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1.
Article in English | MEDLINE | ID: mdl-38733594

ABSTRACT

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.

2.
Ocul Immunol Inflamm ; : 1-7, 2024 May 20.
Article in English | MEDLINE | ID: mdl-38768092

ABSTRACT

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.

3.
Taiwan J Ophthalmol ; 14(1): 102-107, 2024.
Article in English | MEDLINE | ID: mdl-38654983

ABSTRACT

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.

4.
Int Ophthalmol ; 44(1): 76, 2024 Feb 13.
Article in English | MEDLINE | ID: mdl-38351422

ABSTRACT

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.


Subject(s)
Behcet Syndrome , Optic Disk , Optic Nerve Diseases , Humans , Behcet Syndrome/complications , Behcet Syndrome/diagnosis , Fluorescein Angiography , Optic Nerve/diagnostic imaging
5.
Clin Exp Rheumatol ; 41(10): 1945-1954, 2023 Oct.
Article in English | MEDLINE | ID: mdl-37877363

ABSTRACT

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.


Subject(s)
Aneurysm , Behcet Syndrome , Humans , Behcet Syndrome/diagnosis , Behcet Syndrome/drug therapy , Behcet Syndrome/epidemiology , Infliximab/therapeutic use , Tumor Necrosis Factor-alpha/therapeutic use , Sensitivity and Specificity , Tumor Necrosis Factor Inhibitors/therapeutic use
6.
Rheumatol Int ; 43(11): 2099-2106, 2023 11.
Article in English | MEDLINE | ID: mdl-37592141

ABSTRACT

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.


Subject(s)
Behcet Syndrome , Uveitis , Humans , Behcet Syndrome/complications , Behcet Syndrome/diagnosis , Behcet Syndrome/drug therapy , Mycophenolic Acid/adverse effects , Retrospective Studies , Uveitis/drug therapy , Uveitis/etiology , Immunosuppressive Agents/adverse effects
7.
Beyoglu Eye J ; 8(2): 134-138, 2023.
Article in English | MEDLINE | ID: mdl-37521881

ABSTRACT

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.

8.
Rheum Dis Clin North Am ; 49(3): 585-602, 2023 08.
Article in English | MEDLINE | ID: mdl-37331734

ABSTRACT

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.


Subject(s)
Behcet Syndrome , Vasculitis , Humans , Behcet Syndrome/diagnosis , Behcet Syndrome/therapy
9.
Turk J Ophthalmol ; 53(2): 120-123, 2023 04 20.
Article in English | MEDLINE | ID: mdl-37089033

ABSTRACT

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.


Subject(s)
COVID-19 , Macular Degeneration , Retinal Diseases , White Dot Syndromes , Female , Humans , Adult , Retinal Diseases/diagnosis , Retinal Diseases/etiology , COVID-19/complications , Fluorescein Angiography/methods , Scotoma/etiology , Scotoma/complications , Macular Degeneration/complications
10.
Int Ophthalmol ; 43(2): 635-641, 2023 Feb.
Article in English | MEDLINE | ID: mdl-36006550

ABSTRACT

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.


Subject(s)
Astigmatism , Humans , Corneal Topography/methods , Case-Control Studies , Astigmatism/diagnosis , Microscopy/methods , Endothelial Cells , Cornea , Inflammation
11.
Ocul Immunol Inflamm ; 31(6): 1236-1239, 2023 Aug.
Article in English | MEDLINE | ID: mdl-36074579

ABSTRACT

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.


Subject(s)
COVID-19 , Macular Edema , Male , Humans , Aged , Macular Edema/diagnosis , Macular Edema/drug therapy , Macular Edema/etiology , Follow-Up Studies , COVID-19/complications , Tomography, Optical Coherence/methods , Fundus Oculi
12.
Turk J Ophthalmol ; 52(5): 324-330, 2022 10 28.
Article in English | MEDLINE | ID: mdl-36317806

ABSTRACT

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.


