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Background: Sleep disorders and fatigue are prevalent symptoms affecting primary Sjögren's syndrome (pSS) patients. This study aimed to assess the sleep quality of pSS patients as well as its relationship to fatigue and orexin level., Methods: This is a cross-sectional study evaluating fatigue in pSS using the Fatigue Severity Scale (FSS). Sleep quality was evaluated using the Pittsburg Sleep Quality Index (PSQI). The European Alliance of Associations for Rheumatology (EULAR) Sjögren's Syndrome Disease Activity Index (ESSDAI) and EULAR Sjögren's Syndrome Patient-Reported Index (ESSPRI) were calculated., Results: Forty-one patients met the sample criteria and were involved in the final report. They were all females, with a mean (± SD) age and median disease duration of 40.87 ± 10.84 and 36 (6-180) months, respectively. The mean ESSDAI was 0.92 ± 1.3, while the mean ESSPRI was 5.8 ± 2.13. Based on the FSS, 32 (78.04%) patients had a positive test with a mean score of 5.07 ± 1.54. The total PSQI score showed that 60.97% had poor sleep, and the orexin level was lower in patients with pSS than in healthy controls. There was no correlation between orexin level and the presence of fatigue nor the PSQI score., Conclusion: In conclusion, serum orexin levels were lower in patients with pSS than healthy controls, It could be related to impairments in sleep and fatigue in patients with pSS., (© 2023. The Author(s), under exclusive licence to Springer Medizin Verlag GmbH, ein Teil von Springer Nature.)

Objective: This study aimed to assess the presence of subclinical atherosclerosis in patients with psoriatic arthritis using ultrasound radiofrequency data technology., Methods: In all, 29 psoriatic arthritis patients and 42 age- and sex-matched healthy controls were included in this cross-sectional study. Arterial stiffness and carotid intima-media thickness measurements were performed in bilateral common carotid arteries using ultrasound radiofrequency data technology in all participants., Results: In psoriatic arthritis patients, the mean carotid intima-media thickness, α and β stiffness indices, and pulsed wave velocity value were significantly higher than those in the control group (542.3 (81.3) vs. 487.9 (64.1), 9.3 (6.3) vs. 3.9 (0.1), 18.7 (17.7) vs. 8.04 (4.2), and 10.2 (3.8) vs. 6.4 (1.5), p<0.05). The mean distensibility coefficient and compliance coefficient values of the patient group were significantly lower than those of the control group (0.014 (0.01) vs. 0.03 (0.01) and 0.57 (0.33) vs. 1.02 (0.4), p<0.05). No significant correlation was found between carotid artery hemodynamic parameters and symptom duration, duration of diagnosis and treatment, disease activity ındex for psoriatic arthritis scores, erythrocyte sedimentation rate, and C-reactive protein levels (p>0.05)., Conclusion: In the results of our study, evidence of subclinical atherosclerosis has been detected in psoriatic arthritis patients without clinically evident cardiovascular disease or traditional cardiovascular risk factors.

Objectives: Our aim is to understand clinical characteristics, real-life treatment strategies, outcomes of early PsA patients and determine the differences between the inception and established PsA cohorts., Methods: PsArt-ID (Psoriatic Arthritis- International Database) is a multicentre registry. From that registry, patients with a diagnosis of PsA up to 6 months were classified as the inception cohort (n==388). Two periods were identified for the established cohort: Patients with PsA diagnosis within 5-10 years (n = 328), ≥10 years (n = 326). Demographic, clinical characteristics, treatment strategies, outcomes were determined for the inception cohort and compared with the established cohorts., Results: The mean (s.d.) age of the inception cohort was 44.7 (13.3) and 167/388 (43.0%) of the patients were male. Polyarticular and mono-oligoarticular presentations were comparable in the inception and established cohorts. Axial involvement rate was higher in the cohort of patients with PsA ≥10 years compared with the inception cohort (34.8% vs 27.7%). As well as dactylitis and nail involvement (P = 0.004, P = 0.001 respectively). Both enthesitis, deformity rates were lower in the inception cohort. Overall, 13% of patients in the inception group had a deformity. MTX was the most commonly prescribed treatment for all cohorts with 10.7% of the early PsA patients were given anti-TNF agents after 16 months., Conclusion: The real-life experience in PsA patients showed no significant differences in the disease pattern rates except for the axial involvement. The dactylitis, nail involvement rates had increased significantly after 10 years from the diagnosis and the enthesitis, deformity had an increasing trend over time., (© The Author(s) 2020. Published by Oxford University Press on behalf of the British Society for Rheumatology. All rights reserved. For permissions, please email: journals.permissions@oup.com.)

