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

Abstract

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 O 2 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.

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

“…In line with our findings, a Turkish prospective cohort study in patients with BD found an increased infection rate compared to the general Turkish population (26). Ozcifci et al showed a cumulative incidence of COVID-19 in the BD patient group of 20.5%, in the Turkish general population the cumulative incidence of COVID-19 was 5.77% (26). Four patients, one male and three female, in our cohort were admitted because of severe symptomatic COV-ID-19 infection.…”

“…In Table IV we present an overview of the current literature on BD and COV-ID-19. We have added 4 studies which also used a sample survey (23)(24)(25)(26). In total 2014 BD patients were discussed in these studies, 313 patients were tested positive.…”

Safety of SARS-CoV-2 vaccination in patients with Behcet’s syndrome and familial Mediterranean fever: a cross-sectional comparative study on the effects of M-RNA based and inactivated vaccine

“…Skin hypersensitivity and the induction of systemic symptoms in response to streptococcal antigens, as well as significant inflammatory reactions to polysaccharide pneumococcal vaccine in BS are well known [ 38 – 41 ]. Moreover, we observed very recently that 36 of 214 (16.8%) patients with BS experienced a flare during COVID-19 infection [ 42 ].…”

Current utilization of interferon alpha for the treatment of coronavirus disease 2019: A comprehensive review

“…The use of IFNα inhalation was reported in patients with various comorbidities and conditions, including patients with atopic dermatitis, [38] allergic rhinitis, [38] bronchiolitis, [25] chronic obstructive pulmonary disease, [84] , [162] tuberculosis, [55] cardiovascular disease, [22] hypertension, [22] , [84] , [150] , [162] diabetes, [32] , [39] hyperlipidemia, [150] obesity, [126] metabolic syndrome, [192] chronic hepatitis B infection, [76] , [153] chronic kidney disease, [22] , [70] malignancy, [163] liver transplant, [50] kidney transplant, [70] , [172] and pregnancy. [19] The use of subcutaneous injection of IFNα was reported in patients with diabetes, [42] hypertension, [42] , [44] hyperlipidemia, 42 chronic hepatitis B infection, [140] primary myelofibrosis with macrocytic anemia, [44] dementia, [44] chronic kidney disease, [44] Behcet's disease, [190] and osteoporosis. [44] However, whether IFNα treatment is safe and efficacious in COVID-19 patients with these comorbidities requires further clinical studies.…”

“…Another piece of evidence came from the observation of patients with Behcet’s syndrome in Turkey. [190] Behcet’s syndrome is a rare inflammatory disorder that results in a wide range of signs and symptoms and can be treated with IFNα. Using univariate logistic regression, the researchers analyzed data from 1,037 patients and found that patients on IFNα treatment had an odds ratio of 0.12, meaning that they were 88% less likely to contract COVID-19 disease.…”