Efficacy of rituximab on disease activity, severity, and disease-related damage in patients with immunosuppressive-resistant systemic sclerosis.

Background/aim: B-cell depletion with rituximab (RTX) is widely used as a rescue therapy in patients with systemic sclerosis (SSc). The aim herein was to analyze the progress of disease-related outcomes after RTX therapy in severe SSc patients.

Materials And Methods: Included in this study were 27 SSc patients who were followed-up between 2012 and 2020 and received at least 1 cycle of RTX for active disease, despite receiving standard immunosuppressives (ISs).

Course and Prognosis of AA Amyloidosis in Patients with Psoriatic Arthritis: Report of Three Cases from a Single Center Cohort and Review of the Literature.

Objective: Herein, we aimed to evaluate the frequency and clinical features of AA amyloidosis in patients with PsA followed up in our tertiary referral clinic.

Methods: We retrospectively evaluated PsA patients classified according to CASPAR classification criteria followed-up in our tertiary referral clinic for AA amyloidosis. The literature search was also done by three independent researchers using the keywords "psoriatic arthritis AND amyloidosis", "spondyloarthritis AND amyloidosis", "AA amyloidosis", "secondary amyloidosis".

A Case of Budd-Chiari Syndrome Associated With Antiphospholipid Syndrome Treated Successfully by Transjugular Intrahepatic Portosystemic Shunt.

Budd Chiari syndrome (BCS) is defined as obstruction of hepatic venous outflow that can be located anywhere from small hepatic venules up to the entrance of inferior vena cava (IVC) into right atrium. Etiologies of BCS include myeloproliferative disorders, congenital, and acquired hypercoagulable states. Anticoagulation is the mainstay of treatment for all cases of BCS with a demonstrable hypercoagulable state.

A single center survey study of systemic vasculitis and COVID-19 during the first months of pandemic.

Introduction: COVID-19 pandemic created concerns among patients receiving immunosuppressive therapy. Frequency of COVID-19 and impact of lockdown on treatment compliance in patients with vasculitis are largely unknown.

Patients And Method: Patients with ANCA-associated and large vessel vasculitis that have been followed-up in our clinic were contacted by phone and a questionnaire containing home isolation status, treatment adherence and history of COVID -19 between March 1st and June 30th, 2020 was applied.

A shared motif of hla-dpb1 affecting the susceptibility to pr3-anca positive granulomatosis with polyangiitis: comparative analysis of a Turkish cohort with matched healthy controls.

We aimed to analyse the distribution of HLA Class 2 genotypes which were reported among the genetic risk factors for ANCA-associated vasculitis (AAV) among Turkish patients in comparison with healthy subjects and previously reported data of AAV cohorts. Ninety-eight patients (F/M: 47/51 and mean age: 49 ± 1.14) were enrolled in the study and records of gender and birthplace-matched 196 healthy kidney donors were used as the control group.

Long-term follow-up of 89 patients with giant cell arteritis: a retrospective observational study on disease characteristics, flares and organ damage.

The objective of the study is to investigate the clinical characteristics and long-term prognosis including flares and organ damage in patients with giant cell arteritis (GCA) from a tertiary referral centre and compare these features in different subgroups. In this retrospective observational study, patients with GCA who were followed up in our vasculitis clinic between 1998 and 2018 were evaluated by a predefined protocol. Patients with and without cranial symptoms were compared for clinical and laboratory features, flares and permanent damage findings.

Validation of the adjusted global antiphospholipid syndrome score in a single centre cohort of APS patients from Turkey.

The adjusted global antiphospholipid syndrome score (aGAPSS) is a recently developed thrombotic risk assessment score that considers the antiphospholipid antibody (aPL) profile and conventional cardiovascular risk factors. In this retrospective study, we aimed to evaluate the validity of the aGAPSS in predicting clinical manifestations (criteria and extra-criteria) of antiphospholipid syndrome (APS) in a single centre cohort of patients. Ninety-eight patients with APS ± systemic lupus erythematosus (SLE) were classified according to clinical manifestations as vascular thrombosis (VT), pregnancy morbidity (PM) or both (VT + PM).

Comparison of Disease Characteristics, Organ Damage, and Survival in Patients with Juvenile-onset and Adult-onset Systemic Lupus Erythematosus in a Combined Cohort from 2 Tertiary Centers in Turkey.

Objective: Age at onset has been shown to affect the clinical course and outcome of systemic lupus erythematosus (SLE). Herein, we aimed to define the differences in clinical characteristics, organ damage, and survival between patients with juvenile-onset (jSLE) and adult-onset SLE (aSLE).

Methods: For the study, 719 patients (76.

Criteria sets for primary Sjogren's syndrome are not adequate for those presenting with extraglandular organ involvements as their dominant clinical features.

Patients with primary Sjogren's syndrome (pSS) may go undiagnosed or be misclassified due to the insidious nature and wide spectrum of the disease. The available several classification criteria emphasize glandular findings. We aimed to analyze the efficiency of various classification criteria sets in patients diagnosed on the clinical basis by expert opinion and to compare those pSS patients who fulfilled these criteria with those who did not.

Remarkable damage along with poor quality of life in Takayasu arteritis: cross-sectional results of a long-term followed-up multicentre cohort.

Objectives: We aimed to assess the outcome of a large Takayasu arteritis (TAK) cohort using the vasculitis damage index (VDI) and quality of life (QoL) scale, tools which have been validated for vasculitis.

Methods: Disease activity, damage and QoL were cross-sectionally evaluated in 165 TAK patients from 6 centres. SF-36 were applied to 51 age-matched healthy controls (HC).

The relationship between vascular biomarkers and disease characteristics in systemic sclerosis: elevated MCP-1 is predominantly associated with fibrotic manifestations.

