TNF-Related Apoptosis-Inducing Ligand Receptor 1 in Patients With Ankylosing Spondylitis.

OBJECTIVES: Tumor necrosis factor-related apoptosis-inducing ligand (TRAIL) belongs to the tumor necrosis factor (TNF) superfamily and is reported to play a role in autoimmune diseases. In this study, we aimed to measure serum TRAIL receptor 1 (TRAIL-R1) concentration and assess any phenotypic relationship in patients with ankylosing spondylitis (AS). METHODS: Fifty-three patients with AS were recruited from August 2014 to December 2014 cross-sectionally. Fifty-three sex- and age-matched healthy controls were also recruited. Serum TRAIL-R1 concentrations were measured using an enzyme-linked immunosorbent assay. The association between serum TRAIL-R1, TNF-α, disease activity indices, markers of systemic inflammation, and clinical features were evaluated. RESULTS: Serum TRAIL-R1 and TNF-α levels were increased in patients with AS compared with healthy controls (4.5 ± 2.3 vs 3.5 ± 2.3 pg/mL, p = 0.036; 3.8 [1.6-7.7] vs 2.0 [0.21-5.7] pg/mL, p = 0.048, respectively). Serum TRAIL-R1 displayed a medium positive correlation with serum TNF-α concentrations (r = 0.412; p = 0.002). Serum TRAIL-R1 concentration was higher in human leucocyte antigen (HLA)-B27-positive patients compared with non-HLA-B27 patients (5.5 ± 2.2 vs 3.1 ± 1.6 pg/mL, p < 0.001). No relationship was found between serum TRAIL-R1 concentration and disease activity scores. CONCLUSIONS: This study confirms that serum TRAIL-R1 levels are higher in AS patients than healthy controls. The persistence of significantly elevated serum TRAIL-R1 levels, even in patients with low disease activity or after excluding biologic treatment, and the association with HLA-B27 positivity, warrants further investigation due to the unclear role of TRAIL-R1 in the pathophysiology of AS.

Oral ulcer activity assessment with the composite index according to different treatment modalities in Behçet's syndrome: a multicentre study.

OBJECTIVES: The aim of this multicentre study was to understand patients' needs and to evaluate the oral ulcer activity with the Composite Index (CI), according to different treatment modalities in Behçet's syndrome (BS). METHODS: BS patients (n=834) from 12 centres participated in this cross-sectional study. Oral ulcer activity (active vs. inactive) and the CI (0: inactive vs. 1-10 points: active) were evaluated during the previous month. The effects of treatment protocols [non-immunosuppressive: non-IS vs. immunosuppressive: (ISs)], severity (mild vs. severe), disease duration (<5 years vs. ≥5 years) and smoking pattern (non-smoker vs. current smoker) were analysed for oral ulcer activity. RESULTS: Oral ulcer activity was observed in 65.1% of the group (n=543). In both genders, the activity was higher in mild disease course with non-IS treatment group compared to severe course with ISs (p<0.05). As a resistant group, patients with mild disease course whose mucocutaneous symptoms were unresponsive to non-IS medications were treated with ISs in a limited period and achieved the highest CI scores in females. Oral ulcer activity and poor CI score were associated with disease duration less than 5 years compared to others in male patients (p<0.05). CONCLUSIONS: Oral ulcer activity pattern is affected by both the combination of disease course, treatment protocols and disease duration. CI scores reflected the oral clinical activity and CI might be a candidate scale to evaluate the efficacy of treatments during the follow-up of oral ulcer activity in BS.

Histologic outcome of thyroid nodules with repeated diagnosis of atypia in thyroid fine-needle aspiration biopsy.

AIM: We hypothesized that the estimated risk of malignancy for atypia of undetermined significance/follicular lesion of undetermined significance (AUS/FLUS) is higher than anticipated in Bethesda system. Therefore, we analyzed the actual malignancy risk of repeated AUS/FLUS diagnosis of thyroid fine-needle aspiration biopsies (FNAB). MATERIALS & METHODS: We reported retrospective analyzes of 112 cases with repeated AUS/FLUS diagnosis among 10,769 thyroid FNABs. The histologic follow-up were evaluated in the study. RESULTS: 112 cases with a repeated diagnosis of AUS/FLUS, histologic follow-up revealed 56 (50%) benign, 46 (41%) malignant and ten (9%) well-differentiated tumors of uncertain malignant potential outcome. CONCLUSION: The malignancy risk of AUS/FLUS category in thyroid FNABs was higher than anticipated in Bethesda system. Therefore, the management strategy of AUS/FLUS should be revised.

