Search

© 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.

Background/aim: Familial Mediterranean Fever (FMF) is the prototype of hereditary autoinflammatory disorders and caused by mutations on the MEFV gene located on the short arm of chromosome 16. Although some MEFV variants are clearly associated with disease phenotype, there are numerous variants with unknown clinical association which are termed as variants of uncertain significance (VUS). Here, we present clinical correlations of VUS in a large cohort of adult FMF patients from three tertiary centers located in Central Anatolia. Materials and methods: All patients were recruited from FMF in Central Anatolia (FiCA) cohort. Demographic (sex, age at disease onset) and clinical features (disease characteristics, attack frequency, mean colchicine dose, colchicine nonresponsiveness, amyloidosis, and persistent inflammation) of patients with VUS were compared with those harboring pathogenic variants. Disease severity and damage were also evaluated using international severity score for FMF (ISSF) and autoinflammatory disease damage index (ADDI), respectively. Results: Among 971 participants included, MEFV gene analysis results were available for 814 patients. Twenty-six (3.2%) patients had single heterozygous VUS and 54 (6.6%) had pathogenic/VUS complex heterozygous variants. Patients with single heterozygous VUS had similar demographic/clinical features, ISSF and ADDI scores compared to those with single heterozygous pathogenic variant (p > 0.05 for all). No difference was observed in the demographic and clinical features of patients with single heterozygous pathogenic mutation and pathogenic/VUS complex heterozygous variant (p > 0.05 for all). ISSF and ADDI scores were lower in pathogenic/VUS complex heterozygous patients than those harboring single pathogenic mutation (p = 0.006 and 0.004, respectively). Conclusion: Our findings suggest that patients with single heterozygous VUS has mild FMF phenotype similar to those with single pathogenic mutation. Pathogenic/VUS complex heterozygosity does not lead to a more severe clinical phenotype than having a single pathogenic variant.

Background/aim: to evaluate treatment adherence and predictors of drug discontinuation among patients with inflammatory arthritis receiving bDMARDs within the first 100 days after the announcement of the COVID-19 pandemic. Materials and methods: a total of 1871 patients recorded in TReasure registry for whom advanced therapy was prescribed for rheumatoid arthritis (RA) or spondyloarthritis (SpA) within the 3 months (6-9 months for rituximab) before the declaration of COVID-19 pandemic were evaluated, and 1394 (74.5%) responded to the phone survey. Patients' data regarding demographic, clinical characteristics and disease activity before the pandemic were recorded. The patients were inquired about the diagnosis of COVID-19, the rate of continuation on bDMARDs, the reasons for treatment discontinuation, if any, and the current general disease activity (visual analog scale, [VAS]). Results: a total of 1394 patients (493 RA [47.3% on anti-TNF] patients and 901 SpA [90.0% on anti-TNF] patients) were included in the study. Overall, 2.8% of the patients had symptoms suggesting COVID-19, and 2 (0.15%) patients had PCR-confirmed COVID-19. Overall, 18.1% of all patients (13.8% of the RA and 20.5% of the SpA; p = 0.003) discontinued their bDMARDs. In the SpA group, the patients who discontinued bDMARDs were younger (40 [21-73] vs. 44 years [20-79]; p = 0.005) and had higher general disease activity; however, no difference was relevant for RA patients. Conclusion: although the COVID-19 was quite uncommon in the first 100 days of the pandemic, nearly one-fifth of the patients discontinued bDMARDs within this period. The long-term effects of the pandemic should be monitored., Hacettepe Rheumatology Society

Methotrexate (MTX) is one of the most commonly used disease-modifying anti-rheumatic drugs which can cause gastrointestinal side effects. MTX intolerance is defined as gastrointestinal and behavioral symptoms occurring before and after MTX administration. This study aims to evaluate and compare the frequency of methotrexate intolerance in adult and pediatric patients. Patients with a rheumatic disease who used oral or parenteral methotrexate for at least 3 months were included in the study. Methotrexate intolerance was assessed using the Methotrexate Intolerance Severity Score (MISS) questionnaire and visual analog scale (VAS). In the pediatric patient group, the MISS questionnaire and VAS assessment were applied to both patients and families. A total of 200 patients, 100 of whom were children, were enrolled in the study. The mean age for children and adults were 11.9 (± 3.7) and 52.0 (± 10.9). The prevalence of MTX intolerance was higher in the pediatric group, 64.0 and 10.0% (p

