of LE were identified in the participating cen-
ters. The main characteristics of these patients target sign at the left iliac fossa, dilatation of the small
are summarized in Table I. Each case is presented below. bowel lumen and multiple enlarged mesenteric lymph
nodes. Additionally, the lateral wall of bladder was also
# PAtIent 1 thickened suggesting concomitant involvement of the
An 18-years-old female was admitted in the emergen- urinary tract. She was started on IV MPDN 1g pulses
cy room with acute diffuse abdominal pain, fever, nau- followed by oral prednisone 1 mg/kg/day, with com-
sea, vomiting and absence of stools and gas emissions plete resolution of symptoms within 3 days. At 1-year
over the previous 4 days. She had been diagnosed with follow-up, under no therapy apart from hydroxy-
SLE almost one year earlier based on cutaneous, arti- chloroquine (HCQ), the patient remained free of ab-
cular, hematological and immunological features. At dominal complaints or other signs of disease activity.
admission, her abdomen was mildly tender without
audible bowel sounds. Laboratory tests showed low # PAtIent 3
complement, new onset anemia, slight hypoalbu- A 16-years-old female had been diagnosed with SLE 3
minemia and increased serum ferritin. Erythrocyte years earlier, with cutaneous, articular, neurological,
sedimentation rate (ESR), C-reactive protein (CRP) and hematological, renal and immunological involvement.
lactate dehydrogenase (LDH) were within normal She suffered from late stage chronic kidney disease and
ranges. An abdominal ultrasound (US) revealed mo- secondary hypertension as a result of a previous severe
derate ascites and diffused wall thickening of the colon class IV lupus nephritis. She was admitted to the emer-
with increased reflectivity of subjacent fat tissue. Abdo- gency room with acute onset diffuse abdominal pain,
minal computed tomography (CT) clearly showed in- nausea and vomiting that started less than 24 hours
creased wall thickening with abnormal enhancement before. She was hemodynamically unstable, had a ten-
(target sign) - mainly in the jejunum and ileum but der abdomen and no bowel sounds. Laboratory tests
also in the ascending colon - and engorgement of showed worsening of renal function and hypoalbu-
mesenteric vessels. Multiple enlarged mesenteric minemia. Both ESR and CRP were normal. US revealed
lymph nodes were also present (Figure 1). She was small bowel wall edema, increased vascularization on
started on IV methylprednisolone (MPDN) 500mg Doppler and moderate ascites. She wasn’t submitted to
pulses followed by oral prednisone (1mg/kg/day). abdominal CT since her renal function didn’t allow the
Twenty-four hours later the symptoms quickly started administration of iodate contrast. IV MPDN 1g pulses
to resolve and she was discharged 6 days later. After were immediately started with excellent clinical re-
more than a year, the patient remains free of GI symp- sponse, although she maintained changes in control
toms although soon after the present episode lupus US 3 days later. After hospital discharge, recurrent self-
nephritis developed. She was started on induction -limited abdominal pain episodes persisted. Two years
therapy with mycophenolate mofetil (MMF) 2.5g/d later, she presented again with abdominal pain, this
with good response. time more focused on the right iliac fossa, and diar-
rhea. US confirmed the presence of thickened and hy-
# PAtIent 2 poechogenic ascending colon wall with enhancement
A 43-years-old female, with a 10-year history of SLE - of the surrounding fat tissue and associated bilateral
based on the presence of oligoarthritis, pericarditis, hydronephrosis with bladder wall thickening. CT scan
lymphopenia, photosensitive malar rash and im- showed thickening of small bowel wall, multiple small
munological markers - presented with a two-day his- mesenteric adenopathies, increased number of ob-
tory of diffuse acute abdominal pain, vomiting and dy- served mesenteric vessels and mild ascites. She was
suria. She also described pain and swelling of her left again treated with IV MPDN 1g pulses, with no relapse
ankle for the last two months. Physical examination until today, after 5 years follow-up. Due to progressive
showed a tender abdomen and left ankle arthritis. ESR renal function deterioration, she is currently being
(58 mm/1st hour) and LDH (326 mg/dl) were high considered for kidney transplant.
while CRP was slightly elevated (0.62 mg/dl). Urinary
sediment analysis showed new onset proteinuria # PAtIent 4
(527.5 mg/ 24h). Large ascites and jejunum-ileum wall A 40-years-old female, with a 21-year history of SLE
thickening were seen on US. Abdominal CT scan con- with hematological, renal and immunological in-
firmed the presence of thickened intestinal loops with volvement, had been suffering from recurrent episodes
147
C4 0.06 C4 0.07 C4 0.02 C4 0.03 C4 < 0.1 C4 0.13 C4 0.08
anti-dsDNA 140 anti-dsDNA > 50 anti-dsDNA anti-dsDNA 16 anti-dsDNA >50 anti-dsDNA 1284 anti-dsDNA >379
luis m et al
CRP 0.2 CRP 0.6 > 50 CRP < 0.5 CRP 1.6 CRP 5.1 CRP 0.2 CRP 27
ESR 2 ESR 54 ESR < 15 ESR 8 ESR 100 ESR 23 ESR 31
Abdominal US findings Yes Yes Yes Yes Yes Yes Yes
suggestive of LE
Abdominal CT findings Yes Yes NA Yes Yes Yes NA
suggestive of LE
Flare treatment IV MPDN IV MPDN IV MPDN IV MPDN IV MPDN PDN 1mg/kg/day IV MPDN
C3: C3 complement (normal range: 0.80-1.85 g/L); C4: C4 complement (normal range: 0.15-0.53 g/L); CRP: C-reactive protein (normal range: 0-0.5 mg/dL); CpA: Cyclosporine;
CYC: cyclophosphamide; ESR: erythrocyte sedimentation rate (normal range: 0-20 mm/h); F: female; GI: gastrointestinal; HCQ: hydroxychloroquine; IVIG: intravenous immunoglobulin;
MMF: mycophenolate mofetil; MPDN: methylprednisolone; N: within normal range; NA: not applicable; PDN: prednisolone; SLEDAI: Systemic Lupus Erythematosus Disease Activity
Index; US - ultrasound. F: female.
