Long-Term Outcomes of Pediatric Pars Planitis: Retrospective Cohort from a Single Tertiary Center in Turkey.

PURPOSE: To retrospectively analyze long-term outcomes of pediatric pars planitis (PP). METHODS: PP was defined as vitreal inflammation with snowbank or snowball formation in the absence of a related disease. Eighty-five eyes of 44 patients were included in this study. Demographic and clinical characteristics were obtained from medical records. RESULTS: Approximately 70% of the patients were males; the mean patient age was 10.4 ± 3.6 years at presentation, and the mean follow-up time was 42.8 ± 27.9 months. At presentation, the mean best corrected visual acuity (BCVA, logMAR) was 0.17 ± 0.27 in the right eyes and 0.27 ± 0.33 in the left eyes. Common symptoms included blurry vision (29 eyes, 65%), redness (17, 38%), pain (8, 18%), and floaters (5, 11%). Sight-threatening complications included optic disc edema/hyperemia (26, 30%), cataracts (16, 18%), macular edema (16, 18%), and glaucoma (15, 17%). All 38 patients who initially required systemic treatment received corticosteroids. During the follow-up, 24 patients were treated with azathioprine, 20 with methotrexate, 11 with cyclosporine, 20 with adalimumab, and 8 with infliximab. At the final examination, the mean BCVA of the right and left eyes improved significantly (0.08 ± 0.23 and 0.06 ± 0.17, p = 0.006 and p < 0.001, respectively). The severities of vitritis, anterior chamber inflammation, snowbank/snowball formation, and endotheliitis decreased (all p < 0.001). Thirty-one patients remained on systemic treatment, with only four patients still receiving corticosteroids. No life-threatening adverse effects were reported. CONCLUSION: Despite pediatric PP's mild course, severe vision-threatening complications can occur. Immunomodulatory or biologic agents are important for controlling inflammation and tapering corticosteroids. Further research could enhance understanding of optimal treatments.

Diffuse preretinal infiltrates in a patient with orbital atypical T-cell lymphoproliferative infiltration masquerading posterior uveitis.

Purpose: To report an aggressive and rapidly progressive case of atypical T-cell lymphoproliferative infiltration both with intraocular and orbital involvement and preretinal infiltrates. Methods: Medical records and imaging of the patient were retrospectively reviewed. Case presentation: A 25-year-old woman presented first with preretinal infiltrates resembling uveitis and developed orbital and intracranial signs eventually during her evaluation. Clinical presentation worsened gradually. The patient developed bilateral proptosis, pupillary dilation and uvula deviation. Diagnostic orbital incision biopsy revealed T-cell lymphoproliferative disease. Conclusion: This case gives evidence that intraocular involvement due to T-cell lymphoproliferative disease may present as a masquerade syndrome and should be kept in mind in patients with extraordinary presentation.

Behçet's syndrome: one year in review 2024.

The aim of this review is to provide a critical summary of studies published during 2023 that contribute to our understanding of Behçet's syndrome. An increase in the incidence of BS was reported in Northern Spain after 2014.Studies on patient perspectives showed the impact of Behçet's syndrome on quality of life, daily activities, education, work, and relationships. Differences in genetics among Behçet's syndrome patients with different types of organ involvement were reported and an association between HLA-B/MICA and SLCO4A1 polymorphisms and eye involvement and between DDX60L polymorphisms and nervous system involvement were observed. It was suggested that Integrin α9ß1 plays a crucial role in the neutrophil-mediated inflammatory pathways underlying this syndrome and the dysfunction of the PDL1/PD-1 pathway may be associated with the hyperactivity of the Th-1/Th-17 axis. Vein wall thickness seems to be increased in patients with Behçet's syndrome, but its pathogenetic and clinical implications are not clear. There is growing evidence regarding the efficacy of TNF inhibitors in different types of organ involvement. A number of small case series point out to the potential role of TCZ and JAK inhibitors.

De novo manifestations during adalimumab treatment in Behçet's syndrome.

