Reliability and validity of the Turkish version of Scleroderma Skin Patient-Reported Outcome in patients with systemic sclerosis.

Objectives: This study aimed to translate the Scleroderma Skin Patient-Reported Outcome (SSPRO) questionnaire to the Turkish (SSPRO-T) language and to assess its validity and reliability. Patients and methods: Fifty-four systemic sclerosis (SSc) patients (51 females, 3 males; mean age: 49.8±10.4 years; range, 22 to 65 years) participated in the reliability and validity analysis between October 2022 and December 2022. The translation and cross-cultural adaptation of the SSPRO-T was applied in accordance with the procedure described by the Beaton guidelines. The SSPRO-T, the Scleroderma Health Assessment Questionnaire (SHAQ), the Health Assessment Questionnaire Disability Index (HAQ-DI), Skindex-29, and patient global skin severity were conducted in all participants for construct validity. The SSPRO-T was retested to assess its reliability after seven days. Results: The SSPRO-T had a four-factor structure. The total SSPRO-T score and its subgroups correlated positively with SHAQ, HAQ-DI, Skindex-29, and patient global skin severity. The internal consistency and reliability were excellent in overall SSPRO-T and in the subgroups: physical effect, emotional effect, physical limitation, and social effect (Cronbach's α=0.94, 0.80, 0.95, 0.93, and 0.84, respectively). The SSPRO-T had excellent test-retest reliability (r=0.91, p<0.001). In addition, no floor effect or ceiling effect was observed. Conclusion: The SSPRO-T questionnaire is a reliable and valid tool and can be used in research and clinical practice in Turkish patients with SSc.

Coronavirus Disease 2019 Outcomes in Amyloid A Protein Amyloidosis Secondary to Rheumatic Conditions and Signs of Post- Coronavirus Disease 2019 Proteinuria Progression.

BACKGROUND: We aimed to investigate coronavirus diease 2019 (COVID-19) outcomes in patients with amyloid A protein (AA) amyloidosis secondary to rheumatic diseases and discuss factors associated with disease course. METHODS: A retrospective cohort was formed from adult patients with a diagnosis of AA amyloidosis. In patients with a positive severe acute respiratory syndrome coronavirus 2 polymerase chain reaction (PCR) test, rates of hospitalization, intensive care unit admission and mortality due to COVID-19 were collected from medical records. Data regarding to demographics, comorbidities, laboratory tests, medical treatments, adherence to previous treatments during COVID-19 and treatment administered for COVID-19 were collected from hospital databases and patient reviews. RESULTS: In 96 patients with AA amyloidosis, 16 had COVID-19 with a positive PCR. Ten (62.5%) patients were hospitalized, 2 (12.5%) were admitted to ICU, 1 (6.25%) was died. Hospitalized patients tended to be older. Comorbidities seemed to be more frequent in hospitalized patients. None of the patients had rapid progression to end-stage renal disease post-COVID-19. Seven patients had pre-COVID-19 and post-COVID-19 proteinuria levels. Three had notable increase in proteinuria after COVID-19 in 2 of which amyloidosis treatment was revised accordingly. CONCLUSION: Despite high rates of hospitalization in AA amyloidosis patients, mortality was observed only in 1 patient. Progression of proteinuria requiring treatment adjustment may be an issue in these patients. Cite this article as: Güven SC, Erden A, Küçük H, et al. Coronavirus disease 2019 outcomes in amyloid A protein amyloidosis secondary to rheumatic conditions and signs of post-coronavirus disease 2019 proteinuria progression. Eur J Rheumatol. Published online April 4, 2024. DOI:10.5152/eurjrheum.2024.23050.

Vascular Calcification in Takayasu Arteritis; Relationship with Metabolic Syndrome, Left Ventricular Mass Index and Intima-Media Thickness.

