The role of fatty acids in patients with Behçet's disease and their association with thrombosis.

Behçet's disease (BD) is a systemic disease with unknown etiopathogenesis and varying disease presentations. Fatty acids (FA) are essential biological compounds that are involved in complex metabolic pathways. They may contribute to inflammation and endothelial dysfunction by participating in many signaling pathways. Increased FAs levels are associated with an increased risk for various diseases. This study aimed to determine the relationship between FA, BD, and thrombotic complications. A total of 97 patients were recruited from the rheumatology department of a single center as a case-control study. The participants were divided into three groups: 36 patients with BD with thrombosis (Group 1), 24 patients with BD without thrombosis (Group 2), and 37 age- and sex-matched controls (Group 3). The analysis of 37 different FA with carbon numbers in the range of (4:0) and (24:1) in the samples were analyzed and compared between groups. Myristic acid (MA), methyl eicosatrienoate, and stearic acid (STA) levels were found to be significantly higher in BD with thrombosis than in BD without thrombosis, and palmitic acid (PA) levels were significantly higher in BD with thrombosis than in healthy individuals. MA was found to be a significant marker for differentiating between thrombotic BD. PA and STA are important markers for detecting thrombotic BD. In BD, lipotoxicity created by FA, such as PA, STA, and MA, plays a role as an inducer of inflammation and thrombosis through various mechanisms.

Unintentional Monotherapy in Rheumatoid Arthritis Patients Receiving Tofacitinib and Drug Survival Rate of Tofacitinib.

OBJECTIVE: To determine the rate of unintentional monotherapy (UM; switching to monotherapy from combination therapy of patients' own volition) in rheumatoid arthritis patients receiving tofacitinib and to evaluate tofacitinib survival rate. METHODS: This national, multicenter study included patients' data from the TURKBIO Registry. Demographics, clinical characteristics, disease duration and activity, comorbidities, and treatments were analyzed. RESULTS: Data of 231 rheumatoid arthritis patients (84.8% female, median age, 56 years) were included; 153 were initially prescribed combination therapy and continued to their therapies; 31 were initially prescribed combination therapy but switched to monotherapy on their own volition (UM); 21 were initially prescribed monotherapy and switched to combination therapy; 26 were initially prescribed monotherapy and continued to their therapies. The rate of comorbidities at the time of data retrieval was higher in the UM group than in the combination group (83.3% vs. 60.3%, p = 0.031). Presence of comorbidities was a significant factor affecting switching to monotherapy ( p = 0.039; odds ratio, 3.29; 95% confidence interval, 1.06-10.18). The combination and UM groups did not differ regarding remission rate assessed by Disease Activity Score 28-joint count C-reactive protein (60.5% and 70%, respectively; p = 0.328). Drug survival rates of the UM and combination groups did not differ. The median drug survival duration of tofacitinib was 27+ months with 1- and 4-year drug survival rates of 89.6% and 60.2%, respectively, in the UM group. CONCLUSIONS: Although 13.4% of the study population started monotherapy unintentionally, drug survival and remission rates of the UM and combination groups were not different. Comorbidity was a factor affecting transition from combination therapy to monotherapy.

Assessment of serum neopterin and calprotectin as biomarkers for subclinical inflammation in patients with familial Mediterranean fever.

BACKGROUND: Familial Mediterranean fever (FMF) is the most common autoinflammatory disease characterized by short, repeated, and self-limiting attacks of fever and serositis. Subclinical inflammation can persist in the periods with no symptoms and result in amyloidosis even with colchicine treatment. Neopterin and calprotectin have been considered essential players in inflammation and immune response. AIM: The study was aimed to measure serum levels of neopterin and calprotectin in patients with FMF in the attack-free period. METHODS: A total of 160 participants were recruited from the rheumatology department in this single-center, case-control study. Individuals having the inclusion criteria were divided into healthy controls (n = 80) and FMF (n = 80). The laboratory data were acquired from the electronic registration database. Serum calprotectin and neopterin were measured with ELISA test kits. FMF patients and healthy controls' laboratory findings were compared. RESULTS: FMF patients' serum red cell distribution width (RDW), calprotectin, and neopterin values were significantly higher compared to healthy controls. There were no statistically significant differences between calprotectin and neopterin regarding gender, family history, and colchicine response of the FMF patients. CONCLUSIONS: Calprotectin, neopterin, and RDW can be valuable marker candidates to be used in the follow-up of subclinical inflammation in FMF patients.

