Abstract
It has been reported that patients with IgG4-related disease (IgG4-RD) showed an elevated incidence of eosinophilia. We aim to explore the clinical patterns of IgG4-RD patients with and without eosinophilia. Four hundred and twenty-five IgG4-RD patients referred to Peking Union Medical College Hospital were enrolled. Blood eosinophil count higher than 0.5 × 109/L was defined as eosinophilia. Clinical features of all the participants were collected and analyzed statistically. Eighty-seven patients (20%) with eosinophilia were found. As compared to those with a normal range of blood eosinophil count, male predominance, longer disease duration, increased prevalence of dacryoadenitis, sialadenitis, lymphadenopathy, and skin rash, higher IgG4-RD responder index, more organ involvement and higher levels of serum IgG4 (17.0 g/L vs 6.5 g/L, P < 0.001) was found in patients with eosinophilia. There was no significant difference in the incidence of allergic disease between the two groups. Peripheral eosinophil counts were positively correlated with disease duration, the number of involved organs, IgG4-RD responder index, and serum IgG4. Higher recurrence rate during follow-up period was found in patients with eosinophilia [28.6% (20/70) vs 17.1% (42/245), P = 0.034]. IgG4-RD patients with eosinophilia exhibited different clinical patterns from patients without. Eosinophilia appeared independent of allergies in IgG4-RD.
Highlights
Eosinophils are terminally differentiated cells of the myeloid lineage implicated in the pathogenesis of numerous inflammatory processes1
We investigated the prevalence of eosinophilia in Immunoglobulin G4-related disease (IgG4-RD) patients in the largest prospective IgG4-RD cohort in China, and report here for the first time that patients with eosinophilia presented with significantly different clinical patterns in comparison to those with normal peripheral eosinophil count
One study found that the presence of eosinophilia did not result in a different clinical pattern in patients with type 1 autoimmune pancreatitis11
Summary
Eosinophils are terminally differentiated cells of the myeloid lineage implicated in the pathogenesis of numerous inflammatory processes. Eosinophilia had been reported to be associated with IgG4-RD to varying degrees (11–38%). Eosinophilia had been reported to be associated with IgG4-RD to varying degrees (11–38%)5–11 This eosinophilia appeared inherent to the IgG4-RD rather than atopic disease. Eosinophilic angiocentric fibrosis was recently described as a form of IgG4-related systemic disease. In 2010, Sah et al reported a similar clinical profile in type 1 autoimmune pancreatitis patients with and without peripheral eosinophilia. In 2014, Della-Torre et al reported that there was a positive correlation between eosinophil count and serum IgG410. We investigated the prevalence of eosinophilia in IgG4-RD patients in the largest prospective IgG4-RD cohort in China, and report here for the first time that patients with eosinophilia presented with significantly different clinical patterns in comparison to those with normal peripheral eosinophil count
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