OBJECTIVES: Treatment response may be variable across organ manifestations of Behçet syndrome (BS). We aimed to determine the frequency of de novo manifestations during adalimumab treatment. METHODS: We conducted a chart review of all BS patients who received adalimumab in our center between 2008 and 2023. Demographic data, reasons for initiating adalimumab, concurrent medications, previous treatments, and outcomes were recorded. We defined de novo manifestations as new BS manifestations that occurred for the first time during treatment with adalimumab. For patients with vascular involvement, a new vascular event at another vessel was also considered as a de novo manifestation. RESULTS: Among the 335 patients, a de novo manifestation developed in 14 (4%) patients. De novo manifestations were vascular involvement in 5 patients, arthritis in 3, anterior uveitis in 2, nervous system involvement in 2, gastrointestinal involvement in 1, and epididymitis in 1 patient. The primary reasons for adalimumab treatment were vascular involvement in 5 patients, uveitis in 4, arthritis in 3, mucocutaneous involvement in 1, and epididymitis in 1 patient. Upon the development of de novo manifestation, adalimumab was switched to another biologic in 4 patients, dose was intensified in 3, colchicine, conventional immunosuppressives, and/or glucocorticoids were added in 5, and topical eye drops were added in 2 patients, leading to remission of de novo manifestations in all patients. CONCLUSION: De novo manifestations were infrequent (4%) among BS patients treated with adalimumab. Of these, 57% were major organ involvement, mainly vascular involvement. None of the patients developed posterior uveitis.

Development of Posterior Uveitis in Behçet Syndrome Patients with Vitreous Cells at Baseline.

PURPOSE: The prognostic importance of vitreous cells (VC) in patients with Behçet syndrome (BS) is unknown. We aimed to determine the frequency of developing posterior uveitis (PU) and any additional risk factors associated with the development of PU in BS patients with VC at diagnosis. METHODS: The charts of 572 consecutive BS patients who were registered between 2010 and 2012 were reviewed. Among the patients with a follow-up of ≥2 years, we included 110 patients with VC in one or both eyes and 147 patients without any eye findings in both eyes at baseline and compared them for the development of PU. RESULTS: Among the 110 included patients with VC, 61 had VC in both eyes, 34 had VC in only one eye, and 15 had VC in one eye and PU in the other eye. There was anterior uveitis (AU) in addition to VC in the same eye in 13 patients at baseline. PU developed in 24 (22%) of these patients during a mean follow-up of 1.9 ± 1.1 years. This was significantly more frequent than the 147 patients without any eye findings at baseline, among whom there were only 2 patients who developed PU (p < 0.001). Multivariate logistic regression analysis showed that having AU in addition to VC in the same eye (OR, 5.03, 95% CI; 1.37-18.47) was an independent risk factor for the development of PU in patients with VC. CONCLUSION: Careful follow-up is required for patients with VC at diagnosis, since 22% developed PU within 2 years.

A Proposal of a New Tool for the Assessment of Damage in Behçet Syndrome Uveitis: Cerrahpasa Ocular Damage Grading System.

INTRODUCTION/OBJECTIVE: There is currently no tool available to assess the severity of damage in uveitis due to Behçet's syndrome (BS). In this preliminary study, we developed a new grading system to evaluate ocular damage and assessed it in a prospective cohort. METHODS: A specialist in BS uveitis (YO) developed a grading system for ocular damage with five grades based on the extent of damage in the posterior segment. YO trained a senior and general ophthalmologist with sample fundus images. BS patients who had undergone color fundus photography during their routine visits in attack-free periods were included in the study. The color fundus photos of this prospective cohort were evaluated blindly by YO and his trainees using the new grading tool. Inter and intra-observer agreement between the graders were assessed by Cohen's kappa analysis. The evaluation of YO was considered as the gold standard. RESULTS: One hundred eighty-five eyes of 108 (29 F/79 M) patients with BS uveitis were graded for damage by two investigators. Their mean age was 38,58 years and their median ocular disease duration was 13 years. The gold standard and the two investigators exhibited substantial concordance with the ocular damage grading system. The inter- and intra-observer agreement were also almost perfect. CONCLUSION: The newly developed ocular damage grading system enables the standardization of damage severity in BS uveitis. It is imperative to conduct internal and external validations across diverse cohorts. Furthermore, future studies should investigate its correlation with other multimodal imaging methods such as fluorescein angiography and optical coherence tomography.

Validation of the Behçet's Syndrome Overall Damage Index (BODI) for Retrospective Studies and a Proposal for Modification.

