Session Information
Title: Systemic Lupus Erythematosus - Clinical Aspects and Treatment: Treatment and Management Studies
Session Type: Abstract Submissions (ACR)
Background/Purpose: Intestinal pseudo-obstruction (IPO) is a rare clinical syndrome characterized by ineffective intestinal motility. IPO has been recently recognized as an uncommon complication of systemic lupus erythematosus (SLE). Though case series of patients with SLE-related IPO (SLE-IPO) have been reported, the epidemiology, characteristics, risk factors and prognosis for SLE-related IPO remain poorly understood.
Methods: To analyze the clinical characteristics and outcome of SLE-IPO, we retrospectively enrolled 68 SLE patients with IPO syndrome as the case group and 323 randomly matched SLE patients without any gastrointestinal manifestations as controls out of 3937 inpatients at Peking Union Medical College Hospital (PUMCH) from 2003 to 2013. IPO was diagnosed according to gastrointestinal symptoms, gaseous small bowel distension with air-fluid levels showed by radiographic signs or thickened gastric wall and dilated small or large bowels showed by CT scan, otherwise, patients were excluded. The case-control study was conducted to compare the clinical and laboratory data. The outcome of SLE-IPO was also investigated.
Results: Within the last 10 years at PUMCH, the prevalence of IPO in SLE patients was 1.73% and the in-hospital fatality rate was 8.8%. 58.8% of the SLE-IPO manifested as the initial presentation of SLE. Ureterohydronephrosis was the most common complication (60.3%) in SLE patients with IPO and the incidence of biliary tract dilation was 7.9%. SLE patients with IPO were always diagnosed at earlier stage of SLE with higher frequency of hematological disturbance, polyserositis and hypocomplementemia than control patients. Ureterohydronephrosis (OR = 90.322, 95% CI 21.283–383.32, p = 0.000), hypocomplementemia (OR = 10.437, 95% CI 1.341–81.217, p = 0.025) and elevated CRP level in serum (OR = 5.143, 95% CI 1.401–18.876, p = 0.014) were independent risk factors for IPO in SLE disease. However, the positivity of anti-dsDNA antibody was a protective factor for SLE with IPO (OR = 0.222, 95% CI 0.061–0.8, p = 0.021). SLE-IPO patients with long IPO duration, diagnosed at late stage of SLE or concurrent with megacholedochus and ureterohydronephrosis always had unfavorable outcome.
Conclusion: IPO can manifest as a complication of SLE and more commonly, as the initial presentation. SLE-IPO always occurs concomitantly with ureterohydronephrosis and biliary dilatation, and these three complications combined indicate a severe situation of SLE. SLE-IPO patients without systemic smooth muscular involvement could achieve better prognosis with early diagnosis and aggressive treatment.
Table 1 A comparison of demographics, clinical manifestations
|
Variable |
Cases |
Control |
p value |
|
Demographics |
|
|
|
|
Female sex n,(%) |
66/68(97.1) |
221/232(95.3) |
0.739 |
|
Age, years, mean±SE |
32.3±1.4 |
32.3±0.83 |
0.975 |
|
Clinical manifestations |
|
|
|
|
Flare age, years, mean±SE |
29.7±1.4 |
32.4±2.7 |
0.594 |
|
Disease duration,months, mean±SE |
30.8±5.5 |
56.2±4.7 |
0.001 |
|
Fatality, n(%) |
6/68(8.8) |
13/232(5.6) |
0.398 |
|
Acute or subacute skin lupus |
23/66(34.8) |
85/232(36.6) |
0.79 |
|
Mucosal Ulcers |
15/67(22.4) |
40/232(17.2) |
0.338 |
|
Arthritis |
29/68(42.6) |
95/232(40.9) |
0.802 |
|
Polyserositis |
54/66(81.8) |
93/232(40.1) |
0.0001 |
|
Nephropathy |
53/68(77.9) |
154/232(66.4) |
0.064 |
|
Nervous system disturbance, n(%) |
12/68(17.6) |
58/224(25.0) |
0.207 |
|
Hematological disturbance, n(%) |
41/67(61.2) |
99/232(42.7) |
0.007 |
|
Urinary system disturbance |
41/68(60.3) |
10/232(4.3) |
0.0001 |
|
Elevated ESR, n(%) |
41/60(68.3) |
155/226(68.6) |
0.97 |
|
Elevated CRP, n(%) |
32/60(53.3) |
63/202(29.9) |
0.001 |
|
Hypocomplementemia, n(%) |
61/68(89.7) |
146/219(66.7) |
0.0001 |
|
ANA, n(%) |
63/68(92.6) |
183/232(78.9) |
0.009 |
|
Anti-dsDNAantibody, n(%) |
21/68(30.9) |
113/224(48.7) |
0.009 |
|
Anti-Smantibody, n(%) |
20/61(32.8) |
56/230(24.3) |
0.182 |
|
Anti-RNP antibody, n(%) |
27/67(40.3) |
81/230(35.2) |
0.45 |
|
Anti-SSA antibody, n(%) |
38/68(55.9) |
124/230(53.9) |
0.775 |
|
Anti-SSB antibody, n(%) |
14/68(20.6) |
27/220(11.7) |
0.063 |
|
Ro-52, n (%) |
9/67(13.4) |
– |
– |
|
Anti-rRNPantibody, n(%) |
6/68(8.8) |
33/180(18.3) |
0.066 |
|
ANCA |
1/60(1.7) |
3/136(2.2) |
1 |
|
ACL antibody, n(%) |
7/59(11.9) |
31/196(15.8) |
0.455 |
|
LA antibody, n(%) |
5/45(11.1) |
21/171(12.3) |
0.843 |
|
SLEDAI, mean±SE |
13.15±0.827 |
10.23±0.40 |
0.001 |
Disclosure:
L. Zhang,
None;
M. Li,
None;
D. Xu,
None;
N. Gao,
None;
L. Zhang,
None;
Y. Hou,
None;
Q. Wang,
None;
X. Zeng,
None.
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