Can antineutrophil cytoplasmic antibody positivity at diagnosis predict the poor outcomes of Sjögren’s syndrome?

  • Soo Bin Lee
  • Hyeok Choi
  • Minyoung Kevin Kim
  • Seung Min Jung
  • Jason Jungsik Song
  • Yong-Beom Park
  • Sang-Won LeeEmail author
Observational Research


We investigated the clinical implication of ANCA positivity at diagnosis on the poor outcomes in patients with Sjögren’s syndrome. The medical records of 606 Korean patients with Sjögren’s syndrome were retrospectively reviewed. The results of perinuclear (P)-ANCA, myeloperoxidase (MPO)-ANCA, cytoplasmic (C)-ANCA, and proteinase 3 (PR3)-ANCA were collected and the frequencies of all-cause mortality, interstitial lung disease (ILD), end-stage renal disease (ESRD), and lymphoma were assessed as the poor outcomes of Sjögren’s syndrome. Comparison of the cumulative patient survivals between the two groups was analysed by the Kaplan–Meier survival analysis. Of the 606 patients, ANCA was detected in 10.2% of Sjögren’s syndrome patients without AAV. Twenty-one patients (3.5%) died, 99 patients (16.3%) suffered from ILD, and 8 patients had ESRD. Lymphoma occurred in 5 patients (0.8%) during 37.5 months. Sjögren’s syndrome patients with ANCA positivity exhibited a lower cumulative ILD-free survival rate than those with ANCA negativity (P = 0.001). Sjögren’s syndrome patients with P-ANCA positivity and those with MPO-ANCA (or P-ANCA) positivity showed a lower cumulative ILD-free survival rate than those without (P = 0.012 and P < 0.001). Also, Sjögren’s syndrome patients with P-ANCA positivity exhibited a lower cumulative ESRD-free survival rate than those without (P = 0.043). ANCA positivity was associated with neither all-cause mortality nor lymphoma in Sjögren’s syndrome patients. ANCA positivity and MPO-ANCA (or P-ANCA) positivity at diagnosis was associated with the development of ILD during follow-up in patients with Sjögren’s syndrome.


Antineutrophil cytoplasmic antibody Sjögren’s syndrome Interstitial lung disease Predictor 


Author contributions

All authors contributed to conception and design, or acquisition of data, or analysis and interpretation of data, and participated in drafting the manuscript or revising it critically for important intellectual content. Also, all authors gave final approval of the version to be published and agreed to be accountable for all aspects of the work in ensuring that questions related to the accuracy or integrity of any part of the work are appropriately investigated and resolved.


This research was supported by Basic Science Research Program through the National Research Foundation of Korea (NRF) funded by the Ministry of Education (2017R1D1A1B03029050) and a Grant from the Korea Health Technology R&D Project through the Korea Health Industry Development Institute, funded by the Ministry of Health and Welfare, Republic of Korea (HI14C1324).

Compliance with ethical standards

Conflict of interest

The authors declare that they have no conflict of interest.

Ethical approval

This study was approved by the Institutional Review Board (IRB) of Severance Hospital (4-2017-0673).

Informed consent

The patients’ written informed consent was waived by the approving IRB, as this was a retrospective study.

Supplementary material

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Supplementary material 1 (SAV 25 kb)
296_2019_4476_MOESM2_ESM.docx (17 kb)
Supplementary material 2 (DOCX 16 kb)


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Copyright information

© Springer-Verlag GmbH Germany, part of Springer Nature 2019

Authors and Affiliations

  1. 1.Department of MedicineYonsei University College of MedicineSeoulRepublic of Korea
  2. 2.Division of Rheumatology, Department of Internal MedicineYonsei University College of MedicineSeoulRepublic of Korea
  3. 3.Institute for Immunology and Immunological DiseasesYonsei University College of MedicineSeoulRepublic of Korea

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