Lingling Zhang1, Dong Xu1, Hong Yang1, Xinping Tian1, Qian Wang1, Yong Hou1, Na Gao1, Li Zhang1, Mengtao Li1, Xiaofeng Zeng2. 1. From the Department of Rheumatology and Clinical Immunology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Key Laboratory of Rheumatology and Clinical Immunology, Ministry of Education; Department of Gastroenterology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, China.L. Zhang, MD, PhD, Department of Rheumatology and Clinical Immunology, Peking Union Medical College Hospital; D. Xu, MD, Department of Rheumatology and Clinical Immunology, Peking Union Medical College Hospital; X. Tian, MD, Department of Rheumatology and Clinical Immunology, Peking Union Medical College Hospital; Q. Wang, MD, Department of Rheumatology and Clinical Immunology, Peking Union Medical College Hospital; Y. Hou, MD, Department of Rheumatology and Clinical Immunology, Peking Union Medical College Hospital; N. Gao, MD, PhD, Department of Rheumatology and Clinical Immunology, Peking Union Medical College Hospital; L. Zhang, MD, Department of Rheumatology and Clinical Immunology, Peking Union Medical College Hospital; M. Li, MD, Department of Rheumatology and Clinical Immunology, Peking Union Medical College Hospital; X. Zeng, MD, Department of Rheumatology and Clinical Immunology, Peking Union Medical College Hospital; H. Yang, MD, Department of Gastroenterology, Peking Union Medical College Hospital. 2. From the Department of Rheumatology and Clinical Immunology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Key Laboratory of Rheumatology and Clinical Immunology, Ministry of Education; Department of Gastroenterology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, China.L. Zhang, MD, PhD, Department of Rheumatology and Clinical Immunology, Peking Union Medical College Hospital; D. Xu, MD, Department of Rheumatology and Clinical Immunology, Peking Union Medical College Hospital; X. Tian, MD, Department of Rheumatology and Clinical Immunology, Peking Union Medical College Hospital; Q. Wang, MD, Department of Rheumatology and Clinical Immunology, Peking Union Medical College Hospital; Y. Hou, MD, Department of Rheumatology and Clinical Immunology, Peking Union Medical College Hospital; N. Gao, MD, PhD, Department of Rheumatology and Clinical Immunology, Peking Union Medical College Hospital; L. Zhang, MD, Department of Rheumatology and Clinical Immunology, Peking Union Medical College Hospital; M. Li, MD, Department of Rheumatology and Clinical Immunology, Peking Union Medical College Hospital; X. Zeng, MD, Department of Rheumatology and Clinical Immunology, Peking Union Medical College Hospital; H. Yang, MD, Department of Gastroenterology, Peking Union Medical College Hospital. zengxfpumc@gmail.com mengtao.li@cstar.org.cn.
Abstract
OBJECTIVE: To analyze the epidemiology, clinical characteristics, and risk factors for systemic lupus erythematosus-related intestinal pseudo-obstruction (SLE-IPO). METHODS: We retrospectively examined 85 patients with SLE with IPO as the case group and 255 randomly matched patients with SLE without any gastrointestinal manifestations as the control group, out of 4331 inpatients at the Peking Union Medical College Hospital (PUMCH) from 2003 to 2014. RESULTS: Over the last 11 years at PUMCH, the prevalence of IPO in patients with SLE was 1.96% and the in-hospital fatality rate was 7.1%. Of these patients, 57.6% presented with IPO as the initial affected system of SLE, and the rate of misdiagnosis was about 78%. Pyeloureterectasis was the most common complication (58.9%) in patients with SLE-IPO and the incidence of biliary tract dilation was 7.1%. Patients with SLE with IPO were always diagnosed at an earlier stage of SLE with a higher frequency of hematological disturbance, polyserositis, and hypocomplementemia. Pyeloureterectasis, hypocomplementemia, and elevated C-reactive protein levels in serum were independent risk factors for IPO in SLE disease. Patients with SLE-IPO with long IPO duration and those diagnosed during late stages of SLE or concurrent with pyeloureterectasis and megacholedochus always had an unfavorable outcome. CONCLUSION: IPO is a rare complication, but commonly presents as the initial affected system of SLE, which can lead to a difficult diagnosis and delayed treatment. SLE-IPO occurrence concomitantly with pyeloureterectasis and megacholedochus showed a severe clinical situation in our cohort. Thus, patients with SLE-IPO with systemic smooth muscular involvement should be diagnosed early and treated aggressively.
OBJECTIVE: To analyze the epidemiology, clinical characteristics, and risk factors for systemic lupus erythematosus-related intestinal pseudo-obstruction (SLE-IPO). METHODS: We retrospectively examined 85 patients with SLE with IPO as the case group and 255 randomly matched patients with SLE without any gastrointestinal manifestations as the control group, out of 4331 inpatients at the Peking Union Medical College Hospital (PUMCH) from 2003 to 2014. RESULTS: Over the last 11 years at PUMCH, the prevalence of IPO in patients with SLE was 1.96% and the in-hospital fatality rate was 7.1%. Of these patients, 57.6% presented with IPO as the initial affected system of SLE, and the rate of misdiagnosis was about 78%. Pyeloureterectasis was the most common complication (58.9%) in patients with SLE-IPO and the incidence of biliary tract dilation was 7.1%. Patients with SLE with IPO were always diagnosed at an earlier stage of SLE with a higher frequency of hematological disturbance, polyserositis, and hypocomplementemia. Pyeloureterectasis, hypocomplementemia, and elevated C-reactive protein levels in serum were independent risk factors for IPO in SLE disease. Patients with SLE-IPO with long IPO duration and those diagnosed during late stages of SLE or concurrent with pyeloureterectasis and megacholedochus always had an unfavorable outcome. CONCLUSION:IPO is a rare complication, but commonly presents as the initial affected system of SLE, which can lead to a difficult diagnosis and delayed treatment. SLE-IPO occurrence concomitantly with pyeloureterectasis and megacholedochus showed a severe clinical situation in our cohort. Thus, patients with SLE-IPO with systemic smooth muscular involvement should be diagnosed early and treated aggressively.