Diagnostic performance of gray-scale ultrasound and shear wave elastography in assessing salivary gland involvement in patients with primary Sjögren's syndrome.

PURPOSE: To investigate the diagnostic performance of gray-scale ultrasound (US) and shear wave elastography (SWE) for determining salivary gland involvement primary Sjögren's syndrome (pSS). METHODS: In this prospective study, the salivary glands of 72 healthy volunteers and 74 participants with pSS were examined by two blinded radiologists with consensus using gray-scale US and SWE. SWE parameters were compared between groups. The area under the curve (AUC), sensitivity, and specificity of gray-scale US and SWE was analyzed. The correlation between SWE and clinical findings was investigated. RESULT: The SWE parameters of the parotid and submandibular glands were significantly higher in the pSS group, but did not differ significantly based on serologic assays, Schirmer test, minor salivary gland biopsy, and comorbidities. The AUC values for gray-scale US of the salivary glands were significantly lower than the AUC values for SWE. The elasticity modulus (kPa) of parotid gland had the highest AUC value (0.937; 95% CI, 0.901-0.973), with a sensitivity of 93.2% and a specificity of 83.3%. SWE had no correlation with age, disease duration, laboratory values, or disease activity. CONCLUSION: SWE provides excellent diagnostic performance for submandibular and parotid gland involvement in pSS and can be used to complement gray-scale US.

Kynurenine pathway of tryptophan metabolism in patients with familial Mediterranean fever.

BACKGROUND: Familial Mediterranean fever (FMF) is an autoinflammatory syndrome characterized by recurrent episodes of fever and aseptic polyserositis. Subclinical inflammation generates a hidden threat to the development of FMF complications such as amyloidosis in attack-free intervals. The kynurenine pathway (KP) has been considered an important player in inflammation and immune response. The study was aimed to measure serum levels of KP metabolites in patients with FMF in the attack-free period. METHODS: A total of 161 participants were recruited from the rheumatology department in this single-centre, case-control study. Participants meeting the eligibility criteria were divided into healthy controls (n = 80) and FMF (n = 81). The laboratory data were obtained from the electronic registration database. Serum tryptophan (TRP), kynurenine (KYN), kynurenic acid (KYNA), 3-hydroxyanthranilic acid, 3-hydroxykynurenine (3HK), and quinolinic acid (QUIN) concentrations were measured with tandem mass spectrometry. Laboratory findings of FMF patients and healthy controls subjects were compared and evaluated. RESULTS: Serum TRP and KYNA levels were significantly decreased in both FMF groups compared to the control group, while the levels of KYN, QUIN, 3HK, the KYN/TRP ratio, and red cell distribution width were higher. CONCLUSION: TRP degradation by the KP is increased in patients with FMF. KP metabolites can be useful in demonstrating subclinical inflammation.

Diagnostic Performance of Lower Extremity Venous Wall Thickness and Laboratory Findings in the Diagnosis of the Behçet Disease.

