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.

Altered kynurenine pathway metabolism and association with disease activity in patients with systemic lupus.

Systemic lupus erythematosus (SLE) is an autoimmune disease accompanied by increased release of proinflammatory cytokines that are known to activate the indoleamine 2,3-dioxygenase (IDO-1) enzyme, which catalyzes the rate-limiting step of the kynurenine pathway (KP). This study aimed to measure KP metabolite levels in patients with SLE and investigate the relationship between disease activity, clinical findings, and KP. The study included 100 patients with SLE and 100 healthy controls. Serum tryptophan (TRP), kynurenine (KYN), kynurenic acid (KYNA), 3-hydroxyanthranilic acid (3HAA), 3-hydroxykynurenine (3HK), quinolinic acid (QA) concentrations were measured with tandem mass spectrometry. Serum KYN, KYNA, 3HAA, 3HK, and QA levels of the patients with SLE were significantly higher than the control group. Serum QA levels were elevated in patients with neurological involvement (four patients with peripheral neuropathy and two patients with mononeuropathy), serum KYN levels and KYN/TRP ratio increased in patients with joint involvement, and serum KYN, 3HK, and 3HAA levels and the KYN/TRP ratio were increased in patients with renal involvement. Moreover, KYN and KYN/TRP ratios were positively correlated with the disease activity score. These findings indicated that imbalances in KP metabolites may be associated with the pathogenesis, activation, and clinical manifestations of SLE.

Assessment of lacrimal gland involvement in primary Sjögren's syndrome using gray-scale ultrasonography and shear wave elastography.

OBJECTIVES: The aim of this study was to assess lacrimal gland involvement in primary Sjögren's syndrome (pSS) using lacrimal gland gray-scale ultrasound (LGUS) and two-dimensional shear wave elastography (2D-SWE). METHODS: Eighty-five pSS patients with a mean age of 51.16 ± 10.61 years and 84 sex- and age-matched healthy subjects with a mean age of 50.94 ± 11.05 years were included in the study. Lacrimal gland parenchymal findings and 2D-SWE values were compared between the two groups and the correlations of LGUS parameters with clinical findings, dry eye tests, and minor salivary gland biopsy (MSGB) were further investigated. RESULTS: LGUS parenchymal grade was 0 in 14 (16.5%), 1 in 45 (52.9%), 2 in 23 (27.1%), and 3 in 3 patients (3.5%) in the pSS group, while in the control group, parenchymal grades were 0 (57.1%) and 1 (42.9%). The mean 2D-SWE value of pSS patients was significantly higher than the control group (p < 0.05) and increased parallel with lacrimal parenchymal grade. The elasticity modulus had a high diagnostic performance in detecting lacrimal gland involvement in pSS patients (AUC 0.901, sensitivity 70.6%, specificity 97.6%), while the diagnostic performance of LGUS was much lower (AUC 0.769, sensitivity 83.5%, specificity 57.1%). LGUS and 2D-SWE values were found to be correlated with dry eye tests and MSGB results (p < 0.05). CONCLUSIONS: LGUS and 2D-SWE are both useful for assessing the lacrimal gland involvement in pSS patients; however, 2D-SWE has a better diagnostic performance than LGUS and found to be correlated with dry eye tests. CLINICAL RELEVANCE STATEMENT: Lacrimal gland US and two-dimensional shear wave elastography (2D-SWE) are imaging modalities that can be used to demonstrate parenchymal involvement of the lacrimal gland in primary Sjögren's syndrome (pSS). KEY POINTS: • Gray-scale US and two-dimensional shear wave elastography (2D-SWE) have been widely used in the recent decade to assess gland involvement in patients with primary Sjögren's syndrome (pSS). • The elasticity modulus had a high diagnostic performance in detecting lacrimal gland involvement in primary Sjögren's syndrome (pSS) patients. • Lacrimal gland US and two-dimensional shear wave elastography (2D-SWE) are both useful for assessing the lacrimal gland in primary Sjögren's syndrome (pSS) patients; however, two-dimensional shear wave elastography (2D-SWE) has a better diagnostic performance than lacrimal gland ultrasound (LGUS).

Transepidermal water loss in allergic diseases.

