Case Report
Copyright ©The Author(s) 2018. Published by Baishideng Publishing Group Inc. All rights reserved.
World J Clin Cases. Nov 6, 2018; 6(13): 688-693
Published online Nov 6, 2018. doi: 10.12998/wjcc.v6.i13.688
Systemic lupus erythematosus complicated by noncirrhotic portal hypertension: A case report and review of literature
Qi-Bin Yang, Yong-Long He, Chun-Mei Peng, Yu-Feng Qing, Qi He, Jing-Guo Zhou
Qi-Bin Yang, Yong-Long He, Chun-Mei Peng, Yu-Feng Qing, Qi He, Department of Rheumatology and Immunology, Affiliated Hospital of North Sichuan Medical College, Nanchong 637000, Sichuan Province, China
Yong-Long He, Clinical Medical School, Chengdu University of Traditional Chinese Medicine, Chengdu 610000, Sichuan Province, China
Jing-Guo Zhou, Department of Rheumatology and Immunology, The First Affiliated Hospital of Chengdu Medical College, Chengdu 610000, Sichuan Province, China
Author contributions: Yang QB, He YL, Peng CM, Qing YF, He Q and Zhou JG contributed to the manuscript writing and revision.
Supported by the National Natural Science Foundation of China, No. 81670801; Medical Association of Sichuan Province, No. S16027; Health and Family Planning Commission of Sichuan Province, No. 17PJ059; and Science and Technology Department of Sichuan Province, No. 2018JY0498.
Informed consent statement: Written informed consent was obtained from the patient.
Conflict-of-interest statement: The authors declare no conflict of interest.
Open-Access: This article is an open-access article which was selected by an in-house editor and fully peer-reviewed by external reviewers. It is distributed in accordance with the Creative Commons Attribution Non Commercial (CC BY-NC 4.0) license, which permits others to distribute, remix, adapt, build upon this work non-commercially, and license their derivative works on different terms, provided the original work is properly cited and the use is non-commercial. See: http://creativecommons.org/licenses/by-nc/4.0/
Correspondence to: Jing-Guo Zhou, MD, Professor, Department of Rheumatology and Immunology, The First Affiliated Hospital of Chengdu Medical College, Xindu District, Chengdu 610000, Sichuan Province, China. jgzhou@cmc.edu.cn
Telephone: +86-139-9088-0518
Received: July 11, 2018
Peer-review started: July 11, 2018
First decision: August 3, 2018
Revised: August 7, 2018
Accepted: October 9, 2018
Article in press: October 9, 2018
Published online: November 6, 2018
Processing time: 118 Days and 7 Hours
Abstract

A 48 year-old Chinese woman suffering from polyarthritis, irregular fever and trichomadesis was admitted to the hospital. A diagnosis of systemic lupus erythematosus (SLE) was made based on polyarthritis, pancytopenia, reduced complement 3, multiple positive autoantibodies, a positive Coomb’s test and protein in her urine. In addition, splenomegaly was detected during physical examination and confirmed by abdominal ultrasonography and magnetic resonance imaging, indicating that the patient had SLE and portal hypertension. Further negative investigations ruled out the possibility of cirrhosis. The patient was diagnosed with active SLE complicated by noncirrhotic portal hypertension (NCPH) without liver histopathology, due to the patient’s refusal for liver biopsy. Portal vein diameter and splenomegaly decreased following treatment with methylprednisolone, hydroxychloroquine and metoprolol tartrate. To date, SLE complicated by NCPH has rarely been reported, as it is under-recognized clinically as well as pathologically. Here we describe a case of SLE complicated by NCPH and review the literature for its characteristics, which may contribute to improving the recognition of NCPH and reducing missed and delayed diagnosis of this disorder.

Keywords: Noncirrhotic; Portal hypertension; Systemic lupus erythematosus; Clinical presentation; Diagnosis

Core tip: It is rare when systemic lupus erythematosus (SLE) complicated by noncirrhotic portal hypertension (NCPH) is reported, likely because it is under-recognized clinically as well as pathologically. NCPH should be considered in any patient with SLE who suffers from clinical manifestations of portal hypertension in the absence of cirrhosis evidence. Magnetic resonance imaging could be one of several non-invasive detection methods used to rule out cirrhosis. The recognition of clinical presentation and associated risk factors of NCPH contributes to the reduction of its missed and delayed clinical diagnoses.