How to diagnose lupus enteritis early? lessons learned from a multicenter case series
filtrate. She was successfully treated with IV MPDN 1g cites and slight hepatosplenomegaly. It was also evi-
pulses with good response and remains without any dent the presence of bilateral pleural effusion and mini-
signs of abdominal disease activity after more than 20 mal pericardial effusion. Kidney biopsy revealed
years. She is currently treated with HCQ and lefluno- mesangial proliferative glomerulonephritis and throm-
mide for her arthritis. botic microangiopathy. She was immediately started on
IV MPDN 1g pulses for 3 days followed by cyclosporine
# PAtIent 6 100 mg/day. She was discharged from the hospital 10
A previously healthy 36-years-old black female had days later, after total symptom resolution. At one-year
been complaining of inflammatory polyarthralgia for follow-up the patient remains symptom-free, with
the last 4 months, when she started suffering from nau- complete kidney response (proteinuria 0.8 mg/24h).
sea, vomiting, diarrhea and diffuse abdominal pain. At
hospital admission, the patient was dehydrated, pale,
had a tender abdomen and peripheral polyarthritis. In- dIscussIon
fectious etiology was ruled out after an extensive
workup. She tested positive for antinuclear and anti- Many GI conditions can mimic LE. The differential
Smith antibodies. Her anti-dsDNA was high and C3 diagnosis can be rather challenging since some of these
complement fraction reduced. ESR and CRP were nor- conditions, despite being nonspecific for SLE, are more
mal. US showed moderate ascites and diffuse small prevalent in these patients, such as infectious colitis
bowel wall thickening. Enteric MRI found diffuse wall (parasitic, bacterial or viral), peritonitis, acute pancre-
thickening of the jejunum and distal ileum, intralumi- atitis, autoimmune hepatitis, primary biliary cirrhosis,
nal dilatation, congestive mucosal folds, target sign, en- mesenteric thrombosis (more commonly associated
gorgement of mesenteric vessels and increased sup- with the presence of antiphospholipid antibodies) or
pression of mesenteric fat. A subsequent abdominal CT even GI toxicity of drugs.4,6,7
scan revealed similar findings: diffuse small bowel wall Several predisposing factors for LE have been pro-
thickening and intraluminal distention, especially in posed, such as simultaneous peripheral or central ner-
the terminal ileum. There was wall thickening of the vous system vasculitis, thrombocytopenia and serum
descending colon to the splenic angle, without dilata- rheumatoid factor.8 None of them were present in our
tion or obstruction. Moderate ascites was also present. series. All of our patients presented with acute onset
Colon biopsy result was unspecific for vasculitis but abdominal pain, mainly diffuse, with associated nausea
allowed the exclusion of other differential diagnosis and vomiting. Three patients suffered from concomi-
(tuberculosis, CMV colitis and Whipple disease among tant lupus cystitis, characterized by urinary bladder
others). She received prednisone 1 mg/kg/day with no wall edema and thickening and, in one case, hy-
response. Nasogastric intubation and parenteral nutri- dronephrosis. This association was previously de-
tion were required, and treatment with IV MPDN 1g scribed in the literature and was even proposed as risk
pulses was started, followed by oral azathioprine. She factor for recurrence.9,10
fully recovered and remains symptom and relapse-free Patients 5 and 6 had LE as inaugural manifestation
until today, after a 3-year follow-up. of SLE. In both cases, SLE was suggested by the con-
comitant presence of arthritis and positive immuno-
# PAtIent 7 logical markers of the disease (antinuclear and anti-
An 18-year-old female with a recent diagnosis of SLE -Smith antibodies, high anti-dsDNA and low comple-
based on hematological, articular, mucocutaneous and ment), although both ESR and CRP were within their
immunological features, presented with fever, diffuse normal range.
abdominal pain, nausea, vomiting and bloody diarrhea In our case series, all patients but two underwent
in the emergency room. At physical examination, she abdominal CT scan and all of them fulfilled the crite-
had a tender abdomen and severe peripheral edema. ria proposed by Byun for LE.11 The widespread use of
Laboratory tests revealed anemia, increased CRP, low abdominal CT allowed higher diagnosis accuracy, ear-
complement fractions and positive anti-dsDNA. Pro- ly screening for complications, treatment planning and
teinuria was identified for the first time at this point disease follow-up. However, CT findings in LE, despite
(2.4g/ 24h). Abdominal US showed moderate parietal its high sensitivity, carry a low specificity since they can
thickening of the left colon and rectum walls, mild as- be found in other GI conditions, mainly pancreatitis,
mechanical bowel occlusion, peritonitis and inflam- and a reasonable alternative to CT scan in selected pa-
matory bowel disease.5,12 tients.
Patients 5 and 6 were submitted to intestinal biop-
corresPondence to
sy. In patient 5, histological findings were indeed su- Mariana Luís
ggestive of mesenteric vasculitis. In patient 6, on the Serviço de Reumatologia
other hand, the result was inconclusive for vasculitis Centro Hospitalar e Universitário de Coimbra
but allowed the exclusion of other possible conditions, Praceta Mota Pinto
such as CMV colitis, Whipple disease and other gra- 3000-075, Coimbra
E-mail: maryanaluys@gmail.com
nulomatous diseases. Janssens et al. conducted a lite-
rature review and concluded that in the 150 cases al- references
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conclusIon