Natural history of Behçet’s disease focusing on remission of oral ulcers

Abstract

Objectives To describe the long-term clinical course of each manifestation of Behçet’s disease (BD) and clarify factors involved in oral ulcer (OU) remission using clinical information of BD patients. Methods We retrospectively studied 155 BD patients visiting our hospital (1989–2020). We defined remission criteria for each manifestation and examined long-term clinical changes. Classification and regression trees and multivariable analyses were performed to investigate OU prognostic factors; hazard ratios were used to assign scores to prognostic factors deemed significant (OU prognosis score: OuP score). Risk stratification was examined by dividing the OuP scores into four stages. Results OUs appeared earliest, with the slowest decline in prevalence observed post-BD diagnosis. OU presence was the most common factor inhibiting complete remission. Young age at OU onset, never smoker, presence of genital ulcers, positive pathergy test, no usage of tumour necrosis factor inhibitors or of immunosuppressants, and long-term non-treatment or symptomatic treatment for OUs were poor OU prognostic factors. Based on multivariable analysis, the area under the curve of the OuP score to predict OU prognosis was 0.678. Conclusions Remission criteria for each symptom clarified that OU had the greatest impact on complete BD remission. Faster OU remission was associated with earlier OU therapeutic intervention other than symptomatic treatment.

Treat to target in Behcet's disease: Should we follow the paradigm of other systemic rheumatic diseases?

“…One of the characteristics of BD is its fluctuating activity, which is more obvious in mucocutaneous manifestations [30,[36][37][38]. Oral ulcer activity may run in parallel with musculoskeletal [30] and genital ulcer activity [37], as well as with psycho-social well-being [33], all of them affecting the functionality and quality of life [30].…”

“…One of the characteristics of BD is its fluctuating activity, which is more obvious in mucocutaneous manifestations [30,[36][37][38]. Oral ulcer activity may run in parallel with musculoskeletal [30] and genital ulcer activity [37], as well as with psycho-social well-being [33], all of them affecting the functionality and quality of life [30]. Moreover, increased mucocutaneous activity, as the activity of oral ulcers, may be associated with new major organ involvement in young female BD patients with systemic involvement [39].…”

“…Oral aphthous ulcers are often the sole manifestation of the disease, denoting some clinical activity in many BD patients without any other findings [35,37,[42][43][44] and are managed with mild agents such as topical CSs or colchicine, to prevent side-effects. In some of these cases, aphthous ulcer-free state cannot be achievable despite the remission of all serious disease manifestations, and using a terminology like "low or minimal disease activity" may be considered to define the partial response of mucocutaneous manifestations to therapy in clinical practice [35,36].…”

“…However, the definitions of "low or minimal disease activity" in BD is not yet clear. Along the same lines, definitions of complete remission, partial remission, relapse, refractory disease [37,45,46] and irreversible functional and anatomical damage attributed to BD [47,48] should also be clarified.…”

Behçet's yndrome: one year in review 2023

“…smoker, presence of genital ulcers, positive pathergy test and not using disease modifying anti-rheumatic drugs were identified as factors that prevent OU remission (42). Pharyngeal scarring with stenosis is a rare complication in BS.…”