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© 2023, The Author(s), under exclusive licence to International League of Associations for Rheumatology (ILAR).Background: This study aimed to assess the mortality of PsA before and during the COVID-19 pandemic. Methods: From the prospective, multicenter PsART-ID (Psoriatic Arthritis Registry-International Database), patients from Turkey were analyzed by linking the registry to the Turkish Cause of Death Registry. The outcome of interest was death from any cause, pre-pandemic (since the onset of registry—March 2014–March 2020), and during the pandemic (March 2020–May 2021). The crude mortality rate and standardized mortality ratio (SMR) were determined. Results: There were 1216 PsA patients with a follow-up of 7500 patient-years. Overall, 46 deaths (26 males) were observed. In the pre-pandemic period, SMR for PsA vs the general population was 0.95 (0.61–1.49), being higher in males [1.56 (0.92–2.63)] than females [0.62 (0.33–1.17)]. The crude mortality rate in PsA doubled during the pandemic (pre-pandemic crude mortality rate: 5.07 vs 10.76 during the pandemic) with a higher increase in females (2.9 vs 8.72) than males (9.07 vs 14.73). Conclusion: The mortality in PsA was found similar to the general population in the pre-pandemic era. The mortality rates in PsA doubled during the pandemic. Whether PsA patients have more risk of mortality than the general population due to COVID-19 needs further studies.Key Points• Decrease in mortality in PsA might be expected with the more effective treatment options and better disease control.• A crude mortality rate is comparable to the general population and not increased until the pandemic.• Currently, there is a 2-fold increase in crude mortality rate possibly due to the COVID-19.

The respiratory system is often affected by systemic sclerosis (SSc), a connective tissue disease characterized by fibrosis, vasculopathy, and inflammation. As a result, especially exertional dyspnea may occur in SSc patients. Evaluation of attitudes towards dyspnea is important in terms of preventing negative consequences such as kinesiophobia. However, no validated tool is available for assessing dyspnea-related kinesiophobia for patients with SSc. The aim of the present study was to perform the Turkish validation of the Breathlessness Beliefs Questionnaire for SSc (BBQ-SSc), which was adapted from the Tampa Kinesiophobia Scale, and to investigate its relationships with physical and clinical characteristics.Fifty patients with SSc (47 females) were included in the study. The patients were evaluated regarding pulmonary function tests, respiratory muscle strength, patient-reported dyspnea, quality of life related to respiratory problems, mood, and fatigue. The patients were re-evaluated 1 week later for the test-retest reliability of the BBQ-SSc.Internal consistency (Cronbach's alpha: 0.862) and test-retest validity (ICC: 0.831; 95% CI: 0.702-0.907) of the 12-item Turkish BBQ-SSc were found to be good. Principal component analysis confirmed the two-dimensional structure of the questionnaire. The scores of the questionnaire were associated with duration of illness, patient-reported dyspnea, quality of life related to respiratory problems, mood, and fatigue (p 0.05).According to our results, the 12-item Turkish BBQ-SSc is a reliable and valid tool to assess dyspnea-related kinesiophobia in SSc patients. Key Points • There are no structured tools available for assessing dyspnea-related kinesiophobia in patients with systemic sclerosis (SSc) • The 12-item Turkish Breathlessness Beliefs Questionnaire for SSc (BBQ-SSc) is a reliable and valid tool to assess dyspnea-related kinesiophobia in SSc patients.