Takayasu's arteritis (TAK) is a chronic vasculitis, affecting predominantly the aorta and/or its major branches. The aim of this study was to compare the differences between childhood and adult onset TAK.We retrospectively evaluated 179 TAK patients followed between August 2005 and July 2019. Demographic characteristics, laboratory features, disease activity, echocardiographic data at diagnosis and treatment regimens in the disease course were compared between the paediatric and adult onset patients.Twenty-five paediatric-onset (18 years of age at diagnosis) and 154 adult-onset patients (≥18 years of age at diagnosis) were enrolled. The mean age at diagnosis for children and adults were 13.6±4 and 35.6±13, respectively. Paediatric onset TAK patients had more intense inflammation at the time of diagnosis reflected in their clinical findings. Acute phase reactants were high in all paediatric patients and significantly higher in patients with paediatric-onset TAK (p=0.006 and p=0.005, respectively). Abdominal predominant disease was more common in the paediatric group, in contrast, focal disease and aortic arch predominant disease were more common in the adult group. Ascending aortic dilatation, left ventricular hypertrophy and moderate-severe aortic insufficiency were more frequent in echocardiography findings of paediatric onset TAK patients. In comorbidities, hypertension was more common in paediatric TAK patients during follow-up, whereas cerebrovascular disease was more common in adult patients.Our paediatric onset TAK patients presented with a more severe inflammation and more widespread vascular involvement. Multicentre studies from different geographic areas are needed to verify our observation and understand the underlying causes.

Tissue hypoxia due to microvasculopathy is the main cause of digital ulcers (DUs) in systemic sclerosis (SSc). Reduced oxygen delivery (DO2) to the tissues may also contribute to the development of DU. This study was conducted to investigate the association between DO2 and DUs in patients with SSc.In all, 111 patients and 30 healthy controls were enrolled. DO2 was calculated by using the formula; DO2 = Cardiac output × arterial oxygen saturation (SpO2) × serum haemoglobin level × 1.39 × 10. Both right index finger SpO2 measurements (index-SpO2) and highest value of SpO2 (maximum SpO2) obtained among the fingers of the subjects were used for the calculations and DO2 results were adjusted both for weight and body surface area (BSA).Mean DO2 was lower in SSc patients as compared to controls in all 4 different calculations but the difference was only statistically significant when using index-SpO2 and adjusting for BSA (498 mL/min/mDO2 in SSc patients seems to be lower than healthy controls. However, DO2 is similar between the patients with and without DUs. Our results suggest that the contribution of DO2 is negligible to the development of DU and support the major role of microvasculopathy in SSc patients with DUs.

Objective: The aim of the study is to describe the clinical characteristics of Antineutrophil cytoplasmic antibodies-associated vasculitis and to analyze the parameters affecting the outcome. Materials and Methods: The study is a retrospective cohort study. Totally 130 patients with Antineutrophil cytoplasmic antibodies-associated vasculitis (18 years and over) who were followed up between October 2014 and October 2019 were analyzed. Demographic data, laboratory values, clinical course, thorax computed tomography findings and treatment approaches were noted from the charts of patients. Patients were divided into two groups as those with pulmonary involvement and non-pulmonary involvement. Results: We retrospectively reviewed the medical records of 130 patients with Antineutrophil cytoplasmic antibodies-associated vasculitis; 111 with granulomatosis with polyangiitis, 15 with microscopic polyangiitis, 1 with eosinophilic granulomatosis with polyangiitis, and 3 with other types of vasculitis. The ratio of having the abnormality in thoracic computed tomography was 72.2%. There were 84 cases with pulmonary involvement and 46 cases with non-pulmonary involvement. The