Objective: Joints with different sizes and anatomical locations can be affected in psoriatic arthritis (PsA). Our aim was to explore the effect of different joint patterns on patient-reported outcomes (PROs) in patients with mono-oligoarthritis., Methods: Within PsArt-ID (Psoriatic Arthritis- International Database), 387/1670 patients who had mono-oligoarthritis (1-4 tender and swollen joints) were enrolled in cross-sectional assessment. The joints were categorized according to their size (small/large) and location (upper/lower extremity) and PROs, physician global assessment and C-reactive protein (CRP) were compared. Analysis was made by categorizing according to joint counts (1-2 joints/ 3-4 joints)., Results: The mean age (SD) was 46.9 (14.24) with a mean (SD) PsA duration of 3.93 (6.03) years. Within patients with 1-2 involved joints (n = 302), size of the joints only had an impact on CRP values with large joints having higher CRP (P = .005), similar to lower extremity involvement (P = .004). PROs were similar regardless of size or location if 1-2 joints were inflamed. Within patients with 3-4 involved joints (n = 85), patient global assessment (PGA), pain, fatigue and physician global assessment were higher in the group with large joints. Similarly, PGA, pain, and physician global assessment were higher in patients with lower extremity involvement as well as higher CRP values., Conclusion: For PsA patients with 3-4 joints involved, lower extremity and large joints are associated with poorer outcomes with worse PROs, physician global assessment, and higher CRP. The size and anatomical location of the joints are less important for patients with 1-2 joints in terms of the PROs., (© 2020 Asia Pacific League of Associations for Rheumatology and John Wiley & Sons Australia, Ltd.)

Objective: Psoriatic arthritis (PsA) has a genetic background. Approximately 40% of patients with psoriasis or PsA have a family history of psoriasis or PsA, which may affect disease features. The aim of this study was to assess the effects of family history of psoriasis and PsA on disease phenotypes., Methods: Data from 1,393 patients recruited in the longitudinal, multicenter Psoriatic Arthritis International Database were analyzed. The effects of family history of psoriasis and/or PsA on characteristics of psoriasis and PsA were investigated using logistic regression., Results: A total of 444 patients (31.9%) had a family history of psoriasis and/or PsA. These patients were more frequently women, had earlier onset of psoriasis, more frequent nail disease, enthesitis, and deformities, and less frequently achieved minimal disease activity. Among 444 patients, 335 only had psoriasis in their family, 74 had PsA, and 35 patients were not certain about having PsA and psoriasis in their family, so they were excluded from further analysis. In the multivariate analysis, family history of psoriasis was associated with younger age at onset of psoriasis (odds ratio [OR] 0.976) and presence of enthesitis (OR 1.931), whereas family history of PsA was associated with lower risk of plaque psoriasis (OR 0.417) and higher risk of deformities (OR 2.557). Family history of PsA versus psoriasis showed increased risk of deformities (OR 2.143) and lower risk of plaque psoriasis (OR 0.324)., Conclusion: Family history of psoriasis and PsA impacts skin phenotypes, musculoskeletal features, and disease severity. The link between family history of psoriasis/PsA and pustular/plaque phenotypes may point to a different genetic background and pathogenic mechanisms in these subsets., (© 2019, American College of Rheumatology.)

Objectives : Polyneuropathy is the most common neurological complication in primary Sjögren's syndrome (pSS). A ratio of sural nerve and superficial radial nerve sensorial action potential amplitudes (SRARs) of <0.4 is an indicator for early axonal neuropathy. We evaluated the polyneuropathies and SRARs in pSS patients. Method : Fifty-two female patients who were diagnosed with pSS according to the European-American Consensus Criteria and 45 healthy controls were enrolled. Nerve conduction studies were performed to diagnose polyneuropathy. Sensory axonal polyneuropathy was diagnosed in three patients, so SRARs were compared in 49 patients and 50 healthy controls. Results : Fifty-two patients with pSS underwent nerve conduction tests. The sural sensory nerve action potential (SNAP) was <6 µV in threepatients and they were diagnosed with sensory axonal neuropathy. SRARs were evaluated in 49 female patients, with a mean age of 51.98 ± 10.79 years and 50 healthy controls with a mean age of 50.52 ± 12.55 years. The mean disease duration was 7.59 ± 6.17 years. The SRAR values were different between the patient and control groups. SRAR was <0.4 in 20.4% of the patient group and <0.4 in 6% of the control group. The SRAR value was not statistically different within the patient group based on anti-Ro and anti-La. Discussion : The potential for neurological involvement in patients with pSS who have no signs or injury should be evaluated because nervous system involvement in pSS is a negative prognostic factor. SRAR in patients with pSS can be used as a marker for the early detection of axonal neuropathy.