INTRODUCTION: ; The present study aims to determine the frequency of vascular calcification in Takayasu arteritis (TA) and the risk factors for it and to evaluate its relation with atherosclerotic predictors such as metabolic syndrome (MS), left ventricular mass index (LVMI) and carotid intima-media thickness (CIMT). METHOD: A cross-sectional study was conducted in patients with TA; MS was defined according to the US National Cholesterol Education Program/Adult Treatment Panel III (NCEP/ATP III) criteria. The study included 49 TA patients (22 with MS, 27 without MS) and 31 healthy controls (HCs). Non-contrast computed tomography measured calcification in coronary arteries, aorta, and branches. RESULTS: Forty-seven patients (95.9%) were female and mean age was 33.45±8.53 years. Total calcification score (mean±SD; 5223.9±18041.1 AU vs 35.87±72.70 AU (p=0.05)), CIMT and LVMI were found to be significantly higher in TA patients than HCs (p<0.05). While there was no significant difference between the total calcification score of MS (+) TA patients and MS (-) TA patients, in both patient groups, the total calcification score was found to be significantly higher than HCs. MS (+) and MS (-) groups were found to have significantly higher CIMT and LVMI values than the control group, in addition, MS (+) patients were found to have significantly higher LVMI and CIMT values than MS (-) group (p<0.05). CONCLUSION: ; Vascular calcification, CIMT, and LVMI are elevated in all TA patients, with greater impact in the presence of MS.

Assessment and comparison of the 2023 ACR/EULAR APS criteria with the revised Sapporo criteria.

OBJECTIVE: To analyze antiphospholipid antibody (aPL)-positive patients using the 2023 American College of Rheumatology/The European Alliance of Associations for Rheumatology (ACR/EULAR) antiphospholipid syndrome (APS) classification criteria and compare the revised Sapporo criteria and the 2023 ACR/EULAR criteria and evaluate whether the 2023 ACR/EULAR criteria provide added value over the revised Sapporo criteria. METHODS: In this descriptive study, 94 aPL-positive patients (with or without APS diagnosis) were identified from two hospital-based registries (Gazi and Hacettepe University). Patients were classified into four groups to compare both criteria sets. These four groups are as follows: (1) patients classified with only the revised Sapporo criteria; (2) patients classified with only the 2023 ACR/EULAR APS criteria; (3) patients classified with both two criteria sets; and (4) patients classified with neither two criteria set. RESULTS: Of the 94 patients, 11 were classified with only the revised Sapporo criteria; one with only the 2023 ACR/EULAR APS criteria; 52 with both criteria sets; and 30 with neither set of criteria. For these 94 patients, the operating characteristics of the 2023 ACR/EULAR APS criteria, using the revised Sapporo criteria as the gold standard, the 2023 ACR/EULAR APS entry criteria demonstrated 100% sensitivity, and the 2023 ACR/EULAR APS classification criteria demonstrated 98% specificity and 82.5% sensitivity. CONCLUSION: The study emphasizes the importance of recognizing differences in clinical manifestations, such as early pregnancy loss without severe preeclampsia (PEC) and/or severe placental insufficiency (PI) and calls for a nuanced discussion on anticardiolipin (aCL) and anti-beta 2-glycoprotein-I (anti-ß2GPI) immunoglobulin G (IgG) cutoff values.

Calcineurin inhibitors experience in unplanned pregnancies with active lupus disease: A retrospective observational study.

OBJECTIVES: To evaluate the use of calcineurin inhibitors (CNIs), specifically tacrolimus, in unplanned pregnancies with active lupus disease among patients with systemic lupus erythematosus (SLE). MATERIALS AND METHODS: The study includes data from pregnancies in women diagnosed with SLE at Gazi University Hospital in Ankara, Türkiye, between January 2010 and July 2022. The study categorized pregnancies into planned and unplanned groups based on lupus nephritis presence, emphasizing the need for inactive lupus disease for at least 6 months before attempting conception in planned pregnancies. The outcomes of pregnancies involving CNIs, particularly tacrolimus, were assessed. RESULTS: In our cohort comprising 632 SLE patients, 39 individuals reported 42 pregnancies. Among the 42 pregnancies, 14 have a history of lupus nephritis. We observed that 8 of 14 patients with a history of lupus nephritis had unplanned pregnancies. Three patients used cyclosporine and 2 used tacrolimus during their pregnancy; their pregnancies were completely healthy, and no lupus flare was observed during their pregnancies. The pregnancy of 2 patients who used azathioprine and 1 last patient who used no immunosuppressive treatment ended in abortion. CONCLUSION: This study reveals that tacrolimus can be effectively used in unplanned pregnancies with active lupus disease, providing favorable maternal and fetal outcomes. The findings emphasize the importance of considering CNIs, particularly tacrolimus, in the management of SLE pregnancies, even in cases of unplanned pregnancies with a history of lupus nephritis.

