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Definite IgG4-related disease had no overlap with eosinophilic granulomatosis with polyangiitis in Korean patients: a pilot study in one centre

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Abstract

Objectives

Serum IgG4 may often increases in allergic diseases. Eosinophilic granulomatosis with polyangiitis (EGPA) has an allergic phase. For this reason, it was questionable whether IgG4-RD and EGPA may share some clinical and laboratory features. In this study, we investigated whether definite IgG4-RD might share those features with EGPA.

Methods

We retrospectively reviewed the medical records of 42 Korean immunosuppressive drug-naïve patients with definite IgG4-RD. The American College of Rheumatology 1990 criteria for classification (the 1990 ACR criteria) for Churg-Strauss syndrome (CSS, known as EGPA) were applied to patients who were previously diagnosed with definite IgG4-RD and had no antineutrophil cytoplasmic antibody (ANCA). Definite IgG4-RD was defined when 3 components of the comprehensive diagnostic criteria were met.

Results

The median age of 42 patients was 61.0 years and 69% of them were men. The median serum IgG4 level was 379.0 mg/dL and the median IgG4+/IgG+ cells ratio was 50.0%. The most common biopsy site was the salivary gland (14.3%). Antinuclear antibody was detected in 6 patients, and test results for ANCA were negative in all patients. Among 42 patients with definite IgG4-RD without ANCA, none of them met the 1990 ACR classification criteria for EGPA.

Conclusions

Definite IgG4-RD found to have no overlap with EGPA when the 1990 ACR criteria for CCS were applied to Korean patients.

Key Points

• None of 42 Korean patients with definite IgG4-RD without ANCA met the 1990 ACR classification criteria for EGPA.

• Definite IgG4-RD found to have no overlap with EGPA in Korean patients without ANCA.

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Acknowledgements

We would like to thank Dr. Minyoung Kevin Kim and Editage (www.editage.co.kr) for English language editing.

Funding

This research was supported by a grant from the Korea Health Technology R and D Project through the Korea Health Industry Development Institute, funded by the Ministry of Health and Welfare, Republic of Korea (HI14C1324).

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Authors and Affiliations

  1. Division of Rheumatology, Department of Internal Medicine, Yonsei University College of Medicine, 50-1 Yonsei-ro, Seodaemun–gu, Seoul, 03722, Republic of Korea

    Juyoung Yoo, Jason Jungsik Song, Yong-Beom Park & Sang-Won Lee

  2. Institute for Immunology and Immunological Diseases, Yonsei University College of Medicine, Seoul, Republic of Korea

    Jason Jungsik Song, Yong-Beom Park & Sang-Won Lee

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  1. Juyoung Yoo
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Contributions

Sang-Won Lee and Juyoung Yoo contributed to the study conception and design. Juyoung Yoo and Jason Jungsik Song contributed to acquisition of material and data. Juyoung Yoo, Yong-Beom Park and Sang-Won Lee contributed to the analysis and interpretation of data. The first draft of the manuscript was written by Byung-Woo Yoo, and all authors commented and revised on previous versions of the manuscript. All authors read and approved the final manuscript.

Corresponding author

Correspondence to Sang-Won Lee.

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Ethical approval

This study was approved by the institutional Review Board (IRB) of Severance Hospital (4–2019-0695).

Informed consent

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

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Yoo, J., Song, J.J., Park, YB. et al. Definite IgG4-related disease had no overlap with eosinophilic granulomatosis with polyangiitis in Korean patients: a pilot study in one centre. Clin Rheumatol 39, 3009–3015 (2020). https://doi.org/10.1007/s10067-020-05104-5

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