Objective: The effect of smoking in psoriatic arthritis (PsA) is under debate. Our aim was to test whether smoking is increased in axial PsA (axPsA)., Methods: Included in the analysis were 1535 patients from PsArt-ID (PsA-International Database). The effect of smoking on axPsA (compared to other PsA phenotypes) and radiographic sacroiliitis were investigated., Results: Current smoking was more common in axPsA (28.6% vs 18.9%, p < 0.001). It also was found as an independent predictor of axPsA (OR 1.4) and radiographic sacroiliitis (OR 6.6)., Conclusion: Current smoking is significantly associated with both axPsA and radiographic sacroiliitis in patients with PsA.

Objectives: Minimal disease activity (MDA) is an important target in patients with psoriatic arthritis (PsA), however it is also criticised for having a low threshold for patient reported outcomes (PRO).The aim of the study was to assess the prevalence of MDA and its components in patients with PsA and to evaluate disease characteristics and patterns in patients with or without MDA (MDA+ or MDA-)., Methods: PsArt-ID (Psoriatic Arthritis-International Database) is a prospective, multicentre web-based registry. PsA patients who had at least 1 year of disease duration and had full data for MDA were included for this analysis (n=317). Patients were considered in MDA+ when they met at least 5/7 of the MDA criteria., Results: MDA was achieved in 46% patients. Within MDA- patients, body surface area (51.2%) and swollen joint count (53.5%) domains could still be achieved in the majority and 93.5% of them had no enthesitis using the Leeds enthesitis index. Of 170 patients with MDA-, 90 patients did not fulfill all 3 PROs of MDA. Mono-arthritis subtype (RR: 2.01), absence of enthesitis (RR: 1.570) and absence of distal interphalangeal (DIP) joint disease (RR: 1.1) were associated with higher probability of achieving MDA., Conclusions: The MDA criteria provide an objective target for treatment in trials and clinical practice; however, in real life PROs are the most significant barriers to achieve MDA. The presence of DIP joints disease makes it difficult to reach MDA due to active PROs.

Aim: We aimed to evaluate subclinical atherosclerosis based on carotid intima-media thickness (CIMT) and arterial stiffness measurements in patients with Behçet's disease (BD), using ultrasound (US) radiofrequency (RF) data technology., Methods: We included 33 BD patients and 33 healthy controls in this study. The participants did not have any primary cardiovascular risk factors, such as diabetes mellitus, hypertension, hyperlipidemia and obesity. All participants were evaluated using Doppler ultrasonography. Arterial stiffness and CIMT measurements were performed in bilateral common carotid arteries using the US RF data technology., Results: The right, left and mean CIMT values were similar between the patient and control groups (P > 0.05). There was also no statistically significant difference in the right side α and β stiffness indices despite higher values in the patient group (P > 0.05). The right, left and mean distensibility coefficient (DC) and compliance coefficient (CC) values of the patient group were significantly lower than the control group (P < 0.05). The left and mean α- and β-stiffness indices, and the right, left and mean pulse wave velocity (PWV) values were significantly higher in the patient group (P < 0.05). We found higher mean CIMT and PWV values, and α- and β-stiffness indices in patients with mucocutaneous involvement compared with those with major organ involvement, while the mean DC and CC values were lower in the former. However, these results did not reach a statistically significant level., Conclusion: This study demonstrated evidence of subclinical atherosclerosis in BD in the absence of major atherosclerotic risk factors., (© 2019 Asia Pacific League of Associations for Rheumatology and John Wiley & Sons Australia, Ltd.)