Colchicine-intolerant familial mediterranean fever patients: A comparative study between different colchicine doses and IL-1 inhibitor monotherapy.

OBJECTIVE: Familial Mediterranean fever (FMF) is the most common monogenic autoinflammatory disease. Colchicine is the primary treatment for FMF, although some patients do not respond well or are unable to tolerate it. For these patients, the addition of interleukin-1 (IL-1) antagonists is the preferred option. However, the impact of colchicine treatment alongside the use of IL-1 antagonists remains unclear. METHODS: We recruited adult FMF patients who satisfied the Eurofever and Pediatric Rheumatology International Trials Organization classification criteria and were receiving IL-1 antagonist treatment from our FMF cohort. All the patients exhibited colchicine intolerance or resistance. As per the FMF cohort protocol, the patients were longitudinally followed up, including assessments of their C-reactive protein, erythrocyte sedimentation rate, autoinflammatory disease activity index (AIDAI), and autoinflammatory damage index (ADDI). RESULTS: Among the 125 patients (68 female and 57 male), 96 received a combination of IL-1 antagonists and the maximum tolerated dose of colchicine, whereas 29 were treated exclusively with IL-1 antagonists due to colchicine intolerance. The patients' inflammatory markers, AIDAI activity, and ADDI damage scores did not differ significantly between the two groups during the follow-up period. Notably, the drug retention rates were significantly higher in the patients treated solely with IL-1 antagonists. CONCLUSION: While the typical approach is to maintain colchicine treatment alongside the use of IL-1 antagonists, for patients who cannot tolerate effective colchicine doses, IL-1 antagonists alone may effectively control FMF disease activity.

Non-criteria autoantibodies in antiphospholipid syndrome may be associated with underlying disease activity.

BACKGROUND: Antiphospholipid syndrome (APS) is a systemic autoimmune disorder characterized by persistent antiphospholipid antibodies (aPLs) with arterial and venous thrombosis and/or pregnancy morbidity. In recent years, several studies have highlighted the potential role of non-criteria aPL in diagnosing APS patients. AIM: This study aimed to determine the association of the presence of non-criteria aPL antibodies to the clinical and laboratory features of patients with a diagnosis of APS. METHODS: Eighty patients diagnosed with APS and under observation in the rheumatology clinic of Ankara City Hospital were assessed. Patient demographic and clinical features were meticulously recorded. Non-criteria antibodies tested in our center included antiphosphatidylserine IgA, antiphosphatidylserine IgM, beta 2 glycoprotein IgA, anti-cardiolipin IgA, antiphospholipid antibody IgG, and antiphospholipid antibody IgM. Antibodies from patients who were tested for at least one non-criteria antibody were documented. RESULTS: Out of 80 patients, 55 (68.8%) were tested for at least one non-criteria antibody, and 29 of those patients (52.7%) tested positive for at least one non-criteria antibody. The antiphospholipid antibody IgM and the beta 2 glycoprotein IgA were the most commonly tested non-criteria antibodies. Patients with non-criteria antibody positivity had a higher frequency of Ds DNA positivity and low complement (62.0% vs. 35.0%, p = 0.042; 69.0% vs. 38.0%, p = 0.023), respectively. In addition, positivity for anti-cardiolipin IgG and b2 glycoprotein IgG was significantly higher in the group positive for non-criteria antibodies (79% vs. 31%, p ≤ 0.001; 72.0% vs. 19%, p ≤ 0.001). There was no significant difference between the clinical features of patients with at least one positivity for non-criteria antibodies and those without. CONCLUSION: Systemic lupus erythematosus (SLE) is the most commonly associated disease with APS, being present in approximately 35% of cases [1]. Since the majority of the patient group in our study had APS that was secondary to SLE, non-criteria antibody positivity may be linked to the immunological activity of SLE. Large multicenter studies are necessary to investigate the clinical significance of isolated/combined positivity for criterion/non-criteria aPLs.