To investigate the effect of cervical stabilization exercises on cervical position error in patients with axial spondyloarthritis (axSpA).Thirty-nine patients with axSpA were randomly allocated to two groups as exercise group (n = 20, 11 males) and control group (n = 19, 12 males). The exercise group performed a progressive home-based cervical stabilization exercise program, while the control group did not receive any exercise intervention. To control exercise adherence and progression, text messages and video instructions were delivered via a freeware and cross-platform messaging service on a weekly basis. All patients were evaluated regarding physical characteristics, disease activity (Bath Ankylosing Spondylitis Disease Activity Index), functional status (Bath Ankylosing Spondylitis Functional Index), and spinal mobility (Bath Ankylosing Spondylitis Metrology Index). Cervical position error was evaluated in flexion, extension, rotation, and lateral flexion directions. All evaluations were performed at baseline and after 6 weeks.Baseline physical and disease-related characteristics were similar between the groups (p 0.05). After 6 weeks, significant improvements were observed in cervical position error in all directions in the exercise group (p 0.05), whereas no improvements were detected in the control group (p 0.05).A 6-week home-based cervical stabilization exercise program seems to be beneficial for improving impaired cervical proprioception in patients with axSpA.ZIEL: Ziel der Arbeit war es, die Wirkung von Übungen zur Stabilisierung der Halswirbelsäule auf deren zervikaler Positionsfehler bei Patienten mit axialer Spondyloarthritis (axSpA) zu untersuchen.Dazu wurden 39 Patienten mit axSpA randomisiert in 2 Gruppen eingeteilt, eine Übungsgruppe (n = 20, 11 m.) und eine Kontrollgruppe (n = 19, 12 m.). Die Übungsgruppe führte ein stufenweise aufbauendes Heimprogramm zur Halswirbelsäulenstabilisierung durch, während die Kontrollgruppe keine Übungsintervention erhielt. Um die Einhaltung der Übungen und ihren Fortschritt zu kontrollieren, wurden wöchentlich Textnachrichten und Videoanleitungen über einen freien und plattformübergreifenden Messagerdienst gesendet. Sämtliche Patienten wurden hinsichtlich körperlicher Merkmale, Krankheitsaktivität (Bath Ankylosing Spondylitis Disease Activity Index), funktionellem Status (Bath Ankylosing Spondylitis Functional Index), und Wirbelsäulenbeweglichkeit (Bath Ankylosing Spondylitis Metrology Index) untersucht. Zervikale Positionsfehler der Halswirbelsäule wurden in Flexion, Extension, Rotation und Lateralflexion beurteilt. Alle Untersuchungen wurden zu Anfang der Studie und nach 6 Wochen durchgeführt.Die körperlichen und krankheitsbezogenen Merkmale zu Anfang waren zwischen den beiden Gruppen ähnlich (p 0,05). Nach 6 Wochen wurden in der Übungsgruppe signifikante Verbesserungen der zervikalen Positionsfehler der Halswirbelsäule in allen Richtungen festgestellt (p 0,05), während in der Kontrollgruppe keine Verbesserungen zu beobachten waren (p 0,05).Ein 6‑wöchiges häusliches Übungsprogramm zur Stabilisierung der Halswirbelsäule scheint vorteilhaft zu sein, um die beeinträchtigte zervikale Propriozeption bei Patienten mit axSpA zu verbessern.

