Folliculitis might be associated with pathergy-positivity in patients with Behçet syndrome.

Pathergy test indicates nonspecific hyper-reactivity of the skin to aseptic trauma in Behçet syndrome (BS) and is considered as an adjunctive diagnostic test with a good specificity albeit with low sensitivity. We tested the hypothesis that a relationship exists between active clinical manifestations of BS and the pathergy-positivity when performed simultaneously. Pathergy test and detailed dermatologic examination were done in 105 BS patients (60M/45F); who were seen consecutively at the multi-disciplinary BS outpatient clinic in a single tertiary center. Information regarding demographic and clinical characteristics, pathergy test results at diagnosis, and details about treatment were obtained from patient charts. Disease activity was assessed using Behçet Disease Current Activity Form. Among 105 patients, 27 (25.7%) were pathergy-positive at the time of the study visit whereas 40.9% were pathergy-positive at the time of the diagnosis. There was no relation between pathergy test and patient age or disease duration, either. Pathergy-positivity was significantly more common in patients with folliculitis compared to those without folliculitis (40.7% vs 19.2%; P = .026). The test was also positive in all 3 patients with leg ulcers due to venous stasis. We found that among all skin-mucosa lesions only the presence of folliculitis was associated with pathergy positivity with statistical significance. It was also remarkable that the current pathergy was positive in all 3 patients with active leg ulcers but this finding warrants further studies because of the low patient numbers.

Increased arterial stiffness and accelerated atherosclerosis in Takayasu arteritis.

INTRODUCTION: Cardiovascular diseases are the leading causes of morbidity and mortality in patients with Takayasu arteritis (TAK). Arterial stiffness and accelerated atherosclerosis have been reported in TAK, however, morphological changes in the arterial wall have not been adequately addressed. Shear wave elastography (SWE) is a new, non-invasive, direct and quantitative method of ultrasonography (US) that evaluates elasticity of biological tissues. METHODS: A total of 50 patients with TAK (44F/6 M; mean age: 39.8 ± 8.2 years), 43 with systemic lupus erythematosus (SLE) (38F/5 M; 38.0 ± 7.9 years) and 57 healthy controls (HCs) (50F/7M: 39.5 ± 7.1 years) were studied using carotid B mode US and SWE. Carotid artery intima-media thickness (CCA IMT) and SWE were measured and the atherosclerotic plaques were recorded. Clinical characteristics and cardiovascular risk factors were determined. Intra and inter observer reproducibility was assessed and found good agreement. RESULTS: The mean IMT in the right and left carotid arteries was significantly higher only among patients with TAK when compared to SLE and HCs. Carotid artery plaques were significantly increased only in patients with TAK. On the other hand, the mean SWE value was significantly increased among both TAK and SLE patients when compared to HCs, whereas patients with TAK had the highest value. These were also true after adjustments were made for atherosclerotic risk factors and after all those with atherosclerotic plaques were excluded from the analysis. TAK itself, diastolic blood pressure levels and IMT were independently associated with SWE. CONCLUSIONS: Markedly increased CCA IMT and SWE values appear to be uniquely associated with TAK, suggesting that they could be used as diagnostic tools. Arterial stiffness occurs independently from atherosclerosis and is associated with arterial thickening. Further studies should investigate whether CCA SWE values could predict cardiovascular morbidity and mortality. Strong association with premature atherosclerosis could be also considered as a unique feature of TAK.

A review of rheumatoid meningitis with case studies.

Rheumatoid arthritis (RA) is an autoimmune disease involving synovial joints, and it is known that extra-articular manifestations that may affect the central and peripheral nervous systems may develop during its course. Rheumatoid meningitis is very rare among all neurological involvements. In this study, cases diagnosed as rheumatoid meningitis with clinical, imaging, laboratory, and histopathological features are presented, and the aim of the study is to present current approaches in the diagnosis and treatment of rheumatoid meningitis in the light of case studies and current literature. The data of the patients who were followed up with the diagnosis of rheumatoid meningitis in neurology clinic between 2017 and 2021 were reviewed retrospectively. Three cases diagnosed with rheumatoid meningitis are presented in detail. In the first case, the diagnosis was reached by clinical, imaging, and laboratory findings as well as treatment response, while the diagnosis was made by histopathological verification in the second case. The third case shows that spontaneous remission can be observed in the course of rheumatoid meningitis. Rheumatoid meningitis, which is one of the rarest involvements in the course of RA, may present with headaches, focal neurological deficits, seizures, and altered consciousness. A meningeal biopsy is recommended when the differential diagnosis cannot be ruled out with imaging and laboratory findings. In the differential diagnosis Mucobacterium tuberculosis, syphilis, neuro-sarcoidosis, immunoglobulin G4-related disease, lymphoproliferative diseases, and systemic metastasis should be kept in mind. Aggressive RA management is recommended for treatment.