Tocilizumab as a first line biologic agent in rheumatoid arthritis patients with inadequate response to disease-modifying anti-rheumatic drugs: real life experience from the TReasure Registry.

OBJECTIVES: To evaluate the retention rate, treatment response and safety of tocilizumab (TCZ) as first-line biologic treatment in rheumatoid arthritis (RA) patients with inadequate response to disease-modifying anti-rheumatic drugs (DMARD-IR). METHODS: The TReasure Registry is a multicentre, web-based registry of RA and spondyloarthritis patients across Turkey. DMARD-IR RA patients who received TCZ as first-line biologic treatment were included in this registry for efficacy and safety. Demographic and clinical data, treatments, and adverse events were collected. Drug retention rate was estimated using Kaplan-Meier analysis. RESULTS: Among 642 RA patients who ever used TCZ, 258 DMARD-IR RA patients (male/female: 18.2%/81.8%, mean age, 54.41 years) received TCZ as first-line biologic. The median disease duration was 97 (range, 60-179) months and the median TCZ treatment duration was 15 (range, 6-28) months. At the 6th and 12th months of TCZ treatment, the decrease in disease activity scores from baseline was significant. The Kaplan-Meier analysis revealed the retention rate of TCZ at the 12th, 24th, 36th, and 60th months as 81.1%, 73.8%, 66.2%, and 63.6%, respectively. Fifty-seven (22%) patients discontinued TCZ; the main reason being primary or secondary inefficacy (n=29). CONCLUSIONS: Over 80% drug retention rate at 12th month of TCZ treatment in this real-world study was concordant with previously conducted TCZ clinical studies. Significant reductions not only in the disease activity score-28 but also in the simplified disease activity index (SDAI) and clinical disease activity index (CDAI) scores, along with health assessment questionnaire (HAQ) scores, supported the impact of TCZ in RA management with a good safety profile.

Is compressed colchicine tablet superior to other colchicine preparations in patients with familial Mediterranean fever?

AIMS: The aim of our study is to evaluate the differences in effectiveness, dosage, and side effect profiles in the use of colchicine preparations and evaluate the superiority of compressed colchicine tablets in familial Mediterranean fever (FMF) patients with resistance or intolerance to coated colchicine tablets. MATERIALS AND METHODS: Patients who were diagnosed with FMF according to the Tel Hashomer criteria, aged 18 years and older, and switched from compressed colchicine to coated colchicine tablets in the rheumatology clinic of Gazi University were identified. The daily colchicine dose and FMF attack frequency before and after switching from coated colchicine tablets to compressed colchicine tablets were compared. RESULTS: The study included 43 female (72.9%) and 16 male patients (27.1%), and the mean age was 34.54 ± 8.3 years. The number of attacks per year was significantly reduced after switching to compressed colchicine tablets, and daily colchicine doses were lower after switching to compressed colchicine tablets (1.97 ± 0.23 vs 1.78 ± 0.39 mg, p < 0.001). CONCLUSION: Compressed colchicine tablets were shown to be superior to other colchicine preparations and compressed colchicine tablets to be a useful treatment option before initiating biological agents in patients who were unresponsive to coated colchicine.

Epidemiological characteristics of hepatitis B and C in patients with inflammatory arthritis: Implications from treasure database.

