Clinical course of COVID-19 infections in patients with Behçet's disease in The Netherlands.

OBJECTIVES: The aim of this study is to investigate the cumulative incidence and the severity of COVID-19 infections in patients with Behçet's disease. METHODS: A retrospective cohort study of patients with Behçet's disease was conducted. We obtained the data systematically from electronic patient files and through telephone interviews between February 2020 and May 1, 2021. Main outcomes were COVID-19 infection, disease duration, hospitalisation, intensive care admission and mortality. Secondary outcome was adherence to quarantine measures as recommended by the government. RESULTS: 185 Behçet's disease patients were included (mean age 42.2 years, 54% female); 58% of the patients were receiving colchicine, 30% anti-TNFα, 16% azathioprine and 8% systemic steroids. 30 patients (16.2%) were positive for COVID-19. Within our cohort, the cumulative incidence of COVID-19 was therefore 16.2% (95% CI 11.2-22.3%), which is significantly increased when compared to the general Dutch population (8.7% (95% CI 8.72-8.73%)) (p < 0.001). Four out of 30 (13%) patients were admitted to the hospital. There was no COVID-19 related mortality observed. Patients adhered to government measures; except in the period between the 1st of June and the 28th of September, this cohort received more visitors than in period 1 and 3. CONCLUSIONS: In this cohort, Behçet's disease patients have a higher risk for COVID-19 infection, without an increase of virus-related mortality. The course of COVID-19 disease in this cohort is relatively mild, with a lower admission rate than expected of patients using immunosuppressive medication.

Behçet's syndrome: one year in review 2022.

This review highlights publications on different aspects of Behçet's syndrome (BS) that appeared in 2021 and provides a critical view. These publications include works on the epidemiology of BS across different continents, newly developed instruments to assess damage in BS, studies highlighting the immunopathogenesis, genetics and epigenetic factors, histopathology of the pathergy lesion, clinical and imaging aspects of vascular involvement, and safety and efficacy of therapeutic agents including tocilizumab, apremilast and direct oral anticoagulants.

Adamantiades-Behçet disease: from clinical heterogeneity to diagnosis during the COVID-19 pandemic.

Adamantiades-Behçet disease (ABD) is a systemic disease with vasculitis, characterized by recurrent oral aphthosis and ocular, cutaneous, articular, vascular, cardiopulmonary manifestations and it is mainly found in the territories of the antique "silk road". ABD pathogenesis remains unknown although genetic, infectious and environmental factors seem to be implicated in the development of the disease, which is considered an auto-inflammatory condition. COVID-19 infection can present some symptoms, in particular at the level of oral and pulmonary mucosa, which require a differential diagnosis with ABD. Furthermore, the immunological alterations of this disease, and the drugs used for its treatment could influence the infection by COVID-19, and its clinical evolution. Nevertheless, vaccination anti-COVID-19 is recommended in ABD patients. The most commonly used diagnostic criteria for ABD are those established in 2014 by the International Team for the Revision of the International Criteria for BD (ITR-ICBD). Furthermore, criteria for disease severity according to the Overall Damage Index of Behçet's Syndrome (BODI) have recently been proposed in order to quantify the severity of the disease as well as the evolution during follow-up. In ABD patients it is mandatory to investigate on the presence of active/latent tuberculosis, because of the common organ involvement, such as eyes and bowel. ABD has a high morbidity and low mortality, sometimes linked to the rupture of an arterial aneurysm and/or neurological complications. This article is based on a general review on ABD ranging from the history of ABD to possible causes and clinical manifestations. A specific section has been dedicated to the COVID-19 pandemic.

Prevalence and clinical course of SARS-CoV-2 infection in patients with Behçet's syndrome.

OBJECTIVES: We aimed to assess the prevalence of SARS-CoV-2 infection among Behçet's syndrome (BS) patients, evaluating the possible association between demographic and clinical features and the risk of infection. Moreover, we aimed to evaluate the possible association between BS disease activity and treatment, and the risk of SARS-CoV-2 infection. METHODS: A survey was conducted on BS patients followed at the Behçet's Centre of the Careggi University Hospital, Florence, Italy. We further evaluated the possible association between BS disease activity and treatment, and the risk of SARS-CoV-2 infection. RESULTS: Out of 335 BS patients contacted, fourteen cases of SARS-CoV-2 were identified between April 1st, 2020 and February 9th, 2021, suggesting a prevalence of SARS-CoV-2 infection among BS patients of 4.2%, in line with the data of the general population in Italy (4.4%). When comparing clinical features between SARS-CoV-2 cases and matched SARS-CoV-2 negative BS patients, we found that the presence of different disease manifestations did not significantly differ between the two groups. SARS-CoV-2 cases and controls were also comparable in terms of immunosuppressive therapy, with the only exception of corticosteroids (71.4% vs. 35.7%, p=0.030), whose daily dose was significantly higher in cases than controls [5mg/day (IQR 0-10,) vs. 0 mg/day (IQR 0-5), p=0.005], suggesting that the right timing of usage and the more appropriate dosage of corticosteroid are a key question for the better management of these patients. CONCLUSIONS: Based on our results, patients with BS do not seem to be at a greater risk of SARS-CoV-2 infection or severe complications compared with the general population.

