Naslov
Clinical phenotype of Behçet’s disease in Croatia: two case reports with review of literature

Autori
Ikić Matijašević, Marina ; Kilić, Paula ; Ikić, Lucija ; Gudelj Gračanin, Ana ; Ostojić, Vedran

Vrsta, podvrsta i kategorija rada
Sažeci sa skupova, sažetak, stručni

Izvornik
Reumatizam 69, Suppl 1 / - , 2022, 59-60

Skup
24. godišnji kongres Hrvatskoga reumatološkog društva s međunarodnim sudjelovanjem

Mjesto i datum
Rovinj, Hrvatska, 27.10.2022. - 30.10.2022

Vrsta sudjelovanja
Poster

Vrsta recenzije
Međunarodna recenzija

Ključne riječi
Behçet’s disease, Croatia, clinical phenotype

Sažetak
Recent findings suggest that aetiology of Behçet’s disease (BD) is between autoimmunity and autoinflammation. In common with other autoimmune diseases BD shares class I MHC (HLA-B51) association, but BD has mostly autoinflammatory clinical features. In BD different clinical phenotypes can be distinguished (Bettiol 2020). Prevalence in Croatia is unknown, and far as we know, only 6 BD case reports (CR) have been published for our population. Case 1. A 32-year- old-woman with a history of recurrent oral aphthae, migratory arthralgias, and acneiform lesions was referred after being diagnosed with bilateral retinal vasculitis (RV). Workup showed positive HLA B51. The ICBD criteria were fulfilled. After induction of 0.5 mg/kg/day of methylprednisolone (MP) and 2.5 mg/ kg/day of azathioprine (AZA) RV resolved. Case 2. A 59-year- old-woman has been suffering from BD for the past 20 years. She was treated by her rheumatologist with NSAR, topical and systemic MP in exacerbations of arthritis and uveitis. Now, after first examination in our clinic, colchicine was administered due to orogenital aphthae, erythema nodosum and arthralgias with a good clinical response. She is now without signs of uveitis. Discussion: Until now, as far as we know, a total of 6 CR (Tekavec 2015, Boban 2016, Barešić 2018, Borić 2019, Krečak 2020, Kardum 2021) and no research on BD were published in last 10 years for our population. Analysing these 8 patients in total, we can conclude that in Croatia dominates parenchymal neurological/ocular, followed by peripheral vascular phenotype. Recommended therapy is high dose of MP and AZA for both ocular and parenchymal manifestations and anti-TNFα in case of severe ocular and parenchymal CNS involvements. This recommendation is in accordance with therapy used in published CR with a good response. Often seen delay in BD diagnosis is due to lack of pathognomonic clinical findings, specific laboratory test and the small number of BD patients. It would be useful to establish BD registry in Croatia because tailoring the treatment on patient’s specific phenotype which is a consequence of the genetic background and the influence of external factors, rather than on single disease manifestation, could be a valid strategy for a personalized therapeutic approach to BD. Also, the registry would enable earlier detection of the disease.

Izvorni jezik
Engleski

Znanstvena područja
Kliničke medicinske znanosti

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