Case Report
Copyright ©The Author(s) 2024. Published by Baishideng Publishing Group Inc. All rights reserved.
World J Clin Cases. Jun 6, 2024; 12(16): 2881-2886
Published online Jun 6, 2024. doi: 10.12998/wjcc.v12.i16.2881
Dynamically changing antineutrophil cytoplasmic antibodies in granulomatosis with polyangiitis: A case report
Yan Zhang, Qiao-Ding Dai, Ji-An Wang, Li-Ping Xu, Qiang Chen, Yang-Zi Jin
Yan Zhang, Qiao-Ding Dai, Ji-An Wang, Li-Ping Xu, Department of Rheumatism and Immunology, The First Affiliated Hospital of Zhejiang Chinese Medical University/Zhejiang Provincial Hospital of Traditional Chinese Medicine, Hangzhou 310006, Zhejiang Province, China
Qiang Chen, Department of Orthopaedics, The Affiliated Xiaoshan Hospital of Hangzhou Normal University, Hangzhou 311202, Zhejiang Province, China
Yang-Zi Jin, Department of Otolaryngology, The First Affiliated Hospital of Zhejiang Chinese Medical University/Zhejiang Provincial Hospital of Traditional Chinese Medicine, Hangzhou 310006, Zhejiang Province, China
Author contributions: Zhang Y performed literature review and wrote the manuscript; Dai QD, Wang JA and Xu LP participated in the patient's diagnosis and treatment process and collected data; Chen Q provided assistance in the English language editing of this article; Jin YZ wrote the manuscript and assisted the manuscript editing. All authors have read and approved the final manuscript.
Supported by The Research Project of Zhejiang Chinese Medical University, No. 2023JKZKTS33.
Informed consent statement: Informed written consent was obtained from the patient for publication of this report and any accompanying images.
Conflict-of-interest statement: The authors declare that they have no conflict of interest to disclose.
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 NonCommercial (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: https://creativecommons.org/Licenses/by-nc/4.0/
Corresponding author: Yang-Zi Jin, MD, Attending Doctor, Department of Otolaryngology, The First Affiliated Hospital of Zhejiang Chinese Medical University/Zhejiang Provincial Hospital of Traditional Chinese Medicine, No. 54 Youdian Road, Hangzhou 310006, Zhejiang Province, China. 20163249@zcmu.edu.cn
Received: February 4, 2024
Revised: March 4, 2024
Accepted: April 10, 2024
Published online: June 6, 2024
Processing time: 115 Days and 0.1 Hours
Abstract
BACKGROUND

Granulomatosis with polyangiitis (GPA) is one of the most prevalent forms of the antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis. GPA is characterized histologically by necrotizing granulomatous inflammation in addition to vasculitis. The diagnosis of GPA depends on clinical presentation, serological evidence of a positive ANCA, and/or histological evidence of necrotizing vasculitis or granulomatous destructive parenchymal inflammation. Cytoplasmic ANCA (c-ANCA) is positive in 65%-75% of GPA patients, accompanied by proteinase 3 (PR3), the main target antigen of c-ANCA, another 5% of GPA patients had negative ANCA.

CASE SUMMARY

The patient, a 52-year-old male, presented with unexplained nasal congestion, tinnitus, and hearing loss. After a duration of 4 months experiencing these symptoms, the patient subsequently developed fever and headache. The imaging examination revealed the presence of bilateral auricular mastoiditis and partial paranasal sinusitis, and the ANCA results were negative. The anti-infective therapy proved to be ineffective, but the patient's symptoms and fever were quickly relieved after 1 wk of treatment with methylprednisolone 40 mg once a day. However, after continuous use of methylprednisolone tablets for 3 months, the patient experienced a recurrence of fever accompanied by right-sided migraine, positive c-ANCA and PR3, and increased total protein in cerebrospinal fluid. The patient was diagnosed with GPA. After receiving a treatment regimen of intravenous methylprednisolone 40 mg/d and cyclophosphamide 0.8 g monthly, the patient experienced alleviation of fever and headache. Additionally, the ANCA levels became negative and there has been no recurrence.

CONCLUSION

For GPA patients with negative ANCA, there is a potential for early missed diagnosis. The integration of histopathological results and multidisciplinary communication plays a crucial role in facilitating ANCA-negative GPA.

Keywords: Anti-neutrophil cytoplasmic antibodies, Granulomatosis with polyangiitis, Antineutrophil cytoplasmic antibody-associated vasculitis, Immunosuppressive therapy, Case report

Core Tip: In this case, otomastoiditis was the initial clinical manifestation, and then the clinical manifestations of central system involvement gradually appeared. Laboratory examination showed that proteinase 3-antineutrophil cytoplasmic antibody (ANCA) changed from negative to positive 3 months later. Therefore, for patients with clinical suspicion of granulomatosis with polyangiitis (GPA), repeated examination of ANCA should be considered, and obtained pathological evidence whenever possible. Meanwhile, the importance of early participation of the rheumatological immunology team is emphasized. Through multidisciplinary communication, the probability of early diagnosis of GPA can be improved, and timely drug intervention can be carried out to prevent disease progression and reduce the risk of local and systemic sequelae.