The Korean journal of internal medicine

The Korean Association of Internal Medicine (대한내과학회)
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Overlap syndrome of systemic sclerosis with antineutrophil cytoplasmic antibody-associated vasculitis according to 2022 ACR/EULAR criteria

  • Jang Woo Ha (Division of Rheumatology, Department of Internal Medicine, Yonsei University College of Medicine) ;
  • Jung Yoon Pyo (Division of Rheumatology, Department of Internal Medicine, Yonsei University College of Medicine) ;
  • Sung Soo Ahn (Department of Internal Medicine, Yongin Severance Hospital, Yonsei University College of Medicine) ;
  • Jason Jungsik Song (Division of Rheumatology, Department of Internal Medicine, Yonsei University College of Medicine) ;
  • Yong-Beom Park (Division of Rheumatology, Department of Internal Medicine, Yonsei University College of Medicine) ;
  • Sang-Won Lee (Division of Rheumatology, Department of Internal Medicine, Yonsei University College of Medicine)
  • Received : 2023.07.05
  • Accepted : 2023.09.09
  • Published : 2024.05.01
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Abstract

Background/Aims: This study applied the 2022 American College of Rheumatology (ACR)/European Alliance of Associations for Rheumatology (EULAR) criteria for antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) to patients with systemic sclerosis (SSc) and investigated the frequency of overlap syndrome of SSc and AAV (SSc-AAV-OS). Methods: Among the 232 patients diagnosed with SSc, 105 with signs suggestive of small- or medium-vessel vasculitis, which were defined as the present of interstitial lung disease (ILD), peripheral neuropathy, or suspected renal vasculitis, were included in this study and analyzed. Results: Among the 105 SSc patients, the detection rate of ANCA was 19.0%. When the 2022 ACR/EULAR criteria were applied, the frequency of SSc-AAV-OS was 20.0%, which was much higher than 1.7% reported with previous criteria for AAV. ANCA positivity contributed to the reclassification of SSc-AAV-OS more than ANCA negativity in SSc patients with signs suggestive of small- or medium-vessel vasculitis. Conclusions: The frequency of SSc-AAV-OS in SSc patients with signs suggestive of small- or medium-vessel vasculitis at diagnosis was 20.0%. Therefore, we suggest that physicians should perform ANCA tests in SSc patients exhibiting signs suggestive of small- or medium-vessel vasculitis and apply the new criteria for AAV.

Keywords

Acknowledgement

This study received funding from the Korea Health Technology R&D Project through the Korea Health Industry Development Institute, funded by the Ministry of Health and Welfare (HI14C1324), Handok Inc., Seoul, Republic of Korea (HANDOK 2021-006), and from CELLTRION PHARM, Inc. Chungcheongbuk-do, Republic of Korea (NCR 2019-6).

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