All-cause and cause-specific mortality among patients with Behçet's disease versus the general population

Se Rim Choi; Anna Shin; You-Jung Ha; Yun Jong Lee; Eun Bong Lee; Eun-So Lee; Eun Ha Kang

doi:10.1093/bjd/ljae051

All-cause and cause-specific mortality among patients with Behçet's disease versus the general population

Br J Dermatol. 2024 Feb 6:ljae051. doi: 10.1093/bjd/ljae051. Online ahead of print.

Authors

Se Rim Choi¹, Anna Shin¹, You-Jung Ha^{1

2}, Yun Jong Lee^{1

2}, Eun Bong Lee^{2

3}, Eun-So Lee⁴, Eun Ha Kang^{1

2}

Affiliations

¹ Division of Rheumatology, Department of Internal Medicine, Seoul National University Bundang Hospital, Seongnam, Korea.
² Department of Internal Medicine, Seoul National University College of Medicine, Seoul, Korea.
³ Division of Rheumatology, Department of Internal Medicine, Seoul National University Hospital, Seoul, Korea.
⁴ Department of Dermatology, Ajou University School of Medicine, Suwon, Korea.

PMID: 38320213
DOI: 10.1093/bjd/ljae051

Abstract

Background: Comparative risk regarding cause-specific mortality between patients with Behcet's disease (BD) and the general population is not known.

Objectives: To compare the risk of all-cause and cause-specific mortality among patients with BD versus the general population.

Methods: Using the 2002-2020 Korea National Health Insurance Service database, we conducted a cohort study comparing BD patients and the general population matched on age and sex at a 1:4 ratio. We used Cox proportional hazard models to estimate hazard ratios (HRs) and 95% confidence intervals (CIs) for all-cause and cause-specific mortality. Subgroup analyses by age and sex were done.

Results: We included 24,669 BD patients and 98,676 age- and sex-matched controls (mean age 40.5 years, 34% male). During a mean follow-up of 11.9 years, the incidence rate of death per 100 person-years was 0.36 in BD and 0.29 in controls with the HR (95% CI) of 1.28 (1.20-1.38). The risk of mortality was highest for one year after BD diagnosis showing a HR (95% CI) of 2.66 (2.09-3.40). BD patients died more in this period due to malignancy, cardiovascular (CV), gastrointestinal, and respiratory disease, and infection: the corresponding cause-specific HR (95% CI) was 1.96 (1.30-2.98), 2.68 (1.45-4.97), 3.50 (1.35-9.07), 5.00 (1.34-18.62), and 3.33 (1.02-10.92), respectively. Mortality by neurological (HR 1.58, 95% CI 1.06-2.35) and genitourinary disease (HR 2.20, 95% CI 1.43-3.37) was also more common in BD during the overall follow-up. Subgroup analyses showed consistent results. The risk of CV mortality compared to the general population was higher in the young than elderly (p for interaction = 0.006), and the risk of gastrointestinal mortality in women than men (p for interaction = 0.04).

Conclusions: This population-based cohort study shows the first year of the disease as the highest risk window for excess mortality among BD patients. The mortality burden in BD derived from a wide spectrum of organ involvements, warning clinicians about the systemic nature of the disease.