Objectives: This study aimed to evaluate the hepatitis B (HBV) and C (HCV) frequency and clinical characteristics among patients with rheumatoid arthritis (RA) or spondyloarthritis (SpA) who receive biological treatments. Patients and methods: The observational study was conducted with patients from the TReasure database, a web-based prospective observational registry collecting data from 17 centers across Türkiye, between December 2017 and June 2021. From this database, 3,147 RA patients (2,502 males, 645 females; median age 56 years; range, 44 to 64 years) and 6,071 SpA patients (2,709 males, 3,362 females; median age 43 years; range, 36 to 52 years) were analyzed in terms of viral hepatitis, patient characteristics, and treatments used. Results: The screening rate for HBV was 97% in RA and 94.2% in SpA patients. Hepatitis B surface antigen (HBsAg) positivity rates were 2.6% and 2%, hepatitis B surface antibody positivity rates were 32.3% and 34%, hepatitis B core antibody positivity rates were 20.3% and 12.5%, HBV DNA (deoxyribonucleic acid) positivity rates were 3.5% and 12.5%, and antibody against HCV positivity rates were 0.8% and 0.3% in RA and SpA patients, respectively. The HBsAg-positive patients were older and had more comorbidities, including hypertension, diabetes, and coronary artery disease. In addition, rheumatoid factor (RF) positivity was more common in HBsAg-positive cases. The most frequently prescribed biologic disease-modifying antirheumatic drugs were adalimumab (28.5%), etanercept (27%), tofacitinib (23.4%), and tocilizumab (21.5%) in the RA group and adalimumab (48.1%), etanercept (31.4%), infliximab (22.6%), and certolizumab (21.1%) in the SpA group. Hepatitis B reactivation was observed in one RA patient during treatment, who received rituximab and prophylaxis with tenofovir. Conclusion: The epidemiological characteristics of patients with rheumatic diseases and viral hepatitis are essential for effective patient management. This study provided the most recent epidemiological characteristics from the prospective TReasure database, one of the comprehensive registries in rheumatology practice.

Could serum HMGB1 levels be a predictor associated with psoriatic arthritis?

Background/aim: Psoriasis is a chronic autoimmune disease that predominantly affects the skin and musculoskeletal system. We hypothesized that HMGB1, an inflammatory nuclear protein, may play a role in the musculoskeletal involvement of psoriasis. Methods: Forty patients with psoriasis and 45 with psoriatic arthritis were involved in the study; the results were compared with 22 healthy controls. Serum HMGB1 levels were evaluated from peripheral blood samples. Results: Serum HMGB1 levels were found to be significantly higher in patients with psoriasis regardless of joint involvement (p < 0.001). Also, HMGB1 levels were correlated with the extent of psoriasis. Conclusion: Serum HMGB1 levels may contribute to the progression of psoriasis to psoriatic arthritis and correlate with the severity of skin involvement.

Psoriasis is an autoimmune skin disease that may also affect the joints. Factors leading to the progression of psoriasis to psoriatic arthritis are still a mystery despite an increasing number of animal studies and real-life data. HMGB1 is a nuclear protein that leads to an increase in molecules that increase inflammation (TNF-α, IL-1 and IL-6) in the body. Until now, there was no report about the relationship between psoriatic arthritis and serum HMGB1 levels. Our study aimed to find any difference in HMGB1 levels between healthy and psoriatic patients. Psoriatic arthritis patients had higher levels of serum HMGB1 than patients with psoriasis. Also, HMGB1 levels were correlated with the severity of skin involvement. Our results showed that serum HMGB1 may indicate a high risk for developing psoriasis that involves the joints. Therefore the HMGB1 level in psoriasis patients can potentially serve as a predictor associated with disease severity and the risk of developing psoriatic arthritis.

Low relapse rate in patients with giant cell arteritis in a multi-centre retrospective Turkish Registry.

OBJECTIVES: Glucocorticoids (GC) are widely accepted as the standard first-line treatment for giant cell arteritis (GCA). However, relapse rates are reported up to 80% on GC-only protocol arms in controlled trials of tocilizumab and abatacept in 12-24 months. Herein, we aimed to assess the real-life relapse rates retrospectively in patients with GCA from Turkey. METHODS: We assembled a retrospective cohort of patients with GCA diagnosed according to ACR 1990 criteria from tertiary rheumatology centres in Turkey. All clinical data were abstracted from medical records. Relapse was defined as any new manifestation or increased acutephase response leading to the change of the GC dose or use of a new therapeutic agent by the treating physician. RESULTS: The study included 330 (F/M: 196/134) patients with GCA. The mean age at disease onset was 68.9±9 years. The most frequent symptom was headache. Polymyalgia rheumatica was also present in 81 (24.5%) patients. Elevation of acute phase reactants (ESR>50 mm/h or CRP>5 mg/l) was absent in 25 (7.6%) patients at diagnosis. Temporal artery biopsy was available in 241 (73%) patients, and 180 of them had positive histopathological findings for GCA. For remission induction, GC pulses (250-1000 methylprednisolone mg/3-7 days) were given to 69 (20.9%) patients, with further 0.5-1 mg/kg/day prednisolone continued in the whole group. Immunosuppressives as GC-sparing agents were used in 252 (76.4%) patients. During a follow-up of a median 26.5 (6-190) months, relapses occurred in 49 (18.8%) patients. No confounding factor was observed in relapse rates. GC treatment could be stopped in only 62 (23.8%) patients. Additionally, GC-related side effects developed in 64 (24.6%) patients, and 141 (66.2%) had at least one Vasculitis Damage Index (VDI) damage item present during follow-up. CONCLUSIONS: In this first multi-centre series of GCA from Turkey, we observed that only one-fifth of patients had relapses during a mean follow-up of 26 months, with 76.4% given a GC-sparing IS agent at diagnosis. At the end of follow-up, GC-related side effects developed in one-fourth of patients. Our results suggest that patients with GCA had a low relapse rate in real-life experience of a multi-centre retrospective Turkish registry, however with a significant presence of GC-associated side effects during follow-up.