OBJECTIVE: Assessment of damage accrual over time is important for evaluating and comparing long-term results of treatment modalities and strategies. Retrospective studies may be useful for assessing long-term damage, especially in rare diseases. We aimed to validate Behçet's Syndrome Overall Damage Index (BODI) for use in retrospective studies by evaluating its construct validity, reliability, and feasibility in retrospectively collected data. Additionally, we aimed to determine missing items by evaluating Behçet's syndrome patients with different types of organ involvement and long-term follow-up. METHODS: We included 300 patients who had at least 2 clinic visits at 1-year intervals. The construct validity for use in retrospective trials was assessed by comparing BODI scores calculated from patient charts and during face-to-face visits. BODI was additionally scored using retrospective chart data by 2 different observers and by the same observer six months apart, in a blinded manner. The time for filling BODI was evaluated to assess feasibility. Additionally, damaged items that were missing from BODI were identified. RESULTS: There was a good correlation between the retrospective and face-to-face evaluation of BODI (ICC 0.99; %95 CI 0.99-0.99). Inter-observer and intra-observer agreement were good (ICC 0.96 and 1, respectively). The main damage items that BODI did not capture were hypertension, liver failure, lung parenchymal involvement, glaucoma, and lymphedema. CONCLUSION: BODI seems to be a reliable and feasible instrument for assessing damage in retrospective studies. Modifying BODI using the additional damage items identified in this study may make it an even better scale.

Retinal nerve fiber layer thickness and peripapillary vasculature of post-COVID-19 patients with and without olfactory/gustatory dysfunction symptoms.

PURPOSE: We aimed to compare retinal nerve fiber layer (RNFL) thickness and peripapillary vessel density values between COVID-19 patients with or without olfactory/gustatory dysfunction symptoms and healthy controls. MATERIALS AND METHODS: We evaluated RNFL and radial peripapillary capillary vessel density (RPC-VD) values of 41 patients who had COVID-19 history and age- and gender-matched control group including 31 healthy individuals with optical coherence tomography angiography. First, post-COVID-19 group's and control group's RNFL and RPC-VD values were compared, then post-COVID-19 patients were divided into subgroups according to the presence (subgroup-A) and absence (subgroup-B) of olfactory/gustatory dysfunction symptoms, and same parameters were analyzed for subgroups. RESULTS: Forty-one eyes of 41 post-COVID-19 patients and 31 eyes of 31 age- and gender-matched healthy controls were included in this cross-sectional study. In RNFL analysis, inferior sector thickness was found significantly lower in post-COVID-19 patients by comparison with control group (P = 0.041). In subgroup analyses, COVID-19 patients who first presented with olfactory/gustatory dysfunction symptoms had higher peripapillary and whole image optic disc capillary density (P = 0.011 and P = 0.002) compared to those who had not had these symptoms. CONCLUSION: Lower RPC-VD and RNFL thickness were detected in COVID-19 patients compared to healthy controls. Higher Disc-VD values were found in COVID-19 patients with chemosensorial dysfunction (CSD) symptoms compared to those who had not had these symptoms probably due to milder disease course in COVID-19 with CSD. Sectorial RNFL attenuation in COVID-19 might have occurred secondary to peripapillary capillary circulation defect.

Investigation of optic nerve function in Behçet's patients with optic disc hyperfluorescence in fundus fluorescein angiography.

PURPOSE: The aim was to investigate the changes in optic nerve function that may help in the diagnosis of subclinical optic nerve involvement in patients with Behçet's disease (BD) and isolated optic disc (OD) hyperfluorescence in fluorescein angiography (FA). MATERIALS AND METHODS: Three groups were formed; BD patients with isolated OD hyperfluorescence in FA, BD patients without ocular involvement (normal FA) and control group. A total of 88 eyes of 45 patients were included. The groups were compared in terms of OCT-RNFL, contrast sensitivity and VEP latency. RESULTS: When the OCT-RNFL values were compared, there was a significant difference between the control group and Behçet's patients with normal FA. Contrast sensitivity values differed significantly among the groups, and the lowest mean contrast sensitivity was observed in the group with OD hyperfluorescence (p < 0.05). CONCLUSION: As far as we know, this is the first publication that investigates optic nerve function in BD patients with isolated OD hyperfluorescence in FA. Assessment with FA of asymptomatic BD patients with visual complaints and low contrast sensitivity may be helpful at early detection of inflammatory optic neuropathy by close follow-up in patients with OD hyperfluorescence.

Behçet's syndrome: one year in review 2023.

