Demography of vascular Behcet’s disease with different gender and age: an investigation with 166 Chinese patients

Yong Chen†,
Jian-Fei Cai†,
Chen-Hong Lin and
Jian-Long GuanEmail author

†Contributed equally

Orphanet Journal of Rare Diseases201914:88

https://doi.org/10.1186/s13023-019-1061-1

Received: 14 September 2018
Accepted: 8 April 2019
Published: 29 April 2019

Abstract

Background

The Clinical features of vascular Behcet’s disease (BD) are not well understood because there are few studies. Our study aimed to investigate characteristics of vascular BD in both genders in different age groups.

Results

We enrolled 923 patients with BD who presented to our hospital with adequate medical histories and proper vascular screening exams. The raw incidence rate of vascular BD was 17.98% (166/923). The ratio of vascular BD in male to female patients was 1.868 (p = 0.0004, 95% confidence interval (CI): 1.317 to 2.625). There was a tendency towards higher ESR and CRP in vascular BD patients than in mucocutaneous, but the difference was not significant. The most susceptible affected vessels were cerebral (29.6% in males, 59.4% in females) and lower limb vessels (31.2% in males, and 17.2% in females). The incidence of vascular involvement in younger (< 50 years old) and older (≥ 50 years old) patients were similar, with ratios of 16.58% (122/736) and 23.53% (44/187) respectively. However, in females, younger patients were less likely to have vascular involvement than were older patients (11.43% vs. 20% p = 0.0328, OR: 0.5161, 95% CI: 0.2874 to 0.912). Aneurysm or pseudoaneurysm was diagnosed in 1.84% (17/923) patients, mostly in male patients (p < 0.05, OR: 3.221, 95% CI: 1.097 to 9.112). Twenty vascular BD patients were followed up, and the age at BD diagnosis was 33.23 ± 11.56 year. This did not differ statistically with their age at vascular involvement (36.15 ± 9.52 years). Ages of vascular BD patients did not differ significantly from those of mucocutaneous BD patients (n = 143) in both males and females.

Conclusion

Vascular BD, including lethal types of aneurysm is more likely to occur in male patients. The female patients has a similar incidence rate with the males in their postmenopausal age. There was no evidence of progression course from mucocutaneous BD to vascular involvement.