The Possible Relation of Demodex and Facial Erythema in Connective Tissue Diseases.

Background: We aimed to investigate the frequency of Demodex infestation and clinical implications in connective tissue disease patients with facial erythema. Methods: Patients diagnosed with a connective tissue disease and had facial erythema were consecutively enrolled in the study from 2019-2020. An age and gender matched control group was formed from healthy volunteers. Presence of Demodex was investigated by standardized skin surface biopsy. Number of Demodex mites over 5 per centimeter square was considered meaningful for infestation. Topical or systemic metronidazole treatment was given to the connective tissue disease patients with Demodex infestation. Facial erythema visual analog scale was questioned in patients at treatment onset and one month after. Results: A total of 31 connective tissue disease patients with facial erythema were enrolled. Control group included 31 healthy volunteers. Demographics and comorbidities were similar between groups. Demodex infestation was present in 58.1% of the disease group and in 25.8% of the control group (P=0.01). Pruritus was the most common symptom in patients with infestation. Median (IQR) facial erythema visual analog scale score was 6 (3) at treatment onset and was 2 (2.5) one month later (P<0.001). Conclusion: When evaluating facial cutaneous lesions, Demodex infestation should not be overlooked in a patient group like connective tissue diseases with dysfunctional immune system.

Diffuse generalized venulitis as the primary pathology of Behçet's disease: A comprehensive magnetic resonance venography study.

OBJECTIVE: Behçet's Disease (BD) is a chronic multisystem vasculitis that manifests with destructive inflammation affecting the eyes, central nervous system, and blood vessels. The pathology of vein involvement in BD is poorly characterized. Magnetic resonance (MR) venography gives more comprehensive information about deep veins and adjacent tissues. In this study, we aimed to characterize vein involvement and evaluate the diagnostic utility of MR venography in BD. METHODS: Sixty-five BD patients who fulfilled the International Study Group (ISG) criteria and 20 healthy control subjects were enrolled. Inferior vena cava (IVC), common iliac veins (CIV), external (EIV) and internal iliac veins (IVV), common femoral veins (CFV), femoral veins (FV), and greater saphenous veins (GSV) of BD patients and healthy controls were evaluated with MR venography and ultrasonography for the presence pathologic features, luminal thrombi, vessel wall changes, and perivascular abnormalities. RESULTS: 33 vascular and 32 non-vascular BD patients (mean age 39.3 ± 11.3 years and 48 [73.8%] male) were enrolled. MR venography revealed diffuse concentric thickening of the walls of IVC, CIV, EIV, IIV, CFV, FV, and GSV in BD (healthy controls vs. BD p<0.05 for all vein segments). MR venography provided additional information about veins and perivascular tissues like contrast enhancement, enlarged lymph nodes, and seminal vesicle vascularization, which were remarkably more frequent in vascular BD than non-vascular BD and healthy controls. CONCLUSION: The results of our study suggest that the involvement of the venous system is diffuse and generalized in BD, and demonstration of venulitis might help diagnose the disease.

Long-Term Results of Immunogenicity of Booster Vaccination against SARS-CoV-2 (Hybrid COV-RAPEL TR Study) in Turkiye: A Double-Blind, Randomized, Controlled, Multicenter Phase 2 Clinical Study.

