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
ARTICLE HIGHLIGHTS
Case characteristics

A 48 year-old Chinese woman with polyarthritis, irregular fever and trichomadesis was also found to have splenomegaly during physical examination.

Clinical diagnosis

The diagnosis of noncirrhotic portal hypertension (NCPH) was made in the absence of cirrhosis and was associated with systemic lupus erythematosus (SLE), which was diagnosed based on polyarthritis, pancytopenia, reduced complement 3, multiple positive autoantibodies, a positive Coomb’s test and protein in urine.

Differential diagnosis

Portal hypertension etiology, such as cirrhosis and other obstructive diseases, were considered.

Laboratory diagnosis

Reduced complement 3, multiple positive autoantibodies, positive Coomb’s test and protein in urine.

Imaging diagnosis

Abdominal ultrasonography (USG) and magnetic resonance imaging (MRI) demonstrated splenomegaly and portal hypertension.

Treatment

The patient was treated with methylprednisolone, hydroxychloroquine and metoprolol tartrate to reduce SLE disease activity, control portal hypertension and its complications. In severe conditions, such as variceal bleeding, endoscopic variceal ligation or transjugular intrahepatic portosystemic shunting is recommended.

Related reports

NCPH is commonly called nodular regenerative hyperplasia or idiopathic portal hypertension in previous reports due to the lack of standardized nomenclature and diagnostic criteria. Most reported cases of NCPH associated with SLE had severe complications in late stages. In our patient, who was in the early stage of NCPH, splenomegaly and portal hypertension were identified upon physical examination and diagnostic imaging. MRI, which is more sensitive than CT scan or USG, is an alternative non-invasive diagnostic technique that can both reveal signs of portal hypertension as well as differentiate features commonly seen in cirrhosis.

Experiences and lessons

NCPH, which is characterized by portal hypertension, splenomegaly and hypersplenism, should be considered in SLE patients with clinical manifestations of portal hypertension in the absence of cirrhosis. MRI can be performed to rule out cirrhosis. The recognition of early clinical presentation and the associated risk factors of NCPH can contribute to a reduction in missed and delayed diagnosis of this disorder.