Long-term efficacy of canakinumab in hyperimmunoglobulin D syndrome.

Hyperimmunoglobulin D syndrome (HIDS) is a rare autoinflammatory disorder with autosomal recessive inheritance. It is caused by specific mutations in the mevalonate kinase gene (MVK). No treatment specific to HIDS has been approved to date; however, nonsteroidal anti-inflammatory drugs, steroids, colchicine, tumor necrosis factor-α inhibitors, and anti-interleukin-1 treatments are used, based on case reports and observational studies. Herein, we report a case with recurrent fever and arthritis attacks who did not respond to anakinra and was successfully treated with canakinumab. Long-term remission was achieved without any side effects with 300 mg canakinumab treatment every 4 weeks for 5 years.

Adherence to the Mediterranean Diet in Italian Patients With Systemic Sclerosis: An Epidemiologic Survey.

OBJECTIVE: Systemic sclerosis (SSc) is an orphan disease that can lead to severe involvement of the gastrointestinal tract with a significant impact on patients' quality of life (QoL). The Mediterranean diet (MD) was consistently demonstrated to have beneficial effects on chronic diseases based on biological bases. We aimed to evaluate the adherence to the MD of Italian patients with SSc to preliminarily assess its association with gastrointestinal symptoms and other disease features, mood, and QoL. METHODS: In this cross-sectional study, adherence to the MD was measured in 387 patients from four SSc Italian referral centers through the 14-item Mediterranean Diet Adherence Screener (14-MEDAS) questionnaire. We also registered patients' reported outcomes related to the QoL and mood. RESULTS: Overall, an optimal adherence to MD was observed in 14.7% of patients with SSc, a moderate adherence in 71.3%, and a low adherence in 14.0%. In univariate analysis, poor adherence to the MD was associated with a more prominent depressive mood, time missed at work, and perception of more severe Raynaud's phenomenon and digital ulcers, whereas the 14-MEDAS score inversely correlated with depression score and reflux. CONCLUSION: In our cohort of patients with SSc, overall adherence to MD was moderate. Patients with lower adherence to MD also reported worse outcomes related to QoL and mood. Administration of the 14-MEDAS could be a reasonable choice to assess adherence to the MD in patients with SSc. Future initiatives to study the role of MD in the management of patients with SSc are warranted.

UCLA Scleroderma Clinical Trials Consortium Gastrointestinal Tract (GIT) 2.0 Reflux Scale Correlates With Impaired Esophageal Scintigraphy Findings in Systemic Sclerosis.

OBJECTIVE: The University of California Los Angeles Scleroderma Clinical Trials Consortium Gastrointestinal Tract 2.0 (GIT 2.0) instrument is a self-report tool measuring gastrointestinal (GI) quality of life in patients with systemic sclerosis (SSc). Scarce data are available on the correlation between patient-reported GI symptoms and motility dysfunction as assessed by esophageal transit scintigraphy (ETS). METHODS: We evaluated the GIT 2.0 reflux scale in patients with SSc admitted to our clinic and undergoing ETS, and correlated their findings. RESULTS: Thirty-one patients with SSc undergoing ETS were included. Twenty-seven were female, and 9 had diffuse cutaneous SSc. Twenty-six of 31 (84%) patients had a delayed transit and an abnormal esophageal emptying activity (EA); they also had a higher GIT 2.0 reflux score (P = 0.04). Mean EA percentage was higher in patients with none to mild GIT 2.0 reflux score (81.1 [SD 11.5]) than in those with moderate (55.7 [SD 17.8], P = 0.003) and severe to very severe scores (55.8 [SD 19.7], P = 0.002). The percentage of esophageal EA negatively correlated with the GIT 2.0 reflux score (r = -0.68, P < 0.0001), but it did not correlate with the other GIT 2.0 scales and the total GIT 2.0 score. CONCLUSION: SSc patients with impaired ETS findings have a higher GIT 2.0 reflux score. The GIT 2.0 is a complementary tool for objective measurement of esophageal involvement that can be easily administered in day-to-day clinical assessment.

