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No overlap between IgG4-related disease and microscopic polyangiitis and granulomatosis with polyangiitis despite elevated serum IgG4 at diagnosis: a retrospective monocentric study

 Juyoung Yoo  ;  Sung Soo Ahn  ;  Seung Min Jung  ;  Jason Jungsik Song  ;  Yong-Beom Park  ;  Sang-Won Lee 
 CLINICAL RHEUMATOLOGY, Vol.38(4) : 1147-1154, 2019 
Journal Title
Issue Date
Granulomatosis with polyangiitis ; IgG4-related disease ; Microscopic polyangiitis
OBJECTIVES: We investigated whether elevated serum IgG4 at the time of diagnosis of microscopic polyangiitis (MPA) and granulomatosis with polyangiitis (GPA) may be associated with concurrent IgG4-related disease (IgG4-RD) in immunosuppressive drug-naïve patients.

METHODS: We retrospectively reviewed the medical records of 46 MPA and GPA patients with results on serum IgG4 and histology at diagnosis. Elevated serum IgG4 was defined as IgG4 > 135 mg/dL. We collected clinical and laboratory data at diagnosis including ANCA, white blood cell (WBC) count, haemoglobin, platelet, erythrocyte sedimentation rate (ESR), C-reactive protein (CRP), and serum IgG4, and calculated Birmingham vasculitis activity score (BVAS) at diagnosis. We compared variables between patients with MPA and GPA and assessed the correlation of serum IgG4 and other continuous variables.

RESULTS: Twenty-eight patients (60.9%) were classified as MPA and 18 patients (39.1%) as GPA. The mean age at diagnosis was 61.0 years and 17 patients (37.0%) were men. The serum IgG4 at diagnosis was 1202.7 mg/dL and 37 patients (80.4%) had elevated serum IgG4 at diagnosis. We found no patients, who could be classified as IgG4-RD according to comprehensive diagnostic criteria for IgG4-RD among 46 patients. The mean serum IgG at diagnosis was not different between the two groups. Serum IgG4 was significantly correlated with inflammation-related variables at diagnosis including BVAS (r = 0.367), platelet (r = 0.398), ESR (r = 0.327), and CRP (r = 0.373).

CONCLUSIONS: Elevated serum IgG4 is not associated with concurrent IgG4-RD, and it may reflect activity and inflammatory burden of vasculitis in patients with MPA and GPA at diagnosis.
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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
Ahn, Sung Soo(안성수) ORCID logo https://orcid.org/0000-0002-9002-9880
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
Jung, SeungMin(정승민) ORCID logo https://orcid.org/0000-0003-3465-2181
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