AB0536 EFFECT OF HYDROXYCHLOROQUINE TREATMENT IN MUCOCUTANEOUS MANIFESTATIONS IN PATIENTS WITH BEHÇET’S SYNDROME
Authors: F. Kerstens, S. Mohamed, I. Visman, F. Turkstra, C. Swearingen, Y. Yazici
Journal: Annals of the Rheumatic Diseases (2020)

Background: Behçet syndrome (BS) is a rare multisystemic vasculitis, most commonly seen in regions along the ancient Silk Road. It runs a relapsing and remitting course. Mucocutaneous disease, consisting of oral ulcers, genital ulcers and skin lesions is often reported. EULAR recommendations advise colchicine and topical agents for the treatment of these lesions. 1 Not all patients respond adequately, thus, it is important to explore alternative treatment options. Objectives: To study the efficacy of hydroxychloroquine (HCQ) 400 mg daily in patients with mucocutaneous BS. Methods: Data on all patients who presented at the outpatient Behçet clinic in New York were recorded. Patients with a first prescription with HCQ and a follow-up of 3 months (range: 2.75-41.2 months) were included. Patient reported outcomes BSAS and RAPID3 were used to evaluate the effect of HCQ. Results of all patients and of International Study Group (ISG) positive patients were analyzed separately using Wilcoxon rank tests. Results: We included 94 patients with a first prescription of HCQ. 72 patients (76.6%) fulfilled ISG criteria. Mean age was 36.1 years (SD 12.5), 76 patients (80.9%) were female and 11 patients (11.7%) were from Silk Road countries. Mean duration until follow-up was 6.5 months (SD 5.7). Median BSAS scores in ISG+ patients at baseline did not differ significantly from ISG- patients, except for skin lesions (5.0 in ISG+ vs. 0.5 in ISG- p=0.005). BSAS scores at follow-up did not differ significantly (ISG+ vs. ISG-). Median BSAS scores were significantly lower at follow-up compared to baseline for oral ulcers (p=0.010), skin lesions (p=0.018) and overall activity (p=0.019). Regarding genital ulcers there was no significant result, due to only 37 patients reporting complaints of genital ulcers. Performing these analyses in ISG+ patients only did not change these results, except for BSAS overall activity, which lost significance (p=0.057). RAPID3 scores were not statistically different between baseline and follow-up (9.67 vs. 8.75, p=0.145), nor were its separate components function (p=0.67 vs. 0.67, 0.713), pain (4.0 vs. 4.0, p=0.157) and patient global (5.0 vs. 4.5, p=0.095). The majority of patients used prednisone at baseline (58.5%) and at follow-up (57.4%). In 15 patients, prednisone was stopped at follow up, in 13 patients it was started. Table 1. Median BSAS scores of patients treated with HCQ. Baseline (median, IQR ) Follow-up 3 months (median, IQR ) P-values All patients (n=94 ) Oral ulcers 5.0 (2.00-7.88) 3.0 (1.00-6.00) 0.010 Genital ulcers 0.0 (0.00-3.88) 0.0 (0.00-3.00) 0.371 Skin lesions 5.0 (1.25-7.00) 2.5 (0.00-7.00) 0.018 Overall activity 5.5 (4.00-8.00) 5.0 (2.00-7.25) 0.019 ISG+ patients (n=72 ) Oral ulcers 5.25 (2.00-7.63) 3.25 (1.00-6.00) 0.007 Genital ulcers 0.5 (0.00-4.00) 0.0 (0.00-3.00) 0.684 Skin lesions 5.0 (2.00-7.13) 3.0 (0.00-7.00) 0.015 Overall activity 6.0 (4.00-8.00) 5.0 (2.00-7.50) 0.057 Conclusion: HCQ improves median BSAS scores for oral ulcers, skin lesions and overall activity at 3 months follow-up compared to baseline. These results were similar in ISG+ patients (except for overall activity). Additional research is needed to assess the effect of HCQ in more patients and over multiple time points. References: [1]Hatemi G et al. 2018 update of the EULAR recommendations for the management of Behçet’s syndrome. Ann Rheum Dis 2019;77:808-818 Disclosure of Interests: Floor Kerstens: None declared, Shreen Mohamed: None declared, Ingrid Visman: None declared, Franktien Turkstra: None declared, Christopher Swearingen: None declared, Yusuf Yazici Consultant of: BMS, Celgene Corporation, Genentech, Sanofi – consultant, Consultant of: BMS, Celgene Corporation, Genentech, Sanofi – consultant