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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

 Juyoung Yoo  ;  Jason Jungsik Song  ;  Yong-Beom Park  ;  Sang-Won Lee 
 CLINICAL RHEUMATOLOGY, Vol.39(10) : 3009-3015, 2020-10 
Journal Title
Issue Date
Eosinophilic granulomatosis with polyangiitis ; IgG4-related disease ; Overlap
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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1. College of Medicine (의과대학) > Dept. of Internal Medicine (내과학교실) > 1. Journal Papers
Yonsei Authors
Park, Yong Beom(박용범)
Song, Jason Jungsik(송정식) ORCID logo https://orcid.org/0000-0003-0662-7704
Yoo, Juyoung(유주영) ORCID logo https://orcid.org/0000-0001-8882-1695
Lee, Sang-Won(이상원) ORCID logo https://orcid.org/0000-0002-8038-3341
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