Primary Sjögren's syndrome (pSS) is a chronic inflammatory autoimmune disease, and inflammation is highly associated with atherosclerosis and increased cardiovascular risk. Carotid intima-media thickness (CIMT) and arterial stiffness measurements are commonly used to detect subclinical atherosclerosis. The aim of this study was to non-invasively demonstrate the presence of subclinical atherosclerosis in patients with pSS through these measurements, using ultrasound (US) radiofrequency (RF) data technology. 30 pSS patients as the study group and 30 age-and sex-matched healthy volunteers as the control group were included in this study. The age of the participants in the entire sample ranged from 18 to 60 years, and no primary cardiovascular risk factors were present, such as diabetes mellitus, hypertension, hyperlipidemia, or obesity. The participants in the study and control groups were evaluated with doppler ultrasonography. Arterial stiffness and CIMT measurements were made from the bilateral common carotid arteries (CCA) using US RF data technology. No statistically significant difference was identified between the patients with pSS and the controls in terms of the right, left, and mean CCA IMT; the right side distensibility coefficient (DC) and compliance coefficient (CC); or the right- and left-side α and β stiffness indices values (p > 0.05). Compared to the control subjects, the pSS patients had higher right and left side pulsed wave velocity (PWV), the mean value of the right and the left sides α stiffness index, β stiffness index, and PWV (p < 0.05). The pSS patients' left DC, left CC, and the mean value of the right and left sides DC and CC were lower than controls (p < 0,05). It was found that patients with pSS show evidence of subclinical atherosclerosis. To determine this situation in patients with pSS, CIMT and PWV measurements may serve as a guide. Radiofrequency data technology represents a non-invasive approach to the accurate and quantitative measurement of CIMT elevation and decreases in vascular elasticity.

Psoriatic arthritis (PsA) may affect different joints, including the spine. The prevalence of spinal involvement is variable depending on the definition and a subset of patients have been identified in cohorts that do not have clinical features of axial disease and yet have imaging findings. Still, there is not a consensus on how and when to screen axial disease. In this study, we aimed to investigate factors associated with being underdiagnosed for axial psoriatic arthritis (axPsA) and its impacts on outcomes. Disease features and outcomes of axPsA according to the physician (n = 415) were compared with patients with imaging findings only (sacroiliitis fulfilling the modified New York criteria, n = 112), using data from a real-life PsA registry. Patients with imaging findings only were more frequently women (83/220 (37.7%) vs 29/122 (23.8%); p = 0.008). This group also had higher peripheral disease activity (imaging only vs clinical AxPsA: mean (SD) tender joint count 5.3 (6.1) vs 3.3 (4.7), swollen joint count 1.9 (2.9) vs 1.2 (2.4); p < 0.001 for both comparisons) and was less often treated using TNF inhibitors (16.1 vs 38.2%; p < 0.001) than patients who were classified as axPsA. Patient-reported outcomes were similar in both groups. PsA patients, especially women with more severe peripheral disease, have a higher risk of being underdiagnosed for axPsA. The severity of peripheral symptoms may be a risk factor to mask the spinal features of PsA.

Objectives: Systemic sclerosis (SSc) is a chronic, inflammatory, and autoimmune connective tissue disease that is associated with vascular lesions, and fibrosis of the skin and visceral organs. Cardiac complications may occur as a secondary effect of SSc as a result of pulmonary arterial hypertension and interstitial lung disease. The objective of this study was to assess whether the pulmonary pulse transit time (pPTT) could serve as a diagnostic marker for pulmonary arterial alterations in patients with SSc, prior to development of pulmonary hypertension., Methods: Twenty-five SSc patients as a study group and 25 age- and sex-matched healthy volunteers for the control group were recruited to the study. Right ventricle function parameters, such as tricuspid annular plane systolic excursion (TAPSE), estimated pulmonary artery systolic pressure (ePASP), right ventricular dimensions, right ventricle fractional area changes, and myocardial perfusion index (MPI) were measured and calculated. Pulmonary pulse transit time was defined as the time interval between the R-wave peak in the ECG and the corresponding peak late systolic pulmonary vein flow velocity., Results: Right ventricle myocardial performance index (RVMPI) and eSPAP were significantly higher in the SSc group than the controls (p = 0.032, p = 0.012, respectively). Pulmonary pulse transit time and TAPSE was shorter in the patients with SSc (p = 0.006, p = 0.015, respectively). In correlation analysis, pPTT was inversely correlated with RVMPI (r = - 0.435, p = 0.003), eSPAP (r = - 0.434, p = 0.003), and disease duration (r = - 0.595, p = 0.003). Conversely, it positively correlated with TAPSE (r = 0.345, p = 0.022)., Conclusion: pPTT was found to be shorter in SSc patients. pPTT might serve as a surrogate marker of pulmonary hemodynamics in patients with SSc, even prior to the development of pulmonary hypertension.

