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Cutaneous and Extracutaneous Manifestations of Behçet's Disease Linked to Its Disease Activity and Prognosis
Preeyachat Limtong1, Kumutnart Chanprapaph1, Vasanop Vachiramon1
1Division of Dermatology, Department of Internal Medicine, Faculty of Medicine, Ramathibodi Hospital, Mahidol University, Bangkok, Thailand.
Insights
Minor oral ulcer location in Behçet's disease can predict disease activity. Male gender and ocular issues are linked to worse outcomes in this vasculitis disorder.
Area of Science:
- Rheumatology
- Internal Medicine
- Dermatology
Background:
- Behçet's disease is a severe systemic vasculitis affecting arterial and venous systems.
- Cutaneous manifestations are common but their prognostic significance is unclear.
- Understanding these links is crucial for managing Behçet's disease.
Purpose of the Study:
- To investigate the relationship between cutaneous and extracutaneous manifestations of Behçet's disease.
- To determine the association with disease activity, morbidity, and mortality.
- To identify predictors of disease severity and prognosis.
Main Methods:
- Patients diagnosed using 1990 and 2014 international criteria for Behçet's disease.
- Clinical data, laboratory results, and prognosis were analyzed.
- Disease activity assessed using the 2006 Behçet's disease current activity form (BDCAF).
Main Results:
- Minor oral ulcers on buccal mucosa correlated with lower BDCAF scores (p=0.003).
- Minor oral ulcers on tonsils correlated with higher BDCAF scores (p=0.024).
- Male gender and ocular manifestations were associated with increased corticosteroid use, hospitalization, and morbidity.
Conclusions:
- Specific locations of minor oral ulcers can indicate Behçet's disease activity levels.
- Male patients and those with ocular involvement face poorer prognoses.
- These findings aid in predicting Behçet's disease outcomes and guiding treatment.
Background:
Behçet's disease is a potentially fatal vasculitis disorder involving vessels in both the arterial and venous systems. Cutaneous manifestation is the most common sign in Behçet's disease, but its relation to disease activity and prognosis is lacking.
Objective:
Our study aims to determine the relationship between cutaneous, extracutaneous manifestations with Behçet's disease activity, morbidity, and mortality.
Materials And Methods:
Patients diagnosed with Behçet's disease were identified by using the 1990 International Study Group for Behçet's disease criteria and/or the 2014 international criteria for Behçet's disease. Data regarding patients' clinical manifestations, laboratory results, disease activity, and prognosis were retrieved and analyzed. Disease activity was evaluated using the 2006 Behçet's disease current activity form (BDCAF).
Results:
Of 119 patients, the mean age at diagnosis was 35.7 ± 10.7 years and 60% were female. Median disease duration was 96 months (IQR 48-168). Minor oral ulcer at the buccal mucosa was associated with lower median BDCAF score (p = 0.003), whereas minor oral ulcer at the tonsil was related to higher BDCAF score (p = 0.024). Male gender was associated with higher cumulative dose of corticosteroids (p = 0.003) and hospitalization (OR = 2.89 (95% CI [1.10, 7.57]), p = 0.031). Ocular manifestations were related to higher dose of corticosteroids (p = 0.002) and morbidity (OR = 4.39 (95% CI [1.57, 12.29]), p = 0.005).
Conclusion:
Our study suggests that different locations of minor oral ulcers help predict disease activity. Male gender and the presence of ocular manifestations resulted in less favorable outcomes for Behçet's disease.
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