The immunogenicity of vaccines decreases over time, causing a need for booster doses. This study aimed to present the long-term (Day 84) immunogenicity results of the double-blind, randomized, controlled, phase II Hybrid COV-RAPEL TR Study (NCT04979949), in which the TURKOVAC or CoronaVac vaccines were used as a booster after the second dose of primary vaccination with CoronaVac. A total of 190 participants from the Hybrid COV-RAPEL TR Study, who had both Day 28 and Day 84 immunogenicity results, were included. The immunogenicity on Day 84, regarding the neutralizing antibody positivity (Wuhan and Delta variants) and anti-spike immunoglobulin (Ig) G (IgG) antibody positivity, was compared between TURKOVAC and CoronaVac vaccine arms according to sex and age groups. Overall, antibody positivity showed a slight decrease on Day 84 vs. Day 28, but was not different between TURKOVAC and CoronaVac arms either for sexes or for age groups. However, TURKOVAC produced better antibody response against the Delta variant than CoronaVac, while CoronaVac was superior over TURKOVAC regarding neutralizing antibody positivity in the 50-60 years age group, regardless of the variant. A single booster dose, after the completion of the primary vaccination, increases antibody positivity on Day 28 which persists until Day 84 with a slight decrease. However, an additional booster dose may be required thereafter, since the decrease in antibody titer may be faster over time.

Pulmonary hypertension in Behçet's disease: echocardiographic screening and multidisciplinary approach.

BACKGROUND: Little is known about the prevalence and causes of pulmonary hypertension (PH) in Behçet's disease (BD). This study was conducted to determine the prevalence and causes of PH in BD. METHODS: In this descriptive study, we screened 154 patients with BD for PH using transthoracic echocardiography between February 2017 and October 2017. An estimated systolic pulmonary arterial pressure (sPAP ≥ 40 mmHg) was used as the cutoff value to define PH. Patients with BD were categorized into 5 groups according to organ involvement including mucocutaneous/ articular, ocular, vascular, gastrointestinal, and neurologic involvement. Additional laboratory and imaging results were obtained from hospital file records to determine the causes of PH. RESULTS: PH was detected in 17 (11%) patients. Nine (52.9%) of these patients had group II PH (due to left heart disease), 4 (23.5%) had IV PH (due to pulmonary arterial involvement), and 1 had III PH (due to chronic obstructive lung disease). The frequency of PH was higher in BD patients with vascular involvement than those without (52.9% vs 28.5%; p = 0.04). Among 10 patients with pulmonary artery involvement (PAI) 4 (40%) had PH. Although the vascular BD group had the highest rate of PH, we observed no statistically significant difference in the frequency of PH between the predefined BD subgroups. DISCUSSION: : PH is not rare in patients with BD. The majority of BD patients with PH are in group II or IV PH. Patients with vascularinvolvement carry a higher risk for the development of PH. Monitoring BD patients with PAI should be considered for the development of group IV PH.

The predictors of paradoxical reactions, especially psoriasis, to biologic therapy-findings from the TReasure database: a 5-year follow-up study.

OBJECTIVES: The objectives of this study were to assess the clinical characteristics, predictive factors, and practical algorithms of paradoxical reactions (PRs), specifically paradoxical psoriasis (PP). METHODS: The TReasure database is a web-based prospective observational cohort comprised of patients with RA and SpA from 17 centres around Turkey since 2017. A cohort study and a case-control study nestled within the cohort were identified. RESULTS: In total, 2867 RA and 5316 SpA patients were evaluated. The first biologic agent was found to have caused PRs in 60% of the 136 patients (1.66%) who developed the PRs. The median time interval between the PRs and biological onset was 12 months (range 1-132 months, mean 21 months). The most common types of PP, constituting 92.6% of PRs, were pustular (60.3%) and palmoplantar (30.9%). Adalimumab (30.9%), infliximab (19%) and etanercept (17.4%) were the most common agents causing the PP. In the treatment of most PP patients (73.2%), switching biologic agents was favoured, with TNF inhibitor (TNFi) chosen in 46.03% and non-TNFi in 26.9% of cases. The three most frequently selected drugs were etanercept (24.6%), secukinumab (9.5%) and adalimumab (8.7%). Only 5.17% of patients who switched to another TNFi showed progression. The odds ratios (s) for SSZ, HCQ, and LEF use were significantly higher in RA controls than in PP patients (P = 0.033, OR = 0.15; P = 0.012, OR = 0.15; and P = 0.015, OR = 0.13, respectively). In the PP group with SpA, the number of smokers was significantly higher (P = 0.003, OR: 2.0, 95% CI: 1.05, 3.81). CONCLUSION: Contrary to expectations based on earlier research suggesting that paradoxical reactions develop with the class effect of biological agents, the response of patients who were shifted to another TNFi was favourable.