BACKGROUND/OBJECTIVE: Behçet disease (BD) is not a single unique entity but a syndrome with different clinical phenotypes that can involve arterial and venous vessels of all sizes. To date, there has been no specific test or serum marker to measure and determine the severity of BD, and diagnosis remains based on clinical findings. This study aimed to assess lower extremity venous wall thickness (VWT) measured by ultrasound and laboratory findings and diagnostic performance in patients with BD. METHODS: A total of 106 participants were recruited from the rheumatology department in this single-center, case-control study. Participants meeting the eligibility criteria were divided into healthy controls (n = 52) and BD (n = 54). The VWT values of the common femoral vein, great saphenous vein, and popliteal vein were measured using ultrasonography. Laboratory data were obtained from the electronic registration database. Venous wall thicknesses and laboratory findings in patients with BD and healthy subjects were compared. RESULTS: Venous wall thickness of the lower extremity veins was higher in the BD group and higher in those with a history of deep vein thrombosis than in those without. The mean leukocyte, monocyte, erythrocyte sedimentation rate (ESR), C-reactive protein, plateletcrit (PCT), red cell distribution width (RDW), mean platelet volume (MPV) values, and monocyte-to-lymphocyte ratio (MLR) were higher in BD patients than in the control group. There was a correlation among increased VWT, ESR, PCT, MPV, RDW, and MLR. CONCLUSIONS: C-reactive protein, ESR, MPV, PCT, MLR, RDW, and VWT can be used to assist in the diagnosis of BD.

Elevated levels of neopterin and pentraxin 3 in patients with rheumatoid arthritis.

OBJECTIVES: As a systemic inflammatory disease, rheumatoid arthritis (RA) is the most common inflammatory arthritis in the population and there is no specific diagnostic marker in laboratory tests. The purpose of the study was to determine whether serum neopterin and pentraxin 3 (PTX3) levels may be a marker of increased inflammation in RA patients. MATERIALS AND METHODS: The study were consist of 30 RA patients and 30 healthy controls who were admitted to the department of rheumatology. Blood specimens were taken from both group, and the levels of neopterin were analyzed by chromatography method (HPLC) and the PTX 3 levels were measured by enzyme-linked immunosorbent assay (ELISA). All data and demographic characteristics of participants were also recorded. RESULTS: Serum neopterin and PTX 3 levels of the patient group (25.99 ± 7.24 ng/mL and 4.19 ± 1.01 ng/dL, respectively) was higher than the control group (9.55 ± 0.74 ng/mL and 2.23 ± 0.39 ng/dL, respectively). These results were remarkable significant (p<0.01). No statistically significant correlation was found between age-PTX 3, age-neopterin and PTX 3-neopterin parameters in the patient group. In the control group, a significant negative correlation was found between age and PTX 3 (p<0.05), and a positive correlation between neopterin and PTX 3. CONCLUSIONS: Consequently, the serum neopterin and PTX 3 levels were higher in RA patients as compared to the healthy individuals. Our study suggest that there is a relation between neopterin and PTX 3 levels with RA patients. These findings suggest that neopterin and PTX 3 are important markers in the monitoring of RA disease.

Evaluation of diagnostic performance of haematological parameters in Behçet's disease.

OBJECTIVE: Behçet's Disease (BD) is a polygenic and chronic autoinflammatory multisystemic vasculitis disease characterised by mucocutaneous, musculoskeletal, neurological, gastrointestinal and ophthalmologic lesions. There has been no specific test or serum marker to measure and determine the diagnosis and severity of BD. PURPOSE: The study aimed to investigate the diagnostic performance of haematological parameters as MLR (monocyte to lymphocyte ratio), NLR (neutrophil to lymphocyte ratio), PLR (platelet to lymphocyte ratio), MPV (mean platelet volume), MPVPR (mean platelet volume to platelet ratio), LMR (lymphocyte to monocyte ratio), LPM (lymphocyte and platelet multiplication), WLP (lymphocyte and leukocyte multiplication), RDW (red blood cell distribution width) and PCT (plateletcrit) in BD and compare these with disease activity and clinical findings. METHODS: A total of 266 participants (49 healthy control and 217 BD patients) were recruited from the rheumatology department in a single-centre as a case-control study. The laboratory data were obtained from the electronic registration database. BD Activity scores (BDCAF/Behcet's Disease Current Activity Form) were calculated. Laboratory findings of BD patients and healthy controls were compared and evaluated. RESULTS: RDW, Platelet, PCT, NLR and PLR values were significantly higher in patient group than in the healthy controls. However, haemoglobin, MPVPR and LMR were significantly lower in the patient group which compared with the healthy controls. LPM in BD with genital ulcers, WLP in BD with genital ulcers and arthritis, MPR in BD with uveitis, RDW in BD with thrombosis and neuro-Behçet's disease (NBD), PLR in NBD were observed to be higher. However, LMR in NBD and MPV in BD with thrombosis were lower than those without. There was a positive correlation between BDCAF score and RDW, and NLR. CONCLUSION: Haemoglobin, RDW, Platelet, PCT, NLR, LMR, PLR and MPVPR were statistically significant predictors for BD. RDW, PCT and NLR are the most valuable predictors for BD.

