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

Autor: Hiroyuki Kawahara, Ichiro Mizushima, Shunsuke Tsuge, Seung Shin, Takahiro Yoshinobu, Ryohei Hoshiba, Ryo Nishioka, Takeshi Zoshima, Satoshi Hara, Kiyoaki Ito, Mitsuhiro Kawano
Rok vydání: 2022
Předmět:
Zdroj: Modern Rheumatology.
ISSN: 1439-7609
1439-7595
Popis: Background: Few observations on the long-term prognosis have been conducted in immunoglobulin G4-related disease (IgG4-RD) patients with various organ involvement, not limited to autoimmune pancreatitis. Especially, mortality and its related factors in patients with IgG4-RD with various organ involvement are not well known. This study aimed to clarify mortality trends and its related factors in IgG4-RD with various organ involvement.Methods: We retrospectively reviewed the medical records of patients with IgG4-RD at a single center in Japan. We calculated the crude mortality rate and the standardized mortality ratio (SMR) using national Japan mortality statistics and investigated the cause of death. We performed Cox regression analyses to assess mortality-related factors.Results: A total of 179 patients with IgG4-RD were included and the median follow-up from diagnosis was 47 months (IQR 19-96). Ten patients (5.6%) in our cohort died during the follow-up period. The crude mortality rate was 11.1 per 1,000 person-years. According to national Japan mortality statistics, 11.6 age- and sex-matched deaths would have been expected to occur within the follow-up period, resulting in an SMR of 0.86 (95% confidence interval [CI] 0.41-1.59). Univariate Cox regression analyses indicated that the number of affected organs at diagnosis (hazard ratio [HR] 1.45, 95% CI 1.02-2.05), eGFR 2 at diagnosis (vs. ≥45, HR 8.48, 95% CI 2.42-29.79), and the presence of malignancy during the clinical course (HR 3.93, 95% CI 1.10-14.02) had a significant impact on the time to death.Conclusions: Our findings suggested that IgG4-RD does not significantly affect long-term patient survival. On the other hand, multi-organ involvement and renal dysfunction as well as malignancy might be associated with higher mortality trends in IgG4-RD. Early detection and appropriate management of risk factors may improve the long-term prognosis of IgG4-RD.
Databáze: OpenAIRE
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