CASE REPORT article

Front. Immunol.

Sec. Autoimmune and Autoinflammatory Disorders : Autoimmune Disorders

Volume 16 - 2025 | doi: 10.3389/fimmu.2025.1614777

This article is part of the Research TopicCase Reports in Autoimmune and Autoinflammatory Disorders: Volume IIView all 6 articles

Acute Exacerbation of Anti-Ha Antibody-Positive Antisynthetase Syndrome-Associated Interstitial Lung Disease: A Case Report

Provisionally accepted
Yinying  LIYinying LI1Junyi  KeJunyi Ke1Shu  HuangShu Huang1Shiya  LinShiya Lin1Siyu  LeiSiyu Lei1Hongchun  HuangHongchun Huang1Luxi  LeiLuxi Lei1Qinglan  LiQinglan Li1Yuanlan  WuYuanlan Wu1Minchao  DuanMinchao Duan1,2*
  • 1Guangxi Medical University, Nanning, China
  • 2Wuming Hospital Affiliated to Guangxi Medical University, Nanning, Guangxi Zhuang Region, China

The final, formatted version of the article will be published soon.

Background:Anti-synthetase syndrome (ASS) is a rare autoimmune myopathy, frequently associated with interstitial lung disease, and is characterized by the presence of anti-aminoacyl tRNA synthetase (ARS) antibodies. However, there have been limited reports of cases exhibiting positive anti-Ha antibodies.Methods:This study presents a retrospective analysis of the clinical data from a patient with an acute exacerbation of ASS who tested positive for anti-Ha antibodies.Case presentation:This patient initially presented with interstitial pneumonia. The initial anti-infective treatment was ineffective; however, symptoms improved following the addition of corticosteroids. Upon discontinuation of corticosteroids, the patient experienced a recurrence of cough, progressive worsening of dyspnea, and developed lower general weakness. Comprehensive autoantibody testing revealed positivity for anti-Ha antibodies, and MRI of the lower limbs indicated soft tissue edema. The patient was ultimately diagnosed with ASS with interstitial lung disease. Treatment with methylprednisolone pulse therapy, combined with cyclophosphamide, tacrolimus, tofacitinib citrate, and pirfenidone, led to an improvement in the patient's condition, resulting in discharge. Post-discharge, the patient was maintained on regular oral prednisone, nintedanib, and tofacitinib. Follow-up to date has shown a stable condition, with resolution of pulmonary lesions observed upon re-examination.Conclusion:Anti-Ha antibody is one of the specific antibodies associated with ASS, yet its positive rate remains exceedingly low. This case represents the first reported instance of an anti-Ha antibody-positive ASS in China. Misdiagnosis and missed diagnosis are prevalent in clinical practice, underscoring the importance of screening for autoantibodies when patients present with acute, unexplained interstitial lung changes and a poor response to anti-infective treatment. Furthermore, interstitial lung disease is the most common extra-muscular clinical manifestation observed in ASS patients. Differentiating between acute exacerbations of pulmonary infections and interstitial lung disease associated with rheumatic diseases poses a significant challenge, as both can occur concurrently. Therefore, during diagnosis and treatment, it is crucial to consider not only infections but also to identify the underlying causes of worsening lung lesions.

Keywords: Anti-synthetase syndrome (ASS), anti-Ha antibody, ILD, case report, Corticosteroids therapy

Received: 19 Apr 2025; Accepted: 27 May 2025.

Copyright: © 2025 LI, Ke, Huang, Lin, Lei, Huang, Lei, Li, Wu and Duan. This is an open-access article distributed under the terms of the Creative Commons Attribution License (CC BY). The use, distribution or reproduction in other forums is permitted, provided the original author(s) or licensor are credited and that the original publication in this journal is cited, in accordance with accepted academic practice. No use, distribution or reproduction is permitted which does not comply with these terms.

* Correspondence: Minchao Duan, Guangxi Medical University, Nanning, China

Disclaimer: All claims expressed in this article are solely those of the authors and do not necessarily represent those of their affiliated organizations, or those of the publisher, the editors and the reviewers. Any product that may be evaluated in this article or claim that may be made by its manufacturer is not guaranteed or endorsed by the publisher.