Evaluation of serum interleukin-6 (IL-6), IL-13, and IL-17 levels and computed tomography finding in interstitial lung disease associated with connective tissue disease patients.

OBJECTIVE: Interstitial lung disease (ILD) is one of the most severe complications which is associated with connective tissue disease (CTD) and causes to morbidity and mortality. So, we aimed to determine serum levels of interleukin-6 (IL-6), IL-13, and IL-17, to investigate whether these cytokines are related to CTD-ILD, and to find their possible contribution to determining the prognosis of the disease. METHODS: A total of 150 participants, 80 patients diagnosed with CTD-ILD (mean age, 58.21 ± 12.36) and 70 healthy controls (mean age, 57.07 ± 9.60) were recruited from the rheumatology department between January 2016 and June 2019 in the study. High-resolution computed tomography (HRCT) findings were scored as similarly to previous studies. Serum IL-6, IL 13, and IL-17 levels were measured by ELISA test kits. RESULTS: The levels of IL-6, IL-13, and IL-17 in CTD patients were significantly higher than the healthy individuals (p < 001), but the HRCT score's relation were not determined. IL-6 was associated with disease duration and disease activity scores of DAS28, ESDAII, and dSSc. There was a significant relation between dSSc, HCRT fibrosis, and total score.CRP, hemoglobin, and platelets were associated with the HRCT inflammation pattern. CONCLUSION: At the study, it has been observed that serum IL-13, IL-6 and IL-17 levels are increased in patients with CTD-ILD. Besides, IL-6 was associated with disease activity scores of DAS28, ESDAII, and dSSc. Also, HRCT fibrosis score is associated with dSSc. Further and comprehensive studies are needed to understand better the complex intersection of lung disease with systemic autoimmunity. Key Points • Serum IL-13, IL-6, and IL-17 levels are increased in patients with CTD-ILD. • IL-6 was associated with disease activity scores of DAS28, ESDAII, and diffuse skin involvement. • HRCT fibrosis score is associated with diffuse skin involvement in patients with SSc-ILD. • HRCT inflammation score is associated with PAH.

What do simple hematological parameters tell us in patients with systemic sclerosis?

INTRODUCTION: Systemic sclerosis (SSc) or scleroderma is a clinically heterogeneous disease. Autoantibodies associated with different clinical features may help in predicting organ involvement. Complete blood count (CBC) parameters and neutrophil/lymphocyte (NLR), monocyte/lymphocyte (MLR), and platelet/lymphocyte (PLR) ratios, which are considered biomarkers of systemic inflammation, have been reported many times in various rheumatologic diseases. Studies related to the usefulness of the CBC to assess the severity of SSc are still lacking. This study seeks to determine whether CBC parameters associated with organ involvement, when evaluated together with clinical features and autoantibodies, can additionally contribute to risk estimation. METHODS: Adult patients with SSc (n = 130) and healthy control (n = 129) groups were enrolled in the study. Epidemiological, clinical, laboratory, and radiological findings were obtained by examining patient records. RESULTS: PLR, NLR, and MLR were related to organ involvement. Statistically significant results were obtained with hemoglobin (≤ 13.0 g/dl), lymphocyte count (≤ 1,900 × 103/ml), and mean platelet volume (≤ 8.0 fl) to estimate the risk of interstitial lung disease (p < 0.05). When the lymphocyte count was 1,400 (103/ml) or less, there was a significantly greater risk of pulmonary hypertension. Neutrophil volume ≤ 141 indicated gastrointestinal tract involvement. CONCLUSIONS: Simple hematological parameters can be used for predicting SSc-related organ involvements.