Background/Purpose:  Several studies have implicated the increased risk of malignancy in patients with immunoglobulin G4-related disease (IgG4-RD). Hence, we first evaluated the risk and the type of malignancy in Korean patients with IgG4-RD.

Methods:  Clinical and laboratory results of patients with IgG4-RD according to the comprehensive diagnostic criteria for IgG4-RD were retrospectively collected using Clinical Data Repository in Severance Hospital, Seoul, South Korea, between January 2006 and November 2015. One hundred and eighteen patients were included, and 35 were classified as having definite, 70 as possible and 13 as probable IgG4-RD. We compared baseline clinical and laboratory variables between patients with definite and those with possible/probable IgG4-RD. Also we calculated the standardized incidence ratios (SIRs) for malignancies using the data from the Korea Central Cancer Registry.

Results:  The median age was 60.0 years old, and 91 patients (77.1%) were male. Malignancies were found in 12 of 118 patients (10.1%), and lymphoma (4, (25.0%)) was the most frequent malignancy related to IgG4-RD. Comparison of patients with definite and possible/probable IgG4-RD revealed that definite IgG4-RD patients had higher serum IgG level and frequent multiple organ involvement. Analysis of SIRs elucidated that overall incidence of malignancy was increased in patients with IgG4-RD compared to the general population (SIR 23.08 [95% CI 11.92, 40.31]), and the overall risk of SIRs was highest in Non-Hodgkin lymphoma (SIR 400.00 [95% CI 109.00, 1024.00]).

Conclusion:  Our data showed that the increased risk of malignancy, especially lymphomas, was observed in Korean patients with IgG4-RD compared to general population.

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ACR Meeting Abstracts - https://acrabstracts.org/abstract/malignancies-in-korean-patients-with-immunoglobulin-g4-related-disease/