Subject(s)
COVID-19 , Tomography, Optical Coherence , Male , Humans , Adult , Middle Aged , Female , Tomography, Optical Coherence/methods , Fluorescein Angiography/methods , Fovea Centralis , COVID-19/complications , Heparin, Low-Molecular-Weight , Hydroxychloroquine/therapeutic use , Ferritins
13.
Ann Rheum Dis ; 81(11): 1603-1611, 2022 11.
Article in English | MEDLINE | ID: mdl-35922122

ABSTRACT

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.


Subject(s)
Behcet Syndrome , CD8-Positive T-Lymphocytes , HLA-B51 Antigen , Minor Histocompatibility Antigens , Aminopeptidases/genetics , Behcet Syndrome/genetics , CD8-Positive T-Lymphocytes/immunology , HLA-B51 Antigen/genetics , Humans , Minor Histocompatibility Antigens/genetics , Peptides
14.
Rheumatol Int ; 42(10): 1741-1750, 2022 10.
Article in English | MEDLINE | ID: mdl-35779083

ABSTRACT

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.


Subject(s)
Behcet Syndrome , COVID-19 , Viral Vaccines , Adult , Antibodies, Viral , Antibody Formation , COVID-19/prevention & control , COVID-19 Vaccines , Cross-Sectional Studies , Humans , Middle Aged , RNA, Messenger , SARS-CoV-2 , Tumor Necrosis Factor Inhibitors , Vaccines, Inactivated , Viral Vaccines/adverse effects
15.
Clin Exp Rheumatol ; 40(8): 1461-1471, 2022 Sep.
Article in English | MEDLINE | ID: mdl-35894066

ABSTRACT

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.


Subject(s)
Behcet Syndrome , Anticoagulants/therapeutic use , Behcet Syndrome/diagnosis , Behcet Syndrome/drug therapy , Behcet Syndrome/epidemiology , Humans
16.
Turk J Pediatr ; 64(2): 285-292, 2022.
Article in English | MEDLINE | ID: mdl-35611417

ABSTRACT

BACKGROUND: Retinoblastoma shows high rates of recurrence after initial chemotherapy (systemic or intraarterial). Our aim was to evaluate the effectiveness of iodine-125 radioactive plaque brachytherapy as a salvage treatment with globe-preserving attributes after initial chemotherapy in patients with intraocular retinoblastoma. METHODS: The effect of brachytherapy was investigated retrospectively in 17 eyes of 17 patients who were followed up due to retinoblastoma between May 2012 and June 2018 and who received iodine-125 radioactive plaque brachytherapy as a salvage treatment after systemic or intra-arterial chemotherapy. The regression, ocular toxicity, and enucleation rates were evaluated at the end of the follow-up period. RESULTS: The tumor locations were post equator, macular, anterior to the equator, and peripapillary in 5, 3, 7, and 2 patients, respectively. Regression was initially and rapidly observed in 17 of the 17 eyes that underwent brachytherapy. Enucleation was performed in 5 (29.42%) of these patients due to recurrence with diffuse tumor involvement, and 4 of the tumors were located anterior to the equator. In 12 (70.58%) patients, the eyes were protected from enucleation following local brachytherapy. CONCLUSIONS: Radioactive plaque brachytherapy can be applied as an effective salvage therapy with successful results in retinoblastoma patients who have received initial systemic or intra-arterial chemotherapy. Post equator-located solitary tumors have the highest success rate.


Subject(s)
Brachytherapy , Retinal Neoplasms , Retinoblastoma , Humans , Infant , Iodine Radioisotopes , Retinal Neoplasms/drug therapy , Retinal Neoplasms/radiotherapy , Retinoblastoma/drug therapy , Retinoblastoma/radiotherapy , Retrospective Studies , Salvage Therapy , Treatment Outcome
17.
Rheumatol Int ; 42(6): 973-987, 2022 06.
Article in English | MEDLINE | ID: mdl-35376962