Objectives: To determine the relationship between vascular biomarkers reflecting the vascular injury and organ involvement in systemic sclerosis (SSc).

Methods: Seventy-two SSc patients (66 female) fulfilling 2013 ACR/EULAR Criteria were evaluated. Serum samples of patients were collected for flow-cytometric analysis of sCD40L, tPA, MCP-1, sE-selectin, IL-8, IL-6, VEGF, sP-selectin, TGF-β1 and VCAM levels (Bender MedSystems) in SSc patients and 20 healthy controls.

Capillaroscopic findings and vascular biomarkers in systemic sclerosis: Association of low CD40L levels with late scleroderma pattern.

Objective: To determine the relationship between vascular biomarkers reflecting the vascular injury and neoangiogenesis with capillaroscopic changes in systemic sclerosis (SSc).

Methods: Nailfold video-capillaroscopy (NVC) was performed qualitatively (early, active and late scleroderma patterns) in 72 SSc patients (66 female) fulfilling ACR/EULAR (2013) criteria. Serum samples of patients were collected and analysed by flow cytometer with multiplex kits of sCD40L, tPA, MCP-1, sE-selectin, IL-8, IL-6, VEGF, sP-selectin, TGF-β1 and VCAM at the same time with NVC.

The relationship between nailfold capillaroscopic assessment and telangiectasia score with severity of peripheral vascular involvement in systemic sclerosis.

Objectives: To determine the association of nailfold video-capillaroscopy (NVC) findings and telangiectasia score with digital ulcer (DU) history and severity of peripheral vascular involvement (PVI) in systemic sclerosis (SSc).

Methods: Fifty-nine SSc patients fulfilling Leroy & Medsger criteria were evaluated including telangiectasia score, disease activity and severity scores. NVC was performed according to qualitative (early, active and late patterns) and semi-quantitative assessments.

Cluster analysis of autoantibodies in 852 patients with systemic lupus erythematosus from a single center.

Objective: Associations between autoantibodies and clinical features have been described in systemic lupus erythematosus (SLE). Herein, we aimed to define autoantibody clusters and their clinical correlations in a large cohort of patients with SLE.

Methods: We analyzed 852 patients with SLE who attended our clinic.

Re-evaluation of 129 patients with systemic necrotizing vasculitides by using classification algorithm according to consensus methodology.

Recently, a new classification algorithm (CA) for systemic necrotizing vasculitides was proposed by Watts et al. (Annals Rheum Dis 66:222-227, 2007) by using the American College of Rheumatology (ACR), Chapel Hill Consensus Criteria (CHCC) and Sorensen surrogate markers (So). We aimed to validate CA in our patients.

Risk factors for avascular bone necrosis in patients with systemic lupus erythematosus.

The objective was to investigate the predictive factors for avascular necrosis (AVN) of bone in patients with systemic lupus erythematosus (SLE). The records of 868 patients with SLE from four centers were reviewed retrospectively. Forty-nine patients with AVN were identified.

Association of familial Mediterranean fever-related MEFV variations with ankylosing spondylitis.

Objective: The pathogenesis of ankylosing spondylitis (AS) has a strong genetic contribution. Familial Mediterranean fever (FMF) is an autosomal recessively inherited autoinflammatory disorder caused by MEFV gene missense variations, and a clinical association between FMF and AS has been reported previously. The aim of this study was to analyze the association of common MEFV variations (M694V, M680I, V726A, and E148Q) with AS in a group of Turkish patients.

Serologic response to Epstein-Barr virus antigens in patients with systemic lupus erythematosus: a controlled study.

Previous studies showed a link between systemic lupus erythematosus (SLE) and Epstein-Barr virus (EBV) infection. We sought to determine the features of serologic response to EBV in SLE patients and whether this response differs from those of systemic sclerosis (SSc) and primary antiphospholipid syndrome (PAPS) patients as well as healthy individuals. Sera from 198 consecutive SLE patients have been tested to detect IgG antibodies to EA/D, EBNA-1, VCA P18 and for comparison, cytomegalovirus (CMV) using commercially available ELISA kits (Trinity Biotech, USA).

Pulmonary hypertension in systemic lupus erythematosus: relationship with antiphospholipid antibodies and severe disease outcome.

Pulmonary hypertension (PH) in systemic lupus erythematosus (SLE) is associated with an unfavorable prognosis. We investigated the characteristics of SLE patients with PH. The patients with a pulmonary artery systolic pressure more than 30 mmHg at rest on echocardiogram were diagnosed with PH.

Predictors of damage and survival in patients with Wegener's granulomatosis: analysis of 50 patients.

Objective: To evaluate damage features and impact on survival by Vasculitis Damage Index (VDI) in a cohort of Turkish patients with Wegener's granulomatosis (WG).

Methods: We enrolled 50 (25 female) patients with WG according to ACR criteria. Birmingham Vasculitis Activity Score (BVAS) and VDI were used to analyze disease activity and damage.

Clinical evaluation and comparison of different criteria for classification in Turkish patients with psoriatic arthritis.

Several criteria are being used for the classification of psoriatic arthritis (PsA) and there is a lack of consensus about PsA as a separate entity. Our aim is to investigate the clinical features of our patients with a clinical diagnosis of PsA, compare the sensitivities of different classification criteria, agreement between the criteria. In this study 86 PsA patients were investigated (48 females, mean age 44).

Low frequency of HLA-B27 in ankylosing spondylitis patients from Turkey.

Objectives: Ankylosing spondylitis is strongly associated with HLA-B27. However, the strength of the association with HLA-B27 and the clinical features may vary in different parts of the world. The aim of this study is to compare the clinical features of AS and the frequencies of HLA-B27 and its alleles in patients from Turkey with other series.