AB0760 HIGH SERUM IGA LEVELS HAVE LIMITED CLINICAL SIGNIFICANCE IN PATIENTS WITH IGG4-RELATED DISEASE DIAGNOSED BY EXPERTS
Authors: Tsuge S. , Mizushima I. , Shin S. , Yoshinobu T. , Hoshiba R. , Nishioka R. , Zoshima T. , Hara S. , Suzuki Y. , Ito K. , Kawano M. 
Published: May 2021

Background:

While the diagnostic and classification criteria for IgG4-related disease (IgG4-RD) have been recently developed [1-3], it is known that, without appropriate exclusions, some non-IgG4-RDs may meet these criteria. In particular, hyper IL-6 syndromes, including Castleman disease, can be misdiagnosed as IgG4-RD. Some clinical findings, including elevated serum levels of C-reactive protein (CRP) or IgA, have been suggested to be useful for differentiating hyper IL-6 syndromes from IgG4-RD [4]. However, since few clinical studies have focused on IgG4-RD with high serum IgA levels, its clinical significance has not been well known.

Objectives:

This study aimed to clarify the clinical significance of high serum IgA levels in patients with IgG4-RD.

Methods:

We retrospectively investigated the clinical features of 170 patients with IgG4-RD on the basis of the presence or absence of elevated serum IgA levels (>410 mg/dL) at the time of diagnosis. The diagnosis of IgG4-RD was made by experts on the basis of the fulfillment of the comprehensive diagnostic criteria and/or each organ-specific diagnostic criteria.

Results:

Elevated serum IgA levels were observed in 18 patients (10.6%). In the patients with elevated serum IgA levels, serum CRP levels were higher (1.14 ± 1.18 vs. 0.31 ± 0.63 mg/dL, p=0.003) and the prevalence of relapse during the clinical course was lower (5.6% vs. 27.6%, p=0.046) than in those without elevated serum IgA levels. However, there were no significant differences in the other clinical features including the number of involved organs (2.4 ± 1.3 vs. 2.8 ± 1.6, p=0.443) and inclusion scores of the ACR/EULAR classification criteria (32 ± 14 vs. 36 ± 17, p=0.374). To evaluate the influence of serum IgA elevation on relapse, we performed Cox regression analysis, which showed that the elevated serum IgA levels had no significant association with lower incidence of relapse but a tendency of it (hazard ratio 0.997, 95% confidence interval 0.994-1.000, p=0.055) during the clinical course. In addition, a prompt improvement in the IgG4-RD responder index [5] during the clinical course was seen in the patients with serum IgA elevation, suggesting a similar good response to glucocorticoids as in those without it.

Conclusion:

The findings of the present study suggest that IgG4-RD patients with high serum IgA levels can be diagnosed and treated in the same way as those without it, although they may be characterized by mild serum CRP elevation.

References:

[1]Wallace ZS et al. The 2019 American College of Rheumatology/European League Against Rheumatism classification criteria for IgG4-related disease. Ann Rheum Dis. 2020;79:77-87.

[2]Umehara H et al. The 2020 Revised Comprehensive Diagnostic (RCD) Criteria for IgG4-RD. Mod Rheumatol. 2020 Dec 4:1-14. doi: 10.1080/14397595.2020.1859710. Online ahead of print.

[3]Umehara H et al. Current approach to the diagnosis of IgG4-related disease - Combination of comprehensive diagnostic and organ - specific criteria. Mod Rheumatol. 2017;27:381-91.

[4]Sato Y et al. Systemic IgG4-related lymphadenopathy: A clinical and pathologic comparison to multicentric Castleman’s disease. Mod Pathol.2009;22:589-99.

[5]Carruthers MN et al. Development of an IgG4-RD Responder Index. Int J Rheumatol. 2012;2012:259408.

Disclosure of Interests:

None declared