ABSTRACT

Most of the published data relate to classical forms of rheumatic diseases (RD) and information on rare inflammatory disorders such as Behçet's syndrome (BS) and familial Mediterranean fever (FMF) is limited. We studied the frequency of side effects and disease flares after COVID-19 vaccination with either Pfizer/BioNTech or Sinovac/CoronaVac in 256 patients with BS, 247 with FMF, and 601 with RD. Telephone interviews were conducted using a questionnaire survey in a cross-sectional design in patients with BS, FMF, and RD followed by a single university hospital. Study participants were vaccinated either with CoronaVac (BS:109, FMF: 90, and RD: 343,) or BioNTech (BS: 147, FMF: 157 and RD: 258). The majority have received double dose (BS: 94.9%, FMF 92.3% and RD: 86.2%). BioNTech ensured a significantly better efficacy than CoronaVac against COVID-19 in all patient groups (BS: 1.4% vs 10.1%; FMF: 3.2% vs 12.2%, RD:2.7% vs 6.4%). Those with at least one adverse event (AE) were significantly more frequent among those vaccinated with BioNTech than those with CoronaVac (BS: 86.4% vs 45%; FMF: 83.4% vs 53.3%; and RD: 83.3% vs 45.5%). The majority of AEs were mild to moderate and transient and this was true for either vaccine. There were also AEs that required medical attention in all study groups following CoronaVac (BS: 5.5%, FMF: 3.3%, and RD:2.9%) or BioNTech (BS: 5.4%, FMF: 1.9%, and RD: 4.7%). The main causes for medical assistance were disease flare and cardiovascular events. Patients with BS (16.0%) and FMF (17.4%) were found to flare significantly more frequently when compared to those with RD (6.0%) (p < 0.001). This was true for either vaccine. BS patients reported mainly skin-mucosa lesions; there were however, 11 (4.3%) who developed major organ attack such as uveitis, thrombosis or stroke. Flare in FMF patients were associated mainly with acute serositis with or without fever. Arthralgia/arthritis or inflammatory back pain were observed mainly in the RD group. Our study demonstrates that BS and FMF patients vaccinated with either CoronaVac or BioNTech demonstrated similar AE profile and frequency compared to RD patients. AEs that required physician consultation or hospitalization occurred in all study groups after either CoronaVac or BioNTech. Increased frequency of flares in BS and FMF compared to that seen in RD might reflect defects in innate immunity and deserves further investigation. Caution should be required when monitoring these patients after vaccination.


Subject(s)
Behcet Syndrome , COVID-19 , Familial Mediterranean Fever , Rheumatic Diseases , Behcet Syndrome/complications , COVID-19/prevention & control , COVID-19 Vaccines/adverse effects , Cross-Sectional Studies , Familial Mediterranean Fever/complications , Humans , Pain/complications , RNA , Rheumatic Diseases/complications , SARS-CoV-2 , Vaccination/adverse effects , Vaccines, Inactivated
18.
Rheumatology (Oxford) ; 61(9): 3746-3753, 2022 08 30.
Article in English | MEDLINE | ID: mdl-34958357

ABSTRACT

OBJECTIVES: Infliximab (IFX) is increasingly being used for the treatment of severe manifestations of Behçet's syndrome (BS). However, emergence of new manifestations has also been occasionally reported during IFX treatment. We aimed to assess the frequency of new manifestations in our BS patients treated with IFX. METHODS: A chart review was conducted to identify all BS patients treated with IFX in our clinic between 2004 and 2020. Demographic data, indications for IFX initiation, concomitant treatments and outcomes were recorded. A new manifestation was defined as the emergence of a new organ involvement or mucocutaneous manifestation developing for the first time during IFX treatment or within 12 weeks after the last infusion of IFX. RESULTS: Among our 282 patients who used IFX, 19 (7%) patients had developed a total of 23 new manifestations during a mean follow-up of 20.0 (15.3) months. Patients with vascular involvement were more likely to develop a new manifestation (12/19, 63%). Initial manifestations that required IFX were in remission at the time of new manifestation in 14/19 patients. IFX treatment was intensified (n = 6) and/or glucocorticoids, immunosuppressives or colchicine was added to IFX (n = 21). IFX was switched to another agent for the remaining manifestations (n = 8). These treatment modifications led to remission in 17/19 patients. CONCLUSION: New manifestations developed during IFX treatment in 7% of our patients with BS. They could be managed by intensifying IFX treatment or adding other agents in the majority of these manifestations.