Evaluation of left and right ventricle by two-dimensional speckle tracking echocardiography in systemic sclerosis patients without overt cardiac disease.

BACKGROUND/OBJECTIVE: The aim was to evaluate the left and right ventricular functions concurrently by two-dimensional speckle tracking echocardiography (STE) in systemic sclerosis (SSc) patients without overt cardiac disease. METHODS: A total of 47 patients with SSc and 36 age- and sex-matched controls were evaluated cross-sectionally. Two-dimensional STE was used to assess the longitudinal peak systolic strains (PSS) of both ventricles including apical long-axis (APLAX), apical four-chamber (4-CH), apical two-chamber (2-CH), and global longitudinal measurements. Any association of metabolic, cardiac, and inflammatory biomarkers with PSS was investigated. RESULTS: The longitudinal PSS of the left ventricle [APLAX, 4-CH, 2-CH and global] were significantly lower in SSc patients than controls (- 18.2 ± 3.2 vs - 19.8 ± 2.7% p = 0.02; - 17.8 ± 3.5 vs. - 20.3 ± 3.3% p = 0.001; - 18.6 ± 3.1 vs. - 21.8 ± 3% p < 0.001; - 17.5 ± 5.7 vs. - 20.6 ± 2.7% p = 0.003, respectively). No difference was found between the groups for right ventricular strains. The longitudinal PSS-4CH correlated positively with CRP and ESR (r = 0.349, p = 0.016; r = 0.356, p = 0.014, respectively) and negatively with serum Galectin-3 (r = - 0.362, p = 0.012). Global longitudinal PSS-left ventricle (LV) correlated positively with CRP and homocysteine (r = 0.297, p = 0.043; r = 0.313, p = 0.041, respectively) and negatively with serum Galectin-3 (r = -0.314, p = 0.041). After multivariable adjustment, CRP remained the only predictor of longitudinal PSS-4CH (95% CI 0.35, 0.70, p = 0.028) and global longitudinal PSS of left ventricle (95% CI 0.004, 0.22, p = 0.043). CONCLUSIONS: Biventricular evaluation of patients with SSc by two dimensional STE revealed reduced left ventricular longitudinal strains, despite preserved right ventricular strain, and no diastolic dysfunction. In SSc without overt cardiac disease, global cardiac assessment with 2DSTE is a promising method which seems to contribute to the detection of patients without clinical findings. KEY POINTS: • Two dimensional STE revealed reduced left ventricular longitudinal strains, despite preserved right ventricular strain in SSc patients without overt cardiac disease. • CRP was the predictor of decreased longitudinal strains. • Cardiac assessment in SSc should be made globally.

Predictive factors for work-day loss in Behçet's syndrome: A multi-center study.

OBJECTIVE: The aim of this multi-center study was to assess predictive factors for work-day loss as an indirect cost element in Behçet's syndrome (BS). METHODS: In this cross-sectional, multi-center study, 834 BS patients (F/M: 441/393, age mean: 38.4 ± 10.9 years) were included. Data were collected by a questionnaire regarding treatment protocols, disease duration, smoking pattern, frequency of medical visits during the previous year and self-reported work-day loss during the previous year. RESULTS: Work-day loss was observed in 16.2% of patients (M/F: 103/32). The percentages of being a smoker (81.8%), using immunosuppressive (IS) medications (82%), and having disease duration <5 years (74%) were higher in male patients with work-day loss (P < .05). The majority of males (90.9%) had more than four clinic visits during the previous year. Moreover, the mean work-day loss (30.8 ± 57.7 days) was higher in patients with vascular involvement (56.1 ± 85.9) than those without (26.4 ± 50.6 days) (P = .046). In addition, increased frequency of ocular involvement (25.9%) was also observed in patients with work-day loss compared to others (8.6%) (P = .059). CONCLUSION: Work-day loss was associated with both vascular and ocular involvement. Close associations were observed among male gender, early period of the disease, frequent medical visits, being a smoker and treatment with IS medications in patients with work-day loss.