Purpose: We aimed to evaluate changes in retinal, choroidal, ganglion cell complex (GCC) and retinal nerve fiber layer (RNFL) thicknesses in genetically diagnosed adult patients with familial Mediterranean fever (FMF)., Methods: A total of 50 eyes of 50 genetically diagnosed patients with FMF and 50 eyes of controls were analyzed. Patients were recruited from the Genetic Diagnostic Center of Dışkapı Yıldırım Beyazıt Research and Training Hospital, Turkey. Retinal and choroidal thicknesses were obtained using spectral-domain optical coherence tomography from choroid, retina, GCC, and RNFL., Results: Average baseline choroidal thickness was statistically significantly thinner in patients with FMF than controls at C center (325.85 ± 30.8 µm and 338.97 ± 23.9 µm, respectively, p = 0.038), C nasal500 (328.77 ± 31.6 µm and 349.00 ± 23.3 µm, respectively, p = 0.002), C nasal1000 (324.97 ± 33.6 µm and 351.23 ± 23.8 µm respectively, p = 0.0001) and C nasal1500 (324.75 ± 37.1 µm and 344.61 ± 27.3 µm, respectively, p = 0.008). However, there was no significant difference in temporal choroidal thickness (C temporal500, C temporal1000 and C temporal1500 ) in patients with FMF compared to controls (p > 0.05). There were no significant differences in retinal, GCC and RNFL thicknesses between the groups (p > 0.05)., Conclusion: We hypothesize that the chronic inflammation seen in FMF could be the reason for the reduction seen in choroidal thickness in adult patients with FMF. Retinal, GCC and RNFL thicknesses did not differ from controls.

Objective: The aim was to assess the characteristics of PsA, find out how well the disease is controlled in real life, demonstrate the treatments and identify the unmet needs., Methods: The PsA registry of Turkey is a multicentre Web-based registry established in 2014 and including 32 rheumatology centres. Detailed data regarding demographics for skin and joint disease, disease activity assessments and treatment choices were collected., Results: One thousand and eighty-one patients (64.7% women) with a mean (sd) PsA duration of 5.8 (6.7) years were enrolled. The most frequent type of PsA was polyarticular [437 (40.5%)], followed by oligoarticular [407 (37.7%)] and axial disease [372 (34.4%)]. The mean (sd) swollen and tender joint counts were 1.7 (3) and 3.6 (4.8), respectively. Of these patients, 38.6% were on conventional synthetic DMARD monotherapy, 7.1% were on anti-TNF monotherapy, and 22.5% were using anti-TNF plus conventional synthetic DMARD combinations. According to DAS28, 86 (12.4%) patients had high and 105 (15.2%) had moderate disease activity. Low disease activity was achieved in 317 (45.7%) patients, and 185 (26.7%) were in remission. Minimal disease activity data could be calculated in 247 patients, 105 of whom (42.5%) had minimal disease activity. The major differences among sexes were that women were older and had less frequent axial disease, more fatigue, higher HAQ scores and less remission., Conclusion: The PsA registry of Turkey had similarities with previously published registries, supporting its external validity. The finding that women had more fatigue and worse functioning as well as the high percentage of active disease state highlight the unmet need in treatment of PsA., (© The Author 2016. Published by Oxford University Press on behalf of the British Society for Rheumatology. All rights reserved. For Permissions, please email: journals.permissions@oup.com.)

Purpose: To evaluate the association between familial Mediterranean fever (FMF) and keratoconus (KC)., Methods: This retrospective case-control study was performed to compare the prevalence of KC in patients with FMF with the corresponding prevalence in control patients without FMF referred to Genetic Diagnostic Center at Diskapi Yildirim Beyazit Training and Research Hospital from June 2012 to June 2015. We included all 100 patients with FMF. Each FMF-affected patient was matched to 3 controls., Results: None of the patients in the control group (0%, 0/300) had KC, whereas 4 of 100 patients with FMF (4%) had KC (P < 0.004). Three of 33 patients with a homozygous mutation (9.1%) (M694V/M694V in 2 cases and M680I/M680I in 1 case) and 1 of the 46 patients with a compound heterozygous mutation (2.2%) (M694V/M680I) had KC, whereas none of the 21 patients with a heterozygous mutation (0%) had KC. All patients with KC were women, and mean age was 40.8 years (range, 30-51). Although 1 of the 4 patients with KC had hypertension and type 2 diabetes mellitus, the other 3 patients did not have any systemic illness except FMF. When we compared the prevalence of KC in patients with FMF (4%) with the highest prevalence of KC reported in the literature (0.2%), FMF was a predisposing factor to develop KC [odds ratio: 18.1 (95% CI: 11.9-27.5)] especially in patients with a homozygous mutation [odds ratio: 43.4 (95% CI: 28.6-65.7)]., Conclusions: Mediterranean fever (MEFV) gene mutations, particularly in homozygous mutations of the MEFV gene, may be a predisposing factor in the development of KC.