Case Report
Copyright ©The Author(s) 2022. Published by Baishideng Publishing Group Inc. All rights reserved.
World J Clin Cases. Sep 26, 2022; 10(27): 9851-9858
Published online Sep 26, 2022. doi: 10.12998/wjcc.v10.i27.9851
Pulmonary hypertension secondary to seronegative rheumatoid arthritis overlapping antisynthetase syndrome: A case report
Cheng-Yan Huang, Ming-Jie Lu, Jia-Hua Tian, Dai-Shun Liu, Chun-Yan Wu
Cheng-Yan Huang, Ming-Jie Lu, Jia-Hua Tian, Chun-Yan Wu, Department of Respiratory Medicine, The Third Affiliated Hospital of Zunyi Medical University (The First People’s Hospital of Zunyi), Zunyi 563000, Guizhou Province, China
Dai-Shun Liu, Clinical School, Zunyi Medical University, Zunyi 563000, Guizhou Province, China
Author contributions: Huang CY was responsible for obtaining the patient’s informed consent, collecting patient data, and writing the manuscript; Lu MJ and Tian JH reviewed the literature and contributed to manuscript drafting; Wu CY and Liu DS revised the manuscript; all authors issued final approval for the version to be submitted.
Supported by the Natural Science Foundation of China, No. 82060010.
Informed consent statement: Informed written consent was obtained from the patients for the publication of this report and any accompanying images.
Conflict-of-interest statement: The authors declare that they have no conflict of interest.
CARE Checklist (2016) statement: The authors have read the CARE Checklist (2016), and the manuscript was prepared and revised according to the CARE Checklist (2016).
Open-Access: This article is an open-access article that 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:
Corresponding author: Dai-Shun Liu, MD, Chief Physician, Postdoc, Clinical School, Zunyi Medical University, No. 6 Xuefu West Road, Xinpu New District, Zunyi 563000, Guizhou Province, China.
Received: May 2, 2022
Peer-review started: May 2, 2022
First decision: May 30, 2022
Revised: June 11, 2022
Accepted: August 15, 2022
Article in press: August 15, 2022
Published online: September 26, 2022

Polyarthritis is the most frequent clinical manifestation in antisynthetase syndrome (ASS) forms of idiopathic inflammatory myositis and may be misdiagnosed as rheumatoid arthritis (RA), particularly in patients with seronegative RA (SNRA). It is unclear whether there is an overlap between ASS and RA, or if ASS sometimes mimics RA. Pulmonary hypertension (PAH) is common in connective tissue diseases (CTDs). However, published reports on CTD-PAH do not include overlapping CTDs, and its incidence and impact on patient prognosis are unclear.


We report the case of a 63-year-old woman who presented with a 3-mo history of symptom aggravation of recurrent symmetrical joint swelling and pain that had persisted for over 10 years. The patient was diagnosed with RA and interstitial lung disease. The patient repeatedly presented to the hospital’s respiratory and rheumatology departments with arthralgia, plus shortness of breath after activity. Relevant tests indicated that anti-CCP and RF remained negative, while anti-J0-1 and anti-Ro-52 were strongly positive. It was not until recently that we recognized that this could be an unusual case of SNRA with concurrent ASS. Joint pain was relieved after regular anti-rheumatic treatment. Chest computed tomography scans showed that pulmonary interstitial changes did not progress significantly over several years; however, they showed gradual widening of the pulmonary artery, and cardiac ultrasound indicated elevated pulmonary artery systolic pressure. The prescribed treatment of PAH was not effective in improving shortness of breath.


Overlap of RA and ASS may be missed. Further research is necessary to facilitate early diagnosis, effective evaluation, and prognosis.

Keywords: Rheumatoid arthritis, Arthritis, Antisynthetase syndrome, Pulmonary hypertension, Pulmonary arterial hypertension, Case report

Core Tip: The joint manifestations of antisynthetase syndrome are usually difficult to distinguish from rheumatoid arthritis (RA), particularly seronegative RA (SNRA); consequently, rheumatologists and respiratory pathologists should be aware of this rare and underrecognized special clinical phenotype. Whether this phenotype is more prone to pulmonary hypertension than a single connective tissue disease remains unknown. However, further research into anti-Jo1 antibodies, anti-RO-52, and other extractable nuclear antigen autoantibodies is necessary to facilitate the early diagnosis, evaluation, and prognosis of this overlapping clinical syndrome.