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Malignancies in Patients with IgG4-Related Respiratory Disease
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A1529 - Malignancies in Patients with IgG4-Related Respiratory Disease
Author Block: S. Matsui1, H. Yamamoto2, T. Handa3, S. Okazawa4, K. Tokui4, C. Taka4, S. Imanishi4, K. Kambara4, T. Ichikawa4, M. Inomata4, R. Hayashi4, The study group of IgG4-RD; 1University of Toyama, Toyama, Japan, 2Shinshu University, Matsumoto, Japan, 3Department of Respiratory Medicine, Kyoto Univ Hosp, Kyoto, Japan, 4Toyama University Hospital, Toyama, Japan.
Background and Aim: Immunoglobulin G4-related disease (IgG4-RD) is defined as a multi-organ disease that includes pulmonary disorders. Several reports have shown that patients with IgG4-RD are at increased risk of malignancy. This study evaluated the risk and types of malignancy in patients with IgG4-related respiratory disease (IgG4-RRD). Method: This multicenter retrospective study evaluated the clinical and laboratory data of Japanese patients fulfilling the comprehensive diagnostic criteria for IgG4-RD (CDC, 2011) and the proposed diagnostic criteria for IgG4-RRD (2014) between January 2003 and December 2016. The standardized incidence ratio (SIR) for malignancies was calculated using data from national cancer registries that monitor cancer incidence in Japan. Results: Fifty-two patients (39 men and 13 women), of mean age 66 years fulfilled the diagnostic criteria for IgG4-RRD during the study period. At diagnosis, their mean serum concentrations of IgG, IgG4, and soluble IL-2R were 2879 mg/dl, 1078 mg/dl, and 1193 U/ml, respectively. Fourteen malignancies were found in 12 patients (23.1%) during a mean follow-up period of 5.66 years, with eight (57.1%) of these malignancies found during the first 2 years after diagnosis of IgG4-RD. Four patients had lung cancer; three had colon cancer; three had lymphoma; and one each had pharyngeal cancer, bladder cancer, prostate cancer, and breast cancer. Serum concentrations of IgG and IgG4 in patients with malignancies were 2759 mg/dl and 1048 mg/dl, respectively. One patient (8.3%) died during the follow-up period. The SIR for malignancies in patients with IgG4-RRD was 2.85 (95% confidence interval 1.24-4.46). Conclusion: The incidence of malignancies was higher in patients with IgG4-RRD than in the general population. These malignancies may be found at the time of or after diagnosis of IgG4-RD, but no specific signs or findings have been observed. Careful clinical observation and monitoring are necessary for diagnosis and follow-up of patients with IgG4-RRD.
Author Block: S. Matsui1, H. Yamamoto2, T. Handa3, S. Okazawa4, K. Tokui4, C. Taka4, S. Imanishi4, K. Kambara4, T. Ichikawa4, M. Inomata4, R. Hayashi4, The study group of IgG4-RD; 1University of Toyama, Toyama, Japan, 2Shinshu University, Matsumoto, Japan, 3Department of Respiratory Medicine, Kyoto Univ Hosp, Kyoto, Japan, 4Toyama University Hospital, Toyama, Japan.
Background and Aim: Immunoglobulin G4-related disease (IgG4-RD) is defined as a multi-organ disease that includes pulmonary disorders. Several reports have shown that patients with IgG4-RD are at increased risk of malignancy. This study evaluated the risk and types of malignancy in patients with IgG4-related respiratory disease (IgG4-RRD). Method: This multicenter retrospective study evaluated the clinical and laboratory data of Japanese patients fulfilling the comprehensive diagnostic criteria for IgG4-RD (CDC, 2011) and the proposed diagnostic criteria for IgG4-RRD (2014) between January 2003 and December 2016. The standardized incidence ratio (SIR) for malignancies was calculated using data from national cancer registries that monitor cancer incidence in Japan. Results: Fifty-two patients (39 men and 13 women), of mean age 66 years fulfilled the diagnostic criteria for IgG4-RRD during the study period. At diagnosis, their mean serum concentrations of IgG, IgG4, and soluble IL-2R were 2879 mg/dl, 1078 mg/dl, and 1193 U/ml, respectively. Fourteen malignancies were found in 12 patients (23.1%) during a mean follow-up period of 5.66 years, with eight (57.1%) of these malignancies found during the first 2 years after diagnosis of IgG4-RD. Four patients had lung cancer; three had colon cancer; three had lymphoma; and one each had pharyngeal cancer, bladder cancer, prostate cancer, and breast cancer. Serum concentrations of IgG and IgG4 in patients with malignancies were 2759 mg/dl and 1048 mg/dl, respectively. One patient (8.3%) died during the follow-up period. The SIR for malignancies in patients with IgG4-RRD was 2.85 (95% confidence interval 1.24-4.46). Conclusion: The incidence of malignancies was higher in patients with IgG4-RRD than in the general population. These malignancies may be found at the time of or after diagnosis of IgG4-RD, but no specific signs or findings have been observed. Careful clinical observation and monitoring are necessary for diagnosis and follow-up of patients with IgG4-RRD.
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