This critical review of studies on Behçet's syndrome published during 2022 includes studies on epidemiology, patients' perspective, pathogenesis, diagnosis, clinical features and management. Studies on pathogenesis included potential biomarkers mostly related to macrophages, neutrophil and cytokine balance, new GWAS and polymorphism studies, and studies on miRNAs and long non-coding RNAs. Clinical studies showed that application of pneumococcal vaccine to the prick site increased the sensitivity and specificity of the pathergy test and the prevalence of AA amyloidosis had decreased over the years. Studies on management indicated that more data are needed to understand the effect of apremilast on BS manifestations other than oral ulcers, and new BS manifestations may develop during treatment with infliximab. Other biologics and Jak inhibitors might be an option for patients who are refractory to TNF-α inhibitors. Moreover, endovascular repair of arterial aneurysms might be an alternative to open surgery.

Mycophenolate mofetil may be an alternative for maintenance therapy of Behçet syndrome uveitis: a single-center retrospective analysis.

Experience with mycophenolate in uveitis due to Behçet syndrome (BS) is limited. Twelve patients with panuveitis or posterior uveitis who were started mycophenolate were included. Data on demographic characteristics, therapies, ocular attacks, and adverse events were extracted from patient charts. Seven patients with BS uveitis were prescribed mycophenolate for remission induction, of which 6 were refractory/intolerant to conventional immunosuppressives. Mycophenolate was combined with anti-TNFs in 3 patients, resulting in no further ocular attacks. Mycophenolate had to be stopped in the fourth patient due to adverse events. The remaining 3 patients continued to have ocular attacks and were switched to other agents without any drop in visual acuity. Among the 5 patients who were prescribed mycophenolate for maintenance, 2 were relapse free, but 3 experienced ocular attacks. One patient had an exacerbation of mucocutaneous lesions, and 2 experienced adverse events. Mycophenolate monotherapy may not be adequate for remission induction of refractory BS uveitis, but it can be a safe and effective alternative when combined with a biologic agent. It may also be an option for maintenance therapy.

Idiopathic Unilateral Foveal Hemorrhage in a Young Woman Without Precipitating Factors: Evaluation with Optical Coherence Tomography Angiography.

A 28-year-old young Caucasian female patient without a history of trauma or vascular disease presented with blurred vision and paracentral scotoma in her left eye. Fundus examination showed a small foveal hemorrhage in the superficial retinal layers. Initial visual acuity was 20/50 in the LE. After 2 weeks, visual acuity increased to 20/20, and hemorrhage was resolved in optical coherence tomography angiography (OCT-A) spontaneously. No vascular lesion was seen in any layer of the retina in OCT-A analysis.

Behçet Syndrome.

Behçet's syndrome is a systemic vasculitis affecting arteries and veins of all sizes as well as recurrent oral, genital, and intestinal ulcers, skin lesions, predominantly posterior uveitis, and parenchymal brain lesions. These can be present in various combinations and sequences over time and diagnosis is made by recognizing the manifestations, as there are no diagnostic biomarkers or genetic tests. Treatment modalities include immunomodulatory agents, immunosuppressives and biologics, tailored according to prognostic factors, disease activity, severity, and patients' preferences.

Coincident Acute Macular Neuroretinopathy and Paracentral Acute Middle Maculopathy in COVID-19.

An ophthalmology consultation was requested for a 29-year-old woman complaining of visual field defects. The patient had presented to the emergency department with cough and high fever one day earlier. Chest computed tomography demonstrated pneumonia and two severe acute respiratory syndrome coronavirus 2 polymerase chain reaction tests were positive. The patient had undergone renal transplantation 11 years ago due to glomerulonephritis. Best-corrected visual acuity (BCVA) was 20/40 in the right eye and 20/30 in the left eye. Fluorescein angiography showed macular hypoperfusion, and optical coherence tomography (OCT) showed hyperreflectivity in the inner nuclear, outer plexiform, and outer nuclear layers, as well as discontinuity of the ellipsoid zone. Perimetry confirmed bilateral central scotoma. Levels of D-dimer and fibrinogen were 0.86 g/mL and 435.6 g/mL, respectively. The patient was diagnosed as having concurrent acute macular neuroretinopathy and paracentral acute middle maculopathy and was given low-molecular-weight heparin treatment for one month. Her BCVA improved to 20/20 in both eyes, and regression was observed in the retinal findings, hyperreflectivity and ellipsoid zone disruption on OCT, and scotoma in perimetry. Inflammation, thrombosis, and glial involvement may play a role in the pathogenesis of retinal microvascular impairment in COVID-19.

