Clinical characteristics and prognosis of concomitant systemic lupus erythematosus and primary biliary cholangitis
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ORIGINAL ARTICLE
Clinical characteristics and prognosis of concomitant systemic lupus erythematosus and primary biliary cholangitis Cheng Cheng 1 & Ziqian Wang 1 & Li Wang 1 & Jiuliang Zhao 1 & Qian Wang 1 & Xinping Tian 1 & Mengtao Li 1 Xiaofeng Zeng 1
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Received: 22 July 2020 / Revised: 21 September 2020 / Accepted: 8 October 2020 # International League of Associations for Rheumatology (ILAR) 2020
Abstract Objective This study aimed to identify the clinical characteristics of systemic lupus erythematosus-primary biliary cholangitis (SLE-PBC) patients and to compare the manifestations and prognosis with systemic lupus erythematosus (SLE) patients. Methods Twenty-one inpatients with concomitant SLE and primary biliary cholangitis (PBC) were identified in our hospital. Baseline clinical manifestations, laboratory results, disease activity, and organ damage, as well as changes in disease manifestations and therapies, were retrospectively analyzed. Baseline clinical characteristics, survival rate, and flare-ups were compared with 254 SLE patients also from our hospital. Results The prevalence of concomitant PBC in SLE inpatients was 0.27%. Over half of the patients were diagnosed with SLE and PBC simultaneously. Compared with SLE patients, SLE-PBC patients started the symptom of SLE at an older age, with a longer delay before the diagnosis of SLE (P < 0.05). Hematological and muscular involvement, pulmonary arterial hypertension, and interstitial lung disease were more common in SLE-PBC patients (P < 0.05). Kaplan-Meier estimate showed a significantly lower survival rate in SLE-PBC group, with 3-year survival rate at 88.4%. Conclusion Concomitant PBC might have a negative impact on the survival of SLE, with older age at SLE onset, longer delay before SLE diagnosis, and higher baseline damage. More intensive therapy and prevention of hepatic toxicity need to be considered. Keywords Clinical manifestation . Primary biliary cholangitis (PBC) . Survival . Systemic lupus erythematosus (SLE) Key Points • Hematological and muscular involvement, PAH, and ILD were more common in SLE PBC than in SLE. • The study firstly reported the survival rate of SLE PBC patients. • More intensive therapy and prevention of hepatic toxicity are needed for SLE-PBC.
Introduction Systemic lupus erythematosus (SLE) is a multi-system autoimmune disease, with various factors contributing to its Cheng Cheng and Ziqian Wang contributed equally to this work. * Mengtao Li [email protected] 1
Department of Rheumatology, Peking Union Medical College Hospital, Peking Union Medical College & Chinese Academy of Medical Sciences, National Clinical Research Center for Dermatologic and Immunologic Diseases, Ministry of Science & Technology, Key Laboratory of Rheumatology and Clinical Immunology, Ministry of Education, Beijing 100730, China
pathogenesis [1]. Primary biliary cholangitis (PBC) is also an autoimmune disease characterized by destruction of intrahepatic small bile ducts, mostly progressing to liver cirrhosis if not controlled [2]. The c
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