Aims
Behçet’s disease (BD) is a multisystem inflammatory vasculitis presenting with recurrent mucocutaneous ulcers, ocular inflammation, and skin lesions. Although gastrointestinal symptoms may appear in a substantial number of patients, true intestinal BD is relatively uncommon. This study assessed the clinical characteristics and treatment patterns of intestinal BD at a tertiary center in Korea.
Methods
Medical records of 327 patients who fulfilled the ISG or ICBD criteria for BD and were followed between November 2008 and September 2023 were reviewed. Among them, 261 had BD without gastrointestinal involvement, while 66 patients (20.2%) were diagnosed with intestinal BD. Demographic data, clinical symptoms, laboratory markers, imaging results, and treatment modalities were compared between the two groups.
Results
Intestinal BD accounted for 20.2% of all BD patients. Abdominal pain (74.2% vs. 10.0%, p < 0.001) and gastrointestinal bleeding (19.7% vs. 0.8%, p < 0.001) were significantly more frequent in intestinal BD, whereas uveitis, skin lesions, and arthritis were more common in non-intestinal BD. CRP levels were higher in intestinal BD (2.35 vs. 1.23 mg/dL, p < 0.001), while HLA-B51 positivity was markedly lower (13.0% vs. 50.4%, p = 0.001). Treatments such as 5-ASA, azathioprine, and adalimumab were used more often in intestinal BD. Hospitalization (54.5% vs. 8.4%) and surgery rates (16.7% vs. 0.8%) were also significantly higher. Multivariate analysis identified abdominal pain (adjusted OR 54.1) and HLA-B51 negativity (adjusted OR 0.20) as independent predictors of intestinal involvement.
Table 1. Independent variables associated with the likelihood of intestinal BD in univariate logistic regression analysis
|
Covariates |
OR |
OR 95% CI. |
P-value |
|
Age, yr |
1.022 |
1.000-1.044 |
0.05 |
|
Sex, M/F |
0.632 |
0.365-1.092 |
0.100 |
|
Clinical manifestation |
|||
|
Oral ulcers |
0.800 |
0.383-1.671 |
0.553 |
|
Genital ulcers |
0.738 |
0.429-1.269 |
0.273 |
|
Eye(Uveitis) |
0.207 |
0.072-0.592 |
0.003 |
|
Skin lesion |
0.389 |
0.217-0.699 |
0.002 |
|
Arthritis |
0.397 |
0.172-0.916 |
0.030 |
|
Abdominal pain |
26.052 |
13.140-51.652 |
0.000 |
|
GI Bleeding |
31.764 |
6.963-144.905 |
0.000 |
|
Perforation |
8.125 |
0.725-91.007 |
0.089 |
|
Laboratory findings |
|||
|
WBC, /mm3 |
1.000 |
1.000-1.000 |
0.889 |
|
Hemoglobin, g/dL |
0.845 |
0.712-1.001 |
0.052 |
|
Albumin, g/dL |
0.552 |
0.289-1.056 |
0.073 |
|
Total protein, g/dL |
1.244 |
0.806-1.918 |
0.324 |
|
CRP, mg/dL |
1.065 |
1.000-1.134 |
0.052 |
|
B_51 |
0.147 |
0.041-0.524 |
0.003 |
Table 2. Independent variables associated with the likelihood of intestinal BD in multivariate logistic regression analysis
|
Covariates |
Adjusted OR(Exp(B)) |
95% CI. |
P-value |
|
Abdominal pain |
54.1 |
13.4 – 219.4 |
<0.001 |
|
HLA-B51 |
0.20 |
0.04 – 0.94 |
0.041 |
Conclusions
Intestinal BD shows a distinct phenotype characterized by predominant gastrointestinal symptoms, higher inflammatory activity, and reduced HLA-B51 positivity. Abdominal pain and HLA-B51 negativity may help early diagnostic suspicion. More intensive therapy and higher complication rates highlight the need for timely recognition and individualized management.