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Clues to mortality trends and their related factors in IgG4-related disease: A Japanese single-centre retrospective study

Modern Rheumatology, ISSN: 1439-7609, Vol: 33, Issue: 6, Page: 1154-1161
2023
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Article Description

Objectives: This study aimed to clarify mortality trends and their related factors in immunoglobulin G4-related disease (IgG4-RD) with various organ involvement. Methods: We retrospectively reviewed the medical records of patients with IgG4-RD at a single rheumatology centre in Japan. We calculated the standardized mortality ratio using Japanese national mortality statistics. Cox regression analyses were also performed to assess mortality-related factors. Results: A total of 179 patients with IgG4-RD were included with a median follow-up period of 47 months. The standardized mortality ratio in our cohort was 0.86 (95% confidence interval 0.41–1.59). Univariate Cox regression analyses indicated that the number of affected organs at diagnosis (hazard ratio 1.45, 95% confidence interval 1.02–2.05), estimated glomerular infiltration rate <45 ml/min/1.73 m at diagnosis (vs. ≥45, hazard ratio 8.48, 95% confidence interval 2.42–29.79), and the presence of malignancy during the clinical course (hazard ratio 5.85, 95% confidence interval 1.62–21.15) had a significant impact on the time to death. Conclusions: Our findings suggest that in the rheumatology department, IgG4-RD does not significantly affect long-term patient survival. However, multi-organ involvement, renal dysfunction, and malignancy may be associated with higher mortality trends in IgG4-RD. Early detection and appropriate management of risk factors may improve the long-term prognosis of patients with IgG4-RD.

Bibliographic Details

Kawahara, Hiroyuki; Mizushima, Ichiro; Tsuge, Shunsuke; Shin, Seung; Yoshinobu, Takahiro; Hoshiba, Ryohei; Nishioka, Ryo; Zoshima, Takeshi; Hara, Satoshi; Ito, Kiyoaki; Kawano, Mitsuhiro

Oxford University Press (OUP)

Medicine

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