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.

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.

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.

ANCA-associated vasculitis flare might be provoked by COVID-19 infection: a case report and a review of the literature.

Mesangial immunoglobulin A (IgA) deposition is the hallmark of IgA nephropathy (IgAN). In some cases, crescentic involvement that might be associated with systemic leucocytoclastic vasculitis is documented. In such cases, the disease is called Henoch-Schönlein purpura (IgA vasculitis). Even more rarely, the coexistence of IgAN and anti-neutrophil cytoplasmic antibody (ANCA) seropositivity has been reported. IgAN might be complicated by acute kidney injury (AKI) due to different causes. Herein we present a patient with mesangial IgA deposition and ANCA seropositivity who developed AKI, haematuria and haemoptysis during the course of coronavirus disease 2019 (COVID-19) disease and was diagnosed with ANCA-associated vasculitis based on clinical, laboratory and radiological findings. The patient was treated successfully with immunosuppressive therapy. We also made a systematic review of the literature to reveal and present the cases with COVID-19 and ANCA-associated vasculitis.

Recent Insights into the Management of Behçet Syndrome.

Behçet syndrome (BS) is a multisystem vasculitis with variable vessel involvement that shows significant heterogeneity among patients in terms of clinical manifestations and disease course. Treatment choice and response are both influenced by this heterogeneity. BS treatments' main goals are to quickly suppress inflammatory exacerbations and prevent relapses in order to protect organ functions and provide good quality of life. Besides the long-term experience with steroids and traditional immunosuppressives, biologic drugs, especially TNF inhibitors, have gained increasing importance in the treatment of BS over the years. In this review, we aimed to give an overview of the studies with conventional and biological drugs with proven efficacy in the treatment of BS, as well as promising drugs and current management strategies according to clinical phenotypes.

An evaluation of apremilast for the treatment of adult patients with oral ulcers associated with Behçet's syndrome.

INTRODUCTION: Behçet's syndrome is a chronic, multi-system, variable vasculitis of unknown etiology that can result in significant morbidity and mortality. Mucocutaneous lesions such as oral ulcers and genital ulcers are common manifestations that can affect the quality of life of patients significantly. Treatment for mucocutaneous lesions in Behçet's syndrome continues to be critical, and an unmet need remains a significant issue. AREAS COVERED: This review evaluates the mechanism of action of apremilast, its effect on the number and pain of oral ulcers, other manifestations, such as genital ulcers, disease activity, quality of life and safety profile in Behçet's syndrome patients. Data from clinical trials as well as observational studies were included. EXPERT OPINION: Two randomized placebo-controlled trials and real-world observational data suggest that apremilast is an effective and well-tolerated treatment modality for oral and genital ulcers in Behçet's syndrome. Observational studies additionally showed beneficial results for skin lesions, arthritis, and intestinal involvement.

Willingness to get the COVID-19 vaccine among patients with rheumatic diseases, healthcare workers and general population in Turkey: a web-based survey.

OBJECTIVES: Vaccination against COVID-19 emerges as an effective strategy for combating the pandemic. While many of our patients with rheumatic diseases (RD) wonder whether it is safe to get the vaccine, vaccine hesitancy is rising among the general population. We assessed the willingness to get vaccination and its probable predictors among patients with RD compared to healthcare workers and a sample from the general population. METHODS: We conducted a web-based questionnaire survey in a cross-sectional design in 3 groups of participants just before the mass vaccination program in Istanbul, Turkey. The questionnaire sought socio-demographic variables, COVID-19 related risk factors, willingness to get vaccination, and concerns and thoughts about vaccine. COVID-19 anxiety scale (CAS) was also evaluated. RESULTS: We studied in total 732 patients with RD (Group 1), 763 individuals representing general population (Group 2) and 320 hospital workers (Group 3). Dysfunctional anxiety related to COVID-19 was found in 4.9%, 3.8% and 4.1%, in Group 1, 2 and 3, respectively. Of the patients with RD, 29.2% were willing to be vaccinated, 19.0% were unwilling and 51.8% were undecided. These were somewhat similar among the general population (yes: 34.6%, no: 23.3% and unsure: 42.1%), with significantly less undecided individuals (p < 0.001). On the other hand, hospital workers were significantly more willing (yes: 52.5%, no: 20.9% and unsure: 26.6%) (p < 0.001). Main concerns were probable side effects, unknown scientific results and having no trust. Being male, older age, working in a hospital, not having contracted COVID-19 and high scores on CAS were found to be independently associated with willingness. CONCLUSIONS: The low rate of vaccine acceptance among patients with RD, as well as general population sampling is worrying. Healthcare policies should aim to implement communication, promote confidence and increase demand for COVID-19 vaccine.