Systemic sclerosis (SSc) is a connective tissue disorder characterized by collagen deposits in various organs. Skin involvement is one of the most common symptoms and along with vascular damage, may deteriorate hand functions. However, the status of hand functions has generally been investigated using patient-reported measures in patients with SSc. The aim of the present study was to investigate performance-based hand functions in patients with SSc using the Sollerman Hand Function Test (SHFT).A total of 39 patients with SSc (33 females) were included in the study. Twenty-four patients were classified as limited cutaneous SSc (lcSSc), while 15 patients were classified as diffuse cutaneous SSc (dcSSc). Hand-related physical characteristics were evaluated using the Modified Hand Mobility in Scleroderma Test, grip strength, and pinch strengths. The Duruoz Hand Index (Cochin Hand Functional Disability Scale), Disability of Arm, Shoulder, and Hand Questionnaire, Health Assessment Questionnaire, and Scleroderma Health Assessment Questionnaire were used as patient-reported measures. Performance-based hand functions were evaluated using SHFT.No significant differences were observed between lcSSc and dcSSc subtypes regarding performance-based and patient-reported hand functions (p 0.05). SHFT scores significantly correlated with hand-related physical characteristics and patient-reported hand functions (p 0.05). The highest correlation was determined between SHFT and the Duruoz Hand Index (rho: -0.652, p 0.001).According to our results, performance-based hand functions seem not to be affected by disease subtype. Performance-based hand functions may partially be captured by the patient-reported outcomes, especially the Duruoz Hand Index, in patients with SSc.ZIEL: Die systemische Sklerose (SSc) ist eine Bindegewebserkrankung, welche durch Kollagenablagerungen in verschiedenen Organen gekennzeichnet ist. Eines der häufigsten Symptome stellt die Hautbeteiligung dar, die in Kombination mit einer Gefäßschädigung auch die Funktionen der Hand beeinträchtigen kann. Allgemein wurde allerdings der Status der Handfunktionen bisher mittels Parametern gemäß den subjektiven Angaben von SSc-Patienten untersucht. Ziel der vorliegenden Studie war es, die leistungsbezogenen Handfunktionen bei Patienten mit SSc anhand des Sollerman Hand Function Test (SHFT) zu ermitteln.Dazu wurden 39 Patienten mit SSc (davon 33 Frauen) in die Studie einbezogen; bei 24 Patienten wurde die Erkrankung als limitierte kutane SSc (lcSSc) eingestuft, während sie bei 15 Patienten als diffuse kutane SSc (dcSSc) klassifiziert wurde. Handbezogene körperliche Merkmale wurden mithilfe des Modified Hand Mobility in Scleroderma Test, der Griffstärke und Kneifkraft beurteilt. Der Duruoz Hand Index (Cochin Hand Functional Disability Scale), Disability of Arm, Shoulder, and Hand Questionnaire, Health Assessment Questionnaire und der Scleroderma Health Assessment Questionnaire wurden als Parameter der subjektiven Angaben von Patienten eingesetzt. Die leistungsbasierten Handfunktionen wurden mittels SHFT beurteilt.Zwischen den Subtypen lcSSc und dcSSc wurden keine signifikanten Unterschiede in Bezug auf leistungsbasierte Handfunktionen und Handfunktionen nach patientenbezogenen Angaben (p 0,05) festgestellt. Die SHFT-Werte waren in signifikanter Weise mit den handbezogenen körperlichen Merkmalen und den Handfunktionen nach Patientenangaben korreliert (p 0,05). Dabei wurde die höchste Korrelation zwischen SHFT und dem Duruoz Hand Index (rho: −0,652; p 0,001) festgestellt.Den vorliegenden Ergebnissen zufolge scheinen die leistungsbasierten Handfunktionen nicht vom Krankheitssubtyp beeinflusst zu werden. Leistungsbasierte Handfunktionen bei Patienten mit SSc können mittels der von Patienten berichteten Ergebnisse erfasst werden, insbesondere mit dem Duruoz Hand Index.

To evaluate the choroidal and retinal layers with optical coherence tomography (OCT) and retinal microvascular structures with optical coherence tomography angiography (OCTA) in systemic lupus erythematosus (SLE) patients. In this prospective, cross-sectional and comparative study, a total of 35 SLE patients and 35 healthy control participants were included. SLE patients who were using hydroxychloroquine (HCQ) and/or immunosuppressive agents are evaluated with OCT and OCTA. SLE patients who have no HCQ maculopathy observed in OCT were included in the patient group. Mean macular thickness and ganglion cell inner plexiform layer (GC-IPL) thicknesses were thinner in the patient group. When the parameters obtained with OCTA were evaluated, vessel (VD) and perfusion density (PD) were significantly lower in the patient group. Central foveal thickness and foveal avascular zone parameters were negatively correlated. In addition, VD and PD, and GC-IPL thicknesses were positively correlated. Application of OCTA for the evaluation of microvasculature in SLE patients may be useful in subclinical changes.

The aim of this study is to evaluate the retinal and choroidal structures in r- and nr-axSpA patients using spectral domain optical coherence tomography (SD-OCT) and to compare changes with healthy controls. In this cross-sectional study, 70 axSpA patients (50 radiographic- and 20 nr-axSpA) and 50 healthy control subjects were included. Choroidal thickness (ChT), macular thickness, retinal nerve fiber layer (RNFL), and the ganglion cell complex (GCC) were measured by SD-OCT. For ChT values, seven lines at nasal and temporal were drawn at 500-μm intervals, centering the subfoveal sclerochoroidal junction. Analysis of the data was performed with the SPSS program. Mann–Whitney U test was performed for comparison of non-normally distributed continuous data; Student’s t test was used for normal distributed data. No significant difference was observed between 70 (66% male; mean age 39.7 ± 10.4 years) axSpA patients (50 radiographic and 20 nr-axSpA) and 50 (mean age 41.2 ± 6.2 years) healthy control subjects (p 0.417). R-axSpA and nr-axSpA groups and control group were similar in terms of spherical equivalent, intraocular pressure, axial length, and body mass index (p 0.574, p 0.874, p 0.918, p 0.344, respectively). While mean macular and GCC thicknesses were significantly lower in the patient group than in the healthy group, there was no significant difference between the two groups in terms of RNFL thickness. The present study showed that there was no significant relationship between markers and scores indicating disease activity and ChT, MT, RNFL, and GCC thicknesses. However, an increase in choroidal thickness and involvement of the retinal layers has also been demonstrated in patients with spondyloarthritis. In addition, the relationship between disease activity and retinal layer involvement is remarkable in the r-axSpA group.