Bilateral Cystoid Macular Edema after COVID-19: 1 Year Follow Up.

PURPOSE: The aim is to report a case of bilateral macular edema after COVID-19 pneumonia. CASE REPORT: A 66-year-old male patient with history of COVID-19 pneumonia presented to us with decreased vision. Examination showed bilateral cystoid macular edema (CME), which was confirmed on optical coherence tomography (OCT). There were no findings in the fundus examination. He had no systemic disease, drug or surgery history, or any factors that could explain the clinic presentation. Work-up for uveitis was unremarkable. After topical therapy with brinzolamide 1% and nepafenac 0.1%, macular edema regressed in a month. CONCLUSION: This is an unusual case of CME in previous COVID-19 infection. This presentation may be a parainfectious or a post-viral manifestation of COVID-19.

Case-control study of corneal topography and specular microscopy parameters in JIA patients with and without ocular involvement.

PURPOSE: To assess corneal topography and specular microscopy values in juvenile idiopathic arthritis-related uveitis (JIA-U). METHODS: This case-control study included 30 eyes from JIA-U patients, 20 eyes from JIA patients, and 50 eyes from age- and gender-matched healthy subjects. Patients with a history of ocular diseases or intraocular surgery were excluded. Corneal topography maps (Pentacam HR) and specular microscopy images (CellChek SL) were obtained. The measurements of the groups were compared. RESULTS: Keratometric astigmatism was higher in the JIA-U group than in the control group (p = 0.040). Patients with astigmatism greater than 1.50D were more common in the JIA-U group than in the control group (p = 0.026). The JIA-U group had higher anterior and posterior elevation values than the control group (p = 0.006, p = 0.025). The density of endothelial cells, coefficient of variation, and hexagonality did not change across groups (p = 0.465, p = 0.096, p = 0.869). The total number of exacerbations and the duration of anterior chamber inflammation were both positively correlated with posterior elevation (r = 0.600, p 0.001; r = 0.583, p 0.001). The age of diagnosis was found to be negatively correlated with anterior elevation (r = -0.412, p = 0.021). CONCLUSION: Corneal astigmatism, as well as anterior and posterior elevation values, were all higher in JIA-U patients. Endothelial cell density and morphology, on the other hand, did not differ significantly between groups. Chronic inflammation's impact on stromal remodelling could explain these corneal alterations. The positive correlation between posterior elevation and the number of flares and duration of inflammation represents the importance of early diagnosis and effective treatment.

Evaluation of Retinal Microvascular Impairment after COVID-19 and its Clinical Correlates Using Optical Coherence Tomography Angiography.

Objectives: Retinal vascular complications have been described in patients with coronavirus disease 2019 (COVID-19). This study aimed to analyze retinal microvascular changes and their correlations with clinical findings. Materials and Methods: This case-controlled study was conducted in a university hospital. The right eyes of 52 otherwise healthy patients recovered from COVID-19 and 42 healthy controls were examined with optical coherence tomography angiography. Mann-Whitney U test was used to compare vessel density (VD) and foveal avascular zone (FAZ) parameters. Associations with treatment choices, pneumonia, and laboratory findings were analyzed. Results: Twenty-nine patients (56%) and 18 healthy controls (43%) were men. Mean age of the COVID-19 group was 39.00±13.04 years. Twenty-two patients had pneumonia, 18 (35%) received hydroxychloroquine (HCQ), 17 (33%) received HCQ plus low-molecular-weight heparin (LMWH), and 10 (19%) received favipiravir. The patient group had lower parafoveal VD in the superficial capillary plexus (SCP) and lower parafoveal VD and perifoveal VD in the deep capillary plexus (DCP) than controls (p=0.003, p=0.004, p=0.001). FAZ area did not differ significantly (p=0.953). Perifoveal VD in the DCP was also significantly lower in the HCQ+LMWH group than the HCQ group (p=0.020) and in the presence of pneumonia (p=0.040). C-reactive protein (CRP) and ferritin levels were negatively correlated with perifoveal VD in the DCP (r=-0.445, p=0.023; r=-0.451, p=0.040). Ferritin was also negatively correlated with parafoveal VD in the SCP (r=-0.532, p=0.013). Conclusion: Parafoveal and perifoveal VD was found to be lower in the COVID-19 group. Presence of pneumonia, need for LMWH prophylaxis, and levels of CRP and ferritin were found to be negatively associated with retinal VD. Large-scale studies are needed to evaluate the clinical importance.