Background: In recent years, the epithelial barrier hypothesis has been emphasized in the formation of allergic diseases. Transepidermal water loss (TEWL) occurs through diffusion and evaporation from the skin to the external environment. There are few studies on TEWL in allergic diseases. Objective: This study evaluated the relationship between patients with atopic diseases and healthy controls and hygiene habits in TEWL. Methods: The study was conducted on patients who were followed up for atopic disease (asthma, allergic rhinitis, immunoglobulin E mediated food allergy, and atopic dermatitis) and healthy children. TEWL measurement was in a room that was stable in terms of humidity and temperature by using a widely validated open room system. During the measurement, the participants reported their frequency of taking a shower and cleaning product use. Results: In the study group, TEWL was measured in 182 patients, and the median (min-max) TEWL was 21.3 g/hm² (7.8-101.3 g/hm²) in the disease group and 9.6 g/hm2 (3.9-30.3 g/hm²) in the control group (p < 0.001). The number of weekly baths was higher in the disease group (p < 0.001). The cutoff for atopic diseases was 13.2 g/hm² (sensitivity, 83.2%; specificity, 84.3%; p < 0.001). Conclusion: High TEWL in atopic diseases supports the epithelial barrier hypothesis associated with disease development. Further studies are necessary to determine the threshold between healthy controls and the patients in the disease group. The TEWL measurement can be an effective method to determine the risk groups. Moreover, further studies related to factors on TEWL and treatment methods to reduce this loss are necessary, too.

Impaired kynurenine metabolism in patients with primary Sjögren's syndrome.

OBJECTIVES: Primary Sjögren's syndrome (pSS) is an autoinflammatory disease characterized by inflammation of the exocrine glands. Elevated inflammation causes an increase in kynurenine pathway (KP) metabolite levels by activating indoleamine 2,3-dioxygenase (IDO). The aim of this study was to measure serum KP metabolite concentrations in patients with pSS and to evaluate the relationship between these metabolites with disease activity score and clinical manifestations. DESIGN & METHODS: A total of 80 patients with pSS and 80 healthy controls were enrolled in this study. Serum tryptophan (TRP), kynurenine (KYN), kynurenic acid (KYNA), 3-hydroxyanthranilic acid (3HAA), 3-hydroxykynurenine (3HK), quinolinic acid (QUIN) concentrations were quantified with liquid chromatography with tandem mass spectrometry (LC-MS/MS). Demographic characteristics, clinical manifestations and disease activity score (ESSDAI) of the participants were recorded. RESULTS: The serum level of KYN and QUIN were significantly higher in patients with pSS with low and moderate activity compared those healthy controls, while the serum level of TRP, KYNA/KYN and 3HK/KYN were lower. In addition, the significant difference for the serum level of KYNA was only in patients with moderate activity from healthy controls, and the difference was higher in favor of pSS patients. Moreover, the KYN/TRP levels were significantly increased with disease activity. The ESSDAI score was positively correlated with KYN/TRP ratio, but negatively correlated with KYNA/KYN ratio. CONCLUSIONS: These findings indicated that KP metabolites may play a role in the etiopathogenesis, activation and progression of pSS.

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.

Raised total methylated arginine load in patients with gout.

Aim: The aim of this study was to measure serum levels of methylarginine derivatives and related metabolites in patients with gout. Materials & methods: This study enrolled 100 patients with gout and 80 patients in the control group. Serum asymmetric dimethylarginine, symmetric dimethylarginine, L-N-monomethylarginine, arginine, homoarginine, citrulline and ornithine levels were measured with tandem mass spectrometry. Results: Serum ornithine, citrulline and total methylated arginine load levels were statistically significantly higher in patients with gout compared with the control group, while serum arginine and homoarginine levels and global arginine bioavailability ratio were statistically significantly lower. Conclusion: There may be an association between gout, methylarginine levels and hyperuricemia and increased risk of cardiovascular disease.

Elevated serum levels of kynurenine pathway metabolites in patients with Behçet disease.

Behçet disease (BD) is an inflammatory, multisystemic vasculitis of unknown etiopathogenesis. However, innate and adaptive immune system involvement and immune-mediated networks play a vital role in the inflammatory cascade. Indoleamine 2,3-dioxygenase 1 (IDO1) is activated in chronic inflammatory states and catalyzes the first and rate-limiting step of tryptophan (TRP) metabolism along the kynurenine pathway (KP). The study aimed to measure KP metabolites levels in patients with BD and investigate the relationship between disease activity and clinical findings with these metabolites. The study included 120 patients with BD and 120 healthy volunteers. Serum TRP, kynurenine (KYN), kynurenic acid (KYNA), 3-hydroxyanthranilic acid (3HAA), 3-hydroxykynurenine (3HK), and quinolinic acid (QUIN) levels were measured with the tandem mass spectrometric method. Demographic data, clinical manifestations, and disease activity score (BDCAF) were recorded. Serum KYN, KYNA, 3HK, 3HAA, QUIN levels, and KYN/TRP ratio were higher (p < 0.05) in patients with BD compared to the control group, while TRP levels were lower (p < 0.05). KYN/TRP ratio and QUIN levels were significantly higher in the presence of neuro-Behçet, while serum KYN levels were significantly higher in the presence of arthritis (p < 0.05). In addition, serum QUIN levels were significantly higher in the presence of thrombosis (p < 0.05). BDCAF score positively correlated with KYN/TRP ratio. Our findings showed that serum KP metabolite levels were elevated in patients with BD, and there is a relationship between these metabolites with disease activity, clinical findings, and inflammatory burden.