Background To examine the activity of the mammalian target of rapamycin (mTOR) pathway and its regulators, transforming growth factor (TGF)-β1 and phosphatase and tensin homolog (PTEN), in minor salivary gland biopsies of Sjogren’s syndrome (SS) and systemic sclerosis (SSc) patients. Methods We retrospectively evaluated SS, SSc, and SS-SSc overlap patients admitted to our outpatient rheumatology clinic between January 2007 and December 2015 who underwent a minor salivary gland biopsy. Patient demographics and some clinical features were obtained from hospital records. Immunohistochemistry was used to analyze total mTOR, total PTEN, and TGF-β1 expression in the biopsied tissues. The biopsy specimens were also examined for the presence and degree of fibrosis. Results Minor salivary gland biopsies of 58 SS, 14 SSc, and 23 SS-SSc overlap patients were included in the study. There was no significant difference in mTOR expression between these groups (P = 0.622). PTEN protein was expressed in 87.2% of patients with SS, 57.9% with overlap syndrome, and 100% of the SSC patients, and these differences were statistically different (P = 0.023). Although ductal epithelial TGF-β1 expression was similar between the groups (P = 0.345), acinar cell expression was found to be more frequent in the SSc (72.7%) and overlap patients (85.7%) in comparison with the SS cases (58.2%; P = 0.004). Conclusion mTOR may be one of the common pathways in the pathology of both SS and SSc. Hence, there may be a role for mTOR inhibitors in the treatment of both diseases. Additionally, PTEN and TGF-β1 expression may be a distinctive feature of SSc.

Herein, we reported our experience in colchicine-resistant familial Mediterranean fever (FMF) patients who are treated with anti-interleukin-1 (IL-1) drugs. A retrospective review of medical records of anti-IL-1 recipients was performed. The main clinical characteristics of these patients and the evolution after anti-IL-1 were recorded. There were 20 patients (11 male [M] and 9 female [F]). Despite regular colchicine treatment, median number of attacks per month and per year was 1 (1-4) and 12 (4-50), respectively. Twelve patients were receiving anakinra, and eight patients were treated with canakinumab. The number of monthly and yearly attacks after IL-1 treatment was significantly decreased after the biologic agent (p < 0.05). One patient did not respond to the treatment, and one patient developed serious infection during anti-IL-1. We also observed a significant decrease in proteinuria in the amyloidosis complicated FMF patients. Anti-IL-1 targeting drugs seem safe and effective therapies in colchicine-resistant FMF.

The aim of this study was to compare the performance characteristics of two answer modalities for BASDAI and BASFI in patients with AS and to show validity and reliability of NRS in Turkish version. BASDAI and BASFI were simultaneously employed with a 10-cm VAS and an 11-point NRS. Internal consistency was assessed by using Cronbach's alpha coefficients. Testing was performed on baseline and next day under standardized conditions. Construct validity was determined by association of these measures with ASDAS, DFI, global disease activity, pain scores, ASQOL, HAQ, and SF-36. We also tested the ability of NRS version of BASDAI and BASFI to detect changes. A total 114 patients with AS according to the modified New York criteria were included. There was a good agreement between the total scores of each instrument on day 0 (ICC values were 0.894-0.934). Scores of the both answering modality of each instrument were correlated with ASDAS-CRP, and the scores of DFI, patient global assessment of disease activity and patient-reported pain, ASQOL, HAQ. Response time for BASDAI and BASFI was significantly shorter in NRS answer modality (P < 0.001). Our patients found NRS modality more comprehensible and easier to understand (P < 0.001). Our results showed the validity of NRS version of BASDAI and BASFI and provided an evidence for using these questionnaires in 11-point NRS answer modality in Turkish AS patients.