Tofacitinib experience in patients with enteropathic arthritis.

Background/aim: The JAK-STAT pathway is involved in the pathogenesis of both the inflammatory bowel disease (IBD) and spondyloarthropathy group of diseases. The purpose of this study was to evaluate the effectiveness of tofacitinib, a Janus kinase inhibitor, in the treatment of enteropathic arthritis (EA). Materials & methods: The study included seven patients, four from the authors' follow-up and three from the literature. All cases were recorded for demographic characteristics, comorbidities, IBD and EA symptoms, medical treatments and changes in clinical and laboratory results with treatment. Results & conclusion: Clinical and laboratory remission in terms of IBD and EA was achieved in three patients after tofacitinib treatment. For both spondyloarthritis spectrum diseases and IBD, tofacitinib may be an appropriate choice, as its efficacy has been shown in both conditions.

Tofacitinib, which inhibits the JAK enzyme, is an oral, nonbiologic, disease-modifying drug used in the treatment of rheumatologic diseases. Tofacitinib modulates the immune response and reduces or prevents inflammation. There are limited data on the efficacy of tofacitinib for the treatment of enteropathic arthritis. This study aimed to evaluate the efficacy of tofacitinib in the treatment of enteropathic arthritis. Seven patients, four from the authors' follow-up and three from the literature, were included in the study. All cases were recorded in terms of demographic characteristics, comorbidities, symptoms of inflammatory bowel disease and enteropathic arthritis, medical treatments and changes in treatment and clinical and laboratory results. After tofacitinib treatment, clinical and laboratory improvement was achieved in three patients. As a result, tofacitinib may be a suitable choice for enteropathic arthritis.

Semaphorin 3A levels in vascular and nonvascular phenotypes in systemic sclerosis.

OBJECTIVE: Semaphorin 3A (Sema3A) plays a regulatory role in immune responses. The aim of this study was to evaluate Sema3A levels in patients with systemic sclerosis (SSc), especially in major vascular involvements such as digital ulcer (DU), scleroderma renal crisis (SRC), pulmonary arterial hypertension (PAH), and to compare Sema3A level with SSc disease activity. METHODS: In SSc patients, patients with DU, SRC, or PAH were grouped as major vascular involvements and those without as nonvascular, and Sema3A levels were compared between the groups and with a healthy control group. The Sema3A levels and acute phase reactants in SSc patients, as well as their association with the Valentini disease activity index and modified Rodnan skin score, were evaluated. RESULTS: The Sema3A values (mean ±â€…SD) were 57.60 ±â€…19.81 ng/mL in the control group (n = 31), 44.32 ±â€…5.87 ng/mL in patients with major vascular involvement SSc (n = 21), and 49.96 ±â€…14.00 ng/mL in the nonvascular SSc group (n = 35). When all SSc patients were examined as a single group, the mean Sema3A value was significantly lower than controls (P = .016). The SSc with major vascular involvement group had significantly lower Sema3A levels than SSc with nonmajor vascular involvement group (P = .04). No correlation was found between Sema3A, acute phase reactants, and disease activity scores. Also, no relationship was observed between Sema3A levels and diffuse (48.36 ±â€…11.47 ng/mL) or limited (47.43 ±â€…12.38 ng/mL) SSc types (P = .775). CONCLUSION: Our study suggests that Sema3A may play a significant role in the pathogenesis of vasculopathy and can be used as a biomarker in SSc patients with vascular complications such as DU and PAH.