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.

Effects of hydroxychloroquine and its metabolites in patients with connective tissue diseases.

Hydroxychloroquine has attracted attention in the treatment of COVID-19. Many conflicting findings have been reported regarding the efficacy and safety of this drug, which has been used safely in the rheumatological diseases for years. However, these studies lacked measurement methods that allow accurate assessment of hydroxychloroquine and its metabolite levels. The aim of this study was to measure hydroxychloroquine and its metabolite levels in whole blood samples of patients with rheumatoid arthritis (RA), systemic lupus erythematosus (SLE), Sjogren's syndrome (SS) and scleroderma (Scl) by a robust, simple and accurate validated tandem mass spectrometric method, and to investigate the relationship between these levels with drug-related adverse effects and disease activity scores. The validated LC-MS/MS method was applied to measure blood hydroxychloroquine and its metabolite levels of patients with RA, SLE, SS, Scl. Various haematological and biochemical parameters were measured with Beckman-Coulter AU 5800 and Beckman Coulter LH 780 analyzers, respectively. QTc intervals were calculated with Bazett's formula, and the patients were followed up by clinicians in terms of clinical findings and adverse effects. Hydroxychloroquine levels of patients were similar to previous studies. There was a negative correlation between disease activity scores and hydroxychloroquine levels, while the highest correlation was between QTc interval, creatinine and GFR levels with desethylchloroquine. Bidetylchloroquine had the highest correlation with RBC count and liver function tests. Our findings showed that hydroxychloroquine and its metabolite levels were associated with disease activity scores, renal, hepatic function, QTc prolongation, and hematological parameters.

Mean platelet volume as a predictor in the differentiation of Behçet's disease from recurrent aphthous stomatitis - A single centre, prospective, case-control study.

INTRODUCTION: Recurrent oral ulcers (ROUs) are the most common disorder of the oral cavity in the community. The most challenging issue for the clinician to deal with in the diagnosis is to distinguish whether ROUs are associated with primarily Behçet's disease (BD). We aimed to investigate whether hemogram parameters contribute to reinforce BD suspicion in the differential diagnosis of recurrent aphthous stomatitis (RAS) and BD. METHODS: A total of 260 participants were recruited from the dermatology department in this single-centre, prospective, case-control study. Participants meeting eligibility criteria were divided into three groups as healthy control (n = 90), RAS (n = 97) and mucocutaneous BD (n = 73). The mean platelet volume (MPV), red cell distribution width (RDW), haemoglobin, neutrophils, monocyte, lymphocytes and platelet counts were evaluated with the complete blood count. Furthermore, the neutrophil/lymphocyte ratio (NLR), monocyte/lymphocyte ratio and platelet/lymphocyte ratio were calculated. Erythrocyte sedimentation rate (ESR) and C-reactive protein were recorded. The contribution of hemogram parameters to the differentiation of BD from RAS was analysed. RESULTS: The healthy control, RAS and BD groups were matched in terms of mean age (29.3 ± 5.8, 28.7 ± 5.6 and 29.9 ± 6.8 years; respectively) and sex distribution (women/men rate: 1.4, 1.6 and 1.7; respectively). ESR, neutrophil count and NLR were significantly higher in BD patients. (P = .032, P = .010, P = .019; respectively). MPV is significantly decreased in BD patients than in healthy control and RAS patients (P < .001). Decreased MPV (≤10 fL) and increased RDW (≥13.0%) were useful in predicting BD in patients evaluated with complaints of ROUs (OR = 9.98, 95% CI: [4.65-21.42], P < .001 and OR = 2.23, 95% CI: [1.14-3.36], P = .019, respectively). CONCLUSIONS: Decreased MPV may be predictive for BD in selected patients with ROUs. We consider that more attention is required in terms of BD especially if MPV ≤10.0 fL.