Behçet's disease risk-variant HLA-B51/ERAP1-Hap10 alters human CD8 T cell immunity.

OBJECTIVES: The endoplasmic reticulum aminopeptidase (ERAP1) haplotype Hap10 encodes for a variant allotype of the endoplasmic reticulum (ER)-resident peptide-trimming aminopeptidase ERAP1 with low enzymatic activity. This haplotype recessively confers the highest risk for Behçet's diseases (BD) currently known, but only in carriers of HLA-B*51, the classical risk factor for the disease. The mechanistic implications and biological consequences of this epistatic relationship are unknown. Here, we aimed to determine its biological relevance and functional impact. METHODS: We genotyped and immune phenotyped a cohort of 26 untreated Turkish BD subjects and 22 healthy donors, generated CRISPR-Cas9 ERAP1 KOs from HLA-B*51 + LCL, analysed the HLA class I-bound peptidome for peptide length differences and assessed immunogenicity of genome-edited cells in CD8 T cell co-culture systems. RESULTS: Allele frequencies of ERAP1-Hap10 were similar to previous studies. There were frequency shifts between antigen-experienced and naïve CD8 T cell populations of carriers and non-carriers of ERAP1-Hap10 in an HLA-B*51 background. ERAP1 KO cells showed peptidomes with longer peptides above 9mer and significant differences in their ability to stimulate alloreactive CD8 T cells compared with wild-type control cells. CONCLUSIONS: We demonstrate that hypoactive ERAP1 changes immunogenicity to CD8 T cells, mediated by an HLA class I peptidome with undertrimmed peptides. Naïve/effector CD8 T cell shifts in affected carriers provide evidence of the biological relevance of ERAP1-Hap10/HLA-B*51 at the cellular level and point to an HLA-B51-restricted process. Our findings suggest that variant ERAP1-Hap10 partakes in BD pathogenesis by generating HLA-B51-restricted peptides, causing a change in immunodominance of the ensuing CD8 T cell response.

Higher antibody responses after mRNA-based vaccine compared to inactivated vaccine against SARS-CoV-2 in Behcet's syndrome.

There are limited data about humoral response to vaccine in Behçet's syndrome (BS). We compared SARS-CoV-2 antibody response after two doses of inactivated (Sinovac/CoronaVac) or mRNA (Pfizer/BioNTech) vaccines in patients with BS and healthy controls (HCs). We studied 166 (92M/74F) patients with BS (mean age: 42.9 ± 9.6 years) and 165 (75M/90F) healthy controls (mean age: 42.4 ± 10.4 years), in a single-center cross-sectional design between April 2021 and October 2021. A total of 80 patients with BS and 89 HCs received two doses of CoronaVac, while 86 patients with BS and 76 HCs were vaccinated with BioNTech. All study subjects had a negative history for COVID-19. Serum samples were collected at least 21 days after the second dose of the vaccine. Anti-spike IgG antibody titers were measured quantitatively using a commercially available immunoassay method. We found that the great majority in both patient and HC groups had detectable antibodies after either CoronaVac (96.3% vs 100%) or BioNTech (98.8% vs 100%). Among those vaccinated with CoronaVac, BS patients had significantly lower median (IQR) titers compared to HCs [36.5 (12.5-128.5) vs 102 (59-180), p < 0.001]. On the other hand, antibody titers did not differ among patients with BS and HCs who were vaccinated with BioNTech [1648.5 (527.0-3693.8) vs 1516.0 (836.3-2599.5), p = 0.512). Among different treatment regimen subgroups in both vaccine groups, those who were using anti-TNF-based treatment had the lowest antibody titers. However, the difference was statistically significant only among those vaccinated with CoronaVac. Among patients vaccinated with BioNTech, there was no statistically significant difference between different treatment regimen groups. Compared to inactivated COVID-19 vaccine, mRNA-based vaccine elicited higher antibody titers among BS patients. Only in the CoronaVac group, patients especially those using anti-TNF agents were found to have low titers compared to healthy subjects. BS patients vaccinated with BioNTech were found to have similar seroconversion rates and antibody levels compared to healthy controls. Further studies should assess whether the low antibody titers are associated with diminished protection against COVID-19 in both vaccine groups.

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.