Clinical course of Covid-19 in a cohort of patients with Behçet disease.

OBJECTIVE: The implications of Covid-19 in patients with Behçet's disease (BD) are unknown. Patients with BD usually take long-term therapy with therapeutic agents that have been tested in Covid-19 patients. We aimed to assess the prevalence of Covid-19 in a cohort of patients with BD and investigate whether those patients with a long-term treatment with colchicine, tumor necrosis factor inhibitors (TNFi) or glucocorticoids are at reduced or increased prevalence of Covid-19 related clinical outcomes. METHODS: A retrospective study was conducted among 244 patients with BD (86.1% females; mean age 43.95±11.11 years). Each participant completed an online questionnaire regarding demographics, medical conditions, dispensed colchicine, TNFi or oral glucocorticoids, Covid-19 infection, clinical symptoms and recovery. RESULTS: The prevalence of Covid-19 infection was 14.75%. Regarding dose of colchicine, the presence of ageusia was lower in patients taking 0.5mg/day of colchicine compared to those taking 1.5mg/day (p=0.021). The prevalence of dyspnea was significantly higher in patients taking TNFi compared with those without therapy (p=0.032). With regards to oral glucocorticoids, no significant differences were found. CONCLUSIONS: The prevalence of Covid-19 among patients with BD seems to be higher than that among the general population in Spain. Continuous TNFi therapy might increase the prevalence of worse clinical outcomes such as dyspnea; oral glucocorticoids and colchicine apparently provided no protection against the Covid-19 related clinical outcomes of patients with BD.

The incidence, clinical characteristics, and outcome of COVID-19 in a prospectively followed cohort of patients with Behçet's syndrome.

Initial case series of small number of patients at the beginning of the pandemic reported a rather guarded prognosis for Behçet's syndrome (BS) patients infected with SARS-CoV-2. In this prospective study, we describe the incidence, clinical characteristics, disease course, management, and outcome in a large cohort of BS patients with laboratory-confirmed infection of SARS-CoV-2. We defined a cohort of 1047 registered BS patients who were aged between 16 and 60 years and seen routinely before the pandemic at the multidisciplinary outpatient clinic. We followed prospectively this cohort from beginning of April 2020 until the end of April 2021. During 13 months of follow-up, of the 1047 (599 M/448 F) patients, 592 (56.5%) were tested for SARS-CoV-2 PCR at least once and 215 (20.5%; 95% CI 0.18-0.23) were tested positive. We observed 2 peaks which took place in December 2020 and April 2021. Of the 215 PCR positive patients, complete information was available in 214. Of these 214, 14 (6.5%) were asymptomatic for COVID-19. In the remaining, the most common symptoms were anosmia, fatigue, fever, arthralgia, and headache. A total of 40 (18.7%) had lung involvement, 25 (11.7%) were hospitalized, 1 was admitted to the intensive care unit while none died. Favipiravir was the most prescribed drug (74.3%), followed by colchicine (40.2%), and hydroxychloroquine (20.1%) in the treatment of COVID-19. After COVID-19, 5 patients (2.3%) were given supplemental O2 and 31 (14.5%) antiaggregant or anticoagulants. During COVID-19, of the 214 PCR positive patients, 116 (54.2%) decreased the dose of their immunosuppressives or stopped taking completely; 36 (16.8%) experienced a BS flare which was mostly oral ulcers (10.3%). None of the patients reported a thrombotic event. A total of 93 (43.5%) patients reported BS flares after a median 45 days of COVID-19 infection and this was found to be significantly associated with immunosuppressive drug discontinuation. Multiple regression analysis adjusted for age and gender indicated that smoking and using interferon-alpha decreased the likelihood of getting COVID-19. The incidence and severity of COVID-19 did not differ between those who were using colchicine or not. The cumulative incidence of COVID-19 in this prospectively followed cohort of BS patients was almost two folds of that estimated for the general population living in Istanbul, Turkey, however, the clinical outcome of COVID-19 was not severe and there was no mortality. The protective effect of smoking and interferon deserves further investigation. On the other hand, colchicine did not have any positive or negative effect against COVID-19. Significant number of patients flared after COVID-19, however, this was significantly associated with immunosuppressive discontinuation during the infection. Contrary to our previous observations, COVID-19 did not seem to exacerbate thrombotic events during or after the infection.

