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

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.