gms | German Medical Science

Introduction: Adamantiades-Behçet’s disease (ABD) is a rare chronic, multisystem, inflammatory disease, characterized by chronic vasculitis and a relapsing-remitting course of mucocutaneous and ocular lesions. Oral ulcers are the most common manifestations and significantly impair quality of life [1]. Current epidemiological data on ABD in Germany are lacking. This study assessed ABD incidence and prevalence in Germany, associated comorbidities, and current treatment patterns.

Methods: This retrospective cohort study was based on the InGef research database, with healthcare claims from approximately 4 million patients. Patients (≥18 years) were included based on inpatient or outpatient diagnoses of ABD (ICD-10 code M35.2), in combination with prescriptions of predefined disease-related drugs (azathioprine, prednisone, prednisolone, ciclosporin A, colchicine, interferon alfa-2a, thalidomide, adalimumab, certolizumab pegol, etanercept, golimumab, or infliximab). Data on prevalence, incidence, gender and age distribution, comorbidities, and pharmacological treatment in 2016, 2017, and 2018 were analysed, with incident disease confirmed by a 3-year baseline period. Prevalence and incidence data were projected (age and gender adjusted) to the statutory insured population of about 73 million in Germany, with a 95% confidence interval (CI).

Results: A total of 122, 127, and 150 patients were identified in 2016, 2017, and 2018, respectively. Respective prevalence rates per 100,000 patients (95% CI) were 3.9 (3.2-5.8), 4.1 (3.4-5.9), and 4.7 (4.0-6.4); respective incidence rates were 0.5 (0.2-2.3), 0.5 (0.2-2.2), and 0.6 (0.3-2.1). Females with prevalent disease were on average slightly older (52.1, 52.0, and 52.8 in 2016, 2017, and 2018) than males (48.1, 49.4, 50.7), with overall prevalent disease being highest in ages 40-59. Prednisolone and colchicine were the most commonly prescribed disease-related drugs among prevalent patients (Figure 1 [Fig. 1]); the most common comorbidities in these patients were dorsalgia, disorders of refraction and accommodation, and hypertension (Figure 2 [Fig. 2]).

Conclusion: This study provides new insights into the epidemiology of ABD in Germany using current administrative claims data. Prevalence increased from 2016-2018 and is about four times higher than in the German Registry of Adamantiades-Behçet Disease with a prevalence of 1.1:100,0002, but ABD remains a rare disease. Differences in prevalence may be attributable to differing data sources.

Disclosures: This study was sponsored by Amgen GmbH which provided funding to WIG2 GmbH for the execution of the study and medical writing support. InGef – Institute for Applied Health Research Berlin acted as subcontractor and received funding from WIG2 GmbH for the execution of the study.

C.C. Zouboulis declares no conflicts of interest with respect to the research, authorship, and/or publication of this article. In general, C.C. Zouboulis reports a speaking honorarium from Amgen and honoraria from Amgen and Celgene to the Dessau Municipal Hospital for lectures and his participation in a relevant clinical trial; A. Stein and JP. Medelnik are employees of Amgen GmbH; R. Heinrich, J. Borchert, J. Diesing and T. Schönfelder are employees of WIG2 GmbH; W. Galetzka was employed by InGef – Institute for Applied Health Research Berlin during conduction of the analyses.