Altered kynurenine pathway metabolism in patients with ankylosing spondylitis.

BACKGROUND: Various studies reported that increased proinflammatory cytokines in patients with ankylosing spondylitis (AS). Proinflammatory cytokines can affect the expression of various kynurenine pathway enzymes and therefore lead to metabolic changes that can affect the inflammatory response and immunity. Our aim was to measure serum levels of kynurenine pathway metabolites in patients with AS. METHODS: The study included 85 patients with AS and 50 healthy volunteers. Serum tryptophan, kynurenine, kynurenic acid, 3-hydroxyanthranilic acid, 3-hydroxykynurenine, quinolinic acid concentrations were measured with tandem mass spectrometry. In addition, participants were divided into four groups according to the treatment regimen: TNF-α inhibitor group, conventional therapy group, control group and newly diagnosed AS group. These groups were compared in terms of kynurenine pathways metabolites, interleukin 6 (IL-6), erythrocyte sedimentation rate (ESR) and C-reactive protein (CRP) levels. RESULTS: Serum tryptophan, kynurenic acid, 3-hydroxykynurenine levels were significantly decreased (p < 0.05) in both AS groups compared to the control group, while the levels of kynurenine, quinolinic acid, CRP, ESR, and IL-6 were higher (p < 0.05). The Kynurenine/Tryptophan ratio and CRP levels of the conventional therapy and anti-TNF therapy group were significantly lower than the newly diagnosed AS patients (p < 0.05). CONCLUSION: As a result of our study, we found that altered kynurenine pathway metabolism in patients with AS. Conventional therapy and anti-TNF-α therapy are effective in reducing the Kynurenine/Tryptophan ratio and CRP levels, although the effect of both treatments on other metabolites appears to be limited.

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.

Is There an Optimal Training Interval to Improve the Correct Use of Adrenaline Auto-Injectors?

BACKGROUND: Accurate use of adrenaline auto-injectors (AAIs) for anaphylaxis is critical to decrease mortality and morbidity. OBJECTIVE: In this study, we aimed to assess user knowledge of AAIs and evaluate the factors that affect their correct use. METHODS: The study involved caregivers of pediatric patients diagnosed with anaphylaxis who were trained with trainer injectors up to 24 months ago. The demographics of the caregivers, anaphylaxis history of the patients, usage of AAIs in the case of anaphylaxis, and the reasons for not using AAIs in anaphylaxis were evaluated. Users were asked to demonstrate the use of Penepin® with a trainer injector. RESULTS: Fifty-nine caregivers were enrolled in the study. Forty-seven (79.7%) users stated that they always carry AAIs with them. Forty-one (69.5%) of the users demonstrated all steps of the use of AAIs. The time from the last AAI training was the most significant parameter affecting the ability to use AAIs correctly (OR 0.678, 95% CI 0.546-0.841, p < 0.0001). AAI training every 6 months results in the proper usage of AAIs, with 96% probability. Thirty (50.8%) caregivers stated that anaphylactic reactions occurred in their children after the last AAI training. Of these, 16 (53.3%) users stated that they did not use an AAI for the anaphylactic reaction. The most common reason (50%) was not carrying an AAI on their person. CONCLUSION: Training users at least every 6 months is associated with the proper application of AAIs. Although regular training increases the frequency of AAI use in anaphylaxis, awareness of carrying AAIs is the most important factor for usage of AAI in anaphylaxis.

Relation between serum IL-4, IL-13 and IFN-gamma levels and recurrence of wheezing episodes in infants with acute bronchiolitis.

Lower respiratory tract infections are the most important factors among various causes which trigger wheezing in the first year of life. The factors associated with episodic wheezing in children with acute bronchiolitis are still subjects of research. Infections, environmental factors, immunologic mechanisms are sorted as etiologic risk factors of episodic wheezing. We aimed to investigate the relationship between serum interleukin (IL)-4, IL-13 and gamma-interferon (IFN-gamma) levels and recurrence of wheezing episodes in infants with acute bronchiolitis. One hundred twenty infants between 3 and 36 months with acute bronchiolitis enrolled in the study. Personal histories, clinical and laboratory data of infants were recorded. The patients were followed for a year. Venous blood samples were obtained to determine serum IL-4, IL-13, and IFN-gamma levels during acute bronchiolitis episode. The number of wheezing episodes was significantly higher in infants with a positive family history of allergy. A statistically significant correlation was determined between serum IL-13 levels of infants and number of wheezing episodes. High serum IL-13 levels and a positive history of allergy may have important roles in the recurrence of acute bronchiolitis.