COVID-19 in patients with Behçet's disease: Outcomes and rate of Behçet's exacerbations in a retrospective cohort.

OBJECTIVES: The aim of this study is to investigate the outcomes of coronavirus disease 2019 (COVID-19) in our cohort of Behçet's disease (BD) patients and to reveal the rate of BD exacerbations due to COVID-19. METHODS: Patients who have been followed with a diagnosis of BD were retrospectively investigated for a positive severe acute respiratory syndrome-coronavirus 2 polymerized chain reaction (PCR) test. Data regarding demographics, clinical features and COVID-19 outcomes were collected from medical records for patients with a positive PCR. PCR-positive patients were reached via phone numbers, and 'Behçet's Disease Current Activity Form' (BDCAF) scores for pre- and post-COVID-19 BD symptoms were calculated. RESULTS: Out of a total 648 BD patients, 59 were detected to have a positive PCR test. Three of the 59 patients (5.0%) were found to be hospitalized, none of them was admitted to the ICU or died. An increasing trend in the frequency of comorbid diseases and older age was observed in hospitalized patients. 32.2% of BD patients suffered from exacerbation of at least one symptom related to BD. CONCLUSIONS: We observed no ICU admission or mortality with COVID-19 in our BD patient cohort. A substantial number of patients suffered from exacerbation of BD symptoms.

Coronavirus disease 2019 in patients with Behcet's disease: a report of 59 cases in Iran.

OBJECTIVES: To present the clinical characteristics, disease course, management, and outcomes of COVID-19 infection in patients with Behcet's disease (BD). METHODS: In this retrospective cohort study, we retrieved BD patients with definite diagnosis of COVID-19 infection. Demographic data, comorbidities, features related both to BD and COVID-19 infection, treatments, and outcomes were collected. Comparisons between patients with or without hospitalization were performed. All statistical analyzes were performed using SPSS version 25. We considered p < 0.05 statistically significant. RESULTS: We identified 61 episodes of COVID-19 infection in 59 BD patients. The prevalence was 0.69%. The median age was 45 years (IQR = 20), and the median disease duration was 162 months (IQR = 195). BD features were similar except for higher rate of arterial involvement and positive pathergy test in infected patients. Thirty-five episodes (62.5%) happened in non-active patients; 39% had a comorbid disease. COVID manifestations were the same as the general population. Flu-like symptoms were the most common (85%), followed by fever (66%), ageusia/anosmia (56%), headache (51%), and pulmonary involvement (48%). There was no change in BD symptoms in 74%. Fifteen patients (25.4%) were hospitalized, and one patient (1.7%) died. Receiving glucocorticoids (p < 0.03) and cytotoxic drugs (p < 0.02) were associated with an increased rate of hospitalization. CONCLUSION: The incidence of COVID-19 infection in BD patients was not higher than general population in Iran. They showed milder form of disease with lower morbidity and mortality rate. Most were on immunosuppressive drugs, or had a comorbidity apart from BD. No significant effect on BD course was shown. Key Points • The incidence of COVID-19 infection in patients with Behcet's disease is not higher. • They showed milder form of infection with lower morbidity and mortality rate. • No significant effect on Behcet's disease course was shown with COVID19 infection. • BD patients can be managed according to the guidelines used for general population.

Treatment considerations for Behçet disease in the era of COVID-19: A narrative review.

COVID-19 is a multisystem disease caused by severe acute respiratory syndrome coronavirus 2. It has been declared a pandemic by the World Health Organization in March 2020 and the outbreak still keeps its impacts worldwide. Behçet disease (BD) is a multi-systemic vasculitis involving the skin, mucosa, eyes, joints, nervous system, cardiovascular system, and gastrointestinal system. The precise etiopathogenesis of the disorder is unknown but autoimmunity is believed to play a key role. A considerable part of patients with BD are susceptible to immunosuppression and are more predisposed to infections than healthy individuals. Hence, the protection and control measures for patients with BD against the COVID-19 are of the utmost significance. Given the requirement to balance proper treatment of BD with the smallest risk of COVID-19 associated mortality and morbidity, we aimed to review the management of BD in the era of the pandemic with a special focus on treatment considerations. According to current expert recommendations, there is no reason to discontinue topical treatments, colchicine, and nonsteroidal antiinflammatory drugs. Systemic steroids can be used at the lowest possible dose if needed. Ongoing treatments can be continued unchanged in patients with no suspected or confirmed COVID-19. In cases with COVID-19 symptoms, immunosuppressive and biological agents can be temporarily stopped but the decision should be made on a case by case basis. Considering their potential beneficial effects on the course of COVID-19, colchicine, pentoxifylline, and dapsone can be considered as safe treatment options in BD.