{"id":327643,"date":"2021-10-19T19:00:12","date_gmt":"2021-10-19T17:00:12","guid":{"rendered":"https:\/\/medizinonline.com\/oral-ulcers-in-behcets-disease-apremilast-as-a-game-changer\/"},"modified":"2021-10-19T19:00:12","modified_gmt":"2021-10-19T17:00:12","slug":"oral-ulcers-in-behcets-disease-apremilast-as-a-game-changer","status":"publish","type":"post","link":"https:\/\/medizinonline.com\/en\/oral-ulcers-in-behcets-disease-apremilast-as-a-game-changer\/","title":{"rendered":"Oral ulcers in Beh\u00e7et’s disease – apremilast as a game changer"},"content":{"rendered":"

The phosphodiesterase 4 (PDE4) inhibitor, which has been approved for this indication since last year, persistently reduces both the number of oral ulcers and associated pain. Overall, apremilast treatment is associated with a significant improvement in the quality of life of patients with Beh\u00e7et’s syndrome. However, the differential diagnostic differentiation from aphthoses of other causes sometimes proves challenging. At this year’s SGDV annual meeting, two experts summarized the current state of knowledge and their clinical experience in an interdisciplinary symposium.<\/p>\n

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Beh\u00e7et’s disease (synonym: Beh\u00e7et’s syndrome) is a progressive vasculitis that can lead to severe multisystemic complications if left untreated. Almost all patients experience recurrent, painful oral ulcers during the course of the disease, which are associated with a reduced quality of life [1]. Prof. Dr. med. Peter Villiger, Rheumatologist, Monbijou Medical Center, Bern and Prof. Dr. med. Nikhil Yawalkar, Deputy Medical Director, Bern. Chief of Dermatology, Inselspital Bern, spoke at the SGDV Annual Congress on the epidemiology, clinical features and differential diagnosis of this rare disease and presented current facts on apremilast (Otezla\u00ae) – the first and so far only system therapy available in Switzerland for the treatment of oral aphthae in Beh\u00e7et’s disease(box<\/strong>) [2].* <\/p>\n

*Specifically related to the label of the drugs, as of June 2020. Full Indication Text: Otezla\u00ae is indicated for the treatment of persistent oral ulcers associated with Beh\u00e7et’s disease in adult patients who have had an inadequate response to topical therapy [2].<\/p>\n

M. Beh\u00e7et is widely distributed along the Silk Road<\/strong><\/p>\n

Beh\u00e7et’s disease is most common from the Mediterranean to China, Prof. Villiger said. For example, in Turkey, the prevalence is 370 per 100 000 inhabitants [1], while the corresponding values in Germany are 2.26-4.87 [3]. In Switzerland, the prevalence is estimated at 4.03 per 100 000 population [4]. This is the result of the extrapolation of a Bern study with 60 Beh\u00e7et patients. Among them, immigrants from high-prevalence countries were significantly more likely to be affected, at 19.54%. The causes of Beh\u00e7et’s disease are unknown. (Auto-)immunological and viral or bacterial triggers are thought to be involved and an association with the human leukocyte antigen B51 allele (HLAB51) is suspected.<\/p>\n

Oral ulcers as the most common manifestation of Beh\u00e7et’s syndrome.<\/strong><\/p>\n

In a cohort study of 428 patients, oral ulcers were present in 100% of study participants, 95% suffered from genital ulcers, and 83% were affected by papulopustular lesions. Because oral aphthosis can have numerous other causes, a “diagnostic delay” often occurs. In an analysis published in 2019, the diagnostic latency from the onset of oral ulcers to diagnostic classification as Beh\u00e7et’s disease was a median of eight years, Prof. Villiger noted [4]. In the International<\/strong> Criteria for Beh\u00e7et’s Syndrome (ICBD)<\/strong> classification system, the presence of the following symptoms is evaluated using a point system(Table 1<\/strong>): Oral aphthae, genital aphthae, skin lesions, ocular symptoms, neurological symptoms, vascular manifestations, positive pathergy test [5]. Prof. Yawalkar elaborated on some main symptoms [1]:<\/p>\n