Background/Purpose: Behçet disease (BD) is a multisystem inflammatory disorder characterized by recurrent manifestations in mucocutaneous tissues, eyes, joints, blood vessels, intestines and brain. Since there is no pathognomonic clinical and laboratory finding, diagnosis of BD relies on constellation of a group of manifestations. Recurrent oral aphthous ulcers (ROU) are the commonest manifestation, and widely used classification criteria require ROU in all patients. However, some patients may not have ROU at disease onset, which may cause a challenge and delay in the diagnosis of BD. This study aimed to investigate the disease course and appearance of the manifestations in those patients with or without ROU at the disease onset.

Methods: The study group consisted of 570 patients, who fulfilled the International Study Group (ISG) diagnostic criteria and followed between 1976 and 2016. All patients interviewed personally for their disease course and their medical records were investigated retrospectively. Differences in the disease course were analyzed according to the type of manifestations at the disease onset, sequence of appearance of other manifestations, and their smoking status.

Results: Non-aphthous beginning (NAB) at the disease onset was found in 13.6% of patients, and it was more frequent among smokers compared to non-smokers (18.4% vs 6.8% in males, p=0.019; 22.9% vs 9.7% in females, p=0.038). Frequency of uveitis (54.1% vs 30.2%, p<0.001) and cardiovascular involvement (39.3% vs 24.2%, p=0.019) was higher in patients with NAB compared to the patients with ROU at onset. Both NAB group and ROU at onset group fulfilled the ISG diagnostic criteria within similar disease duration (median 48 vs 54 months). However, a 3-month delay in diagnosis was noted after the fulfillment of ISG criteria in patients with ROU at onset despite a 5.3-month delay in NAB group (p=0.003). Overall, the most frequent manifestations developing at the onset of BD were ROU, genital ulcers, and uveitis; and the latest manifestation during the course was pulmonary parenchymal involvement. Erythema nodosum-like lesions as initial findings were more frequent in females (11.3% vs 5.1%, p=0.024), and deep-vein thrombosis (DVT) was more frequent in males (4.6% vs 0.9%, p=0.04). NAB was identified as an independent risk factor for the development of uveitis (OR=2.06) and DVT (OR= 2.25) by logistic regression analysis. None of the BD patients had arterial aneurysms or thrombosis, pulmonary parenchymal, gastrointestinal or genitourinary involvement as initial manifestations.

Conclusion: This retrospective study revealed that 13.6% of BD patients may not have ROU at the disease onset, and those patients with NAB may have different features in their disease course with a tendency for higher risk of uveitis and DVT. Smoking may also have a role in the increased frequency of NAB in BD patients.

« Back to 2018 ACR/ARHP Annual Meeting

ACR Meeting Abstracts - https://acrabstracts.org/abstract/non-aphthous-beginning-as-an-independent-risk-factor-for-the-course-of-behcets-disease/