To evaluate the circulating levels of adipokines (leptin and adiponectin) and ghrelin in patients with familial Mediterranean fever (FMF) and also to assess the relationships between these molecules and disease-related parameters. Forty-eight FMF patients in attack-free period (31 men, [M], 17 women, [F], mean age 35.8 +/- A 8.6 years, and a mean body mass index [BMI] of 24.7 +/- A 3.1) and 40 age-, sex-, and BMI-matched healthy controls (24 M, 16 F, mean age 35.5 +/- A 8.5 years, and a mean BMI of 24.5 +/- A 2.8) were included in the study. Patients and controls with a history of any other chronic diseases and obese or underweight subjects were excluded. High-sensitive C-reactive protein (hs-CRP), leptin, adiponectin, and total ghrelin concentrations were studied. Age, sex, BMI, waist circumference, and smoking status were similar between FMF patients and controls (P > 0.05). Adipose tissue-derived molecules including leptin, and adiponectin were lower than healthy controls but only adiponectin levels reached the statistically significance (16.7 +/- A 8.9 ng/ml vs. 27.7 +/- A 15.9 ng/ml, P < 0.001) and leptin concentrations just missed significance (25.2 +/- A 16.2 ng/ml vs. 34.9 +/- A 27.2 ng/ml, P = 0.051). Ghrelin concentrations were not different between the groups. Adiponectin levels were significantly and negatively correlated with hs-CRP (P < 0.05, r = -0.24). The results of this study suggest that low-grade chronic inflammation during attack-free period in FMF patients may suppress adiponectin production or low levels of adiponectin might contribute to subclinical inflammation in these patients.

The aim of this study was to compare the effect of chronic inflammation on insulin resistance, serum leptin levels, and body composition (BC) in patients with ankylosing spondylitis (AS) and healthy controls. Twenty-eight AS patients and 17 healthy controls were included in this study. Subjects with hypertension, diabetes, hyperlipidemia, and obesity were excluded. Acute phase reactants and serum levels of glucose, insulin, lipids, and leptin were studied. BC was determined anthropometrically and by foot-to-foot body fat analyzer (BIA, bioelectrical impedance analysis). Quantitative insulin-sensitivity check index, homeostasis model assessment for insulin resistance, and McAuley indices were calculated. Spinal mobility was assessed by the Bath Ankylosing Spondylitis Metrology Index (BASMI). Patients were also evaluated with the Bath Ankylosing Spondylitis Functional Index and the Bath Ankylosing Spondylitis Disease Activity Index. Age, sex distribution, smoking status, serum lipids, insulin concentrations, and insulin resistance indices were comparable between AS patients and controls (p > 0.05). However, acute phase reactants were significantly higher and leptin levels were significantly lower in the AS patients than in controls (p < 0.05). Fat percent assessed by both BIA and anthropometrical methods was lower in the male and female AS patients than in controls, and this reduced fat level reached statistical significance for men (p < 0.05). There were significant correlations between percent body fat, body mass index, leptin, age, and BASMI (p < 0.05; r = 0.6, 0.75, 0.35, -0.41, respectively). On the other hand, body fat percent, waist-to-hip ratio, C-reactive protein, and BASMI were significantly correlated with serum leptin levels (p < 0.05; r = 0.75, -0.42, -0.52, -0.47, respectively). Chronic inflammatory condition in AS may be responsible for the reduced body fat content and lower circulating leptin concentrations. Insulin levels and insulin resistance indices seem similar in patients and controls in the absence of classic vascular risk factors.

Colchicine is effective in controlling the attacks and preventing the development of amyloidosis. Moreover many new generation drugs may be used in FMF treatment. About 5-10% of the patients do not respond to colchicine and inadequate response to other drugs is not as clear as the unresponsiveness of colchicine.