Subject(s)
Behcet Syndrome , Behcet Syndrome/complications , Behcet Syndrome/drug therapy , Colchicine/therapeutic use , Humans , Immunosuppressive Agents/therapeutic use , Infliximab/adverse effects , Treatment Outcome
19.
Eur J Ophthalmol ; 32(3): 1570-1576, 2022 May.
Article in English | MEDLINE | ID: mdl-34053312

ABSTRACT

PURPOSE: This study aims to investigate the effect of using exogenous androgen on retinal microvascular structures in transgender men. METHODS: Retinal microvascular structures of transgender men and healthy women in the control group were visualized with optical coherence tomography angiography (OCTA). Foveal avascular zone (FAZ), superficial capillary vascular density (sCVD), deep capillary vascular density (dCVD), and radial peripapillary capillary (RPC) vascular density were evaluated. RESULTS: Thirty transgender men and 30 healthy women were included in the study. There were no significant differences between transgender men and the control group in terms of FAZ, central macular thickness, parafoveal, and perifoveal retinal thicknesses (p = 0.859, 0.288, 0.561, and 0.719; respectively). sCVD were found to be low in transgender men in the whole image, parafoveal, and perifoveal zones (p = 0.006, 0.025, and 0.005; respectively). Although there were low values of dCVD of the whole image, parafoveal, and perifoveal zones in transgender men, the difference was not statistically significant (p = 0.295, 0.085, and 0.270; respectively). RPC vessel densities in the whole image, peripapillary zone, and the inferior of the optic disc were found to be significantly low in transgender men (p = 0.003, 0.005, and 0.003; respectively). CONCLUSION: In this study, a decrease in vessel density in the superficial retinal layers and around the optic disc was detected in transgender men. These findings suggest that routine ophthalmological examination may be important in individuals who use exogenous androgens.


Subject(s)
Tomography, Optical Coherence , Transgender Persons , Androgens/pharmacology , Female , Fluorescein Angiography , Humans , Male , Retinal Vessels , Tomography, Optical Coherence/methods
20.
Graefes Arch Clin Exp Ophthalmol ; 260(4): 1337-1343, 2022 Apr.
Article in English | MEDLINE | ID: mdl-34735632

ABSTRACT

PURPOSE: To compare the efficacies of iodine-125 brachytherapy (IBT) and gamma knife stereotactic radiosurgery (GKRS) in the treatment of posterior uveal melanoma. METHODS: The demographic data and tumor characteristics at diagnosis of 201 patients treated with IBT and 52 patients treated with GKRS were recorded. The two treatments were then compared in terms of complications, local control, eye retention, metastasis, and overall survival rate. RESULTS: The median follow-up time was 56 months for the GKRS group and 45 months for the IBT group (p = 0.167). There were no significant differences in demographic data or tumor characteristics between the groups at diagnosis. Radiation retinopathy, radiation optic neuropathy, and neovascular glaucoma occurred at similar rates in both groups. However, radiation maculopathy and cataracts occurred more frequently in the GKRS group. The number of cases that have developed vision loss (worsening of best-corrected visual acuity on three or more lines on the Snellen chart) was significantly higher in the GKRS group (60%) compared to the IBT group (44%) (p = 0.048). Local control, metastasis, and 5-year overall survival rates were statistically similar in both groups. CONCLUSIONS: GKRS can be preferred as an eye-sparing treatment option for posterior uveal melanoma in cases where brachytherapy cannot be used.


Subject(s)
Brachytherapy , Radiosurgery , Uveal Neoplasms , Brachytherapy/adverse effects , Humans , Iodine Radioisotopes , Melanoma , Retrospective Studies , Treatment Outcome , Uveal Neoplasms/diagnosis , Uveal Neoplasms/radiotherapy
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