Henoch-Schönlein purpura (HSP) belongs to the category of systemic small-vessel vasculitis. Although long-term outcome is generally good, serious complications may occur. Thrombosis and priapism have been reported only as extremely rare complications of HSP. We describe a 37-year-old man who developed recurrent thrombotic events shortly after he had been diagnosed as having HSP. Although he had additional risk factors for thrombosis, such as prothrombin G20210A mutation and use of celecoxib before the last episode, temporal relation of the thrombotic attacks to the onset of HSP suggest that the disease itself may lead to a prothrombotic state. This case is the first adult HSP patient with priapism, which probably developed secondary to thrombosis of the dorsal penile vein.

Familial Mediterranean fever (FMF) is an autosomal recessive disease, characterized by recurrent attacks of fever and serositis. Musculoskeletal involvement mostly in the form of arthritis is a common manifestation of FMF [1]. Moreover, an increased frequency of spondyloarthritis (SpA) including ankylosing spondylitis (AS) has been suggested by several case reports and two large observational studies [1, 2]. The FMF gene (MEFV), which is located on the short arm of chromosome 16, encodes an immunoregulatory protein called pyrin or marenostrin [3, 4]. There are some reports which suggest a role for the mutation of this gene in some other rheumatic diseases [5, 6]. In a previous study evaluating the signiWcance of paired MEFV mutations in asymptomatic individuals, some of whom were Wrst-degree relatives of FMF patients, we identiWed one patient who was homozygous for M694V, but who had all the typical features of AS [7]. In this report, we are describing detailed information on this patient and one additional similar patient.

Introduction Familial Mediterranean fever (FMF) is an auto-inflammatory disease characterized by recurrent attacks of fever and serositis. Limited data suggest that the prevalence of sacroiliitis is increased in patients with FMF. In our present study, we assessed the prevalence of spondyloarthritis (SpA), including ankylosing spondylitis (AS), among a cohort of FMF patients and their unaffected first-degree relatives (FDRs). Methods The current study cohort comprised a consecutive group of 201 unrelated patients with FMF and 319 FDRs (≥ 16 years old). These subjects were examined according to a standard protocol. Results A total of 157 FMF patients (78.1%) and 233 (73%) unaffected FDRs reported back pain. Fifteen FMF patients (7.5%) and nine unaffected FDRs fulfilled the modified New York (mNY) criteria for AS. One additional FDR with AS was identified after review of the medical records. None of the FMF patients with AS was HLA-B27 positive. The allele frequency of M694V among the FMF patients with radiographic sacroiliitis was significantly higher in comparison with those without sacroiliitis (OR 4.3). When compared with the general population, the risk ratios for SpA and AS among the FDRs of our FMF patients were 3.3 (95% CI; 2.0 to 5.5) and for AS 2.9 (95% CI; 1.3 to 6.4), respectively. Conclusions Our study suggests that a) factors other than HLA-B27 play a role in the association of FMF and SpA/AS; b) MEFV gene variations may be one of the geographic/region-specific potential pathogenetic links between these two disorders in the Turkish population.

Introduction The current markers of disease activity in Takayasu arteritis (TA) are insufficient for proper assessment. We investigated circulating levels of unacylated and acylated ghrelin, leptin and adiponectin and their relationships with disease activity in patients with TA. Methods This study included 31 patients with TA and 32 sex-, age- and body mass index-matched healthy controls. Disease activity was assessed in TA patients using various tools, including Kerr's criteria, disease extent index-Takayasu, physician's global assessment, radiological parameters, and laboratory markers. Plasma unacylated and acylated ghrelin, and serum leptin and adiponectin levels were measured using an enzyme-linked immunosorbent assay. Results Unacylated and acylated ghrelin levels were found to be significantly lower in TA patients than that in healthy controls. Patients with active disease had lower unacylated ghrelin levels than those with inactive disease and had lower acylated ghrelin levels than healthy controls. Ghrelin levels were negatively correlated with various parameters of disease activity. The leptin/ghrelin ratio was significantly higher in TA patients than controls. It was positively correlated with disease activity. There was a positive correlation between unacylated and acylated ghrelin and a negative correlation between leptin and ghrelin. There was no statistical difference in adiponectin levels between TA patients and controls. The radiological activity markers were positively correlated with other parameters of disease activity. Conclusions This study suggests that plasma unacylated and acylated ghrelin levels may be useful in monitoring disease activity and planning treatment strategies for patients with TA. The serum leptin level and leptin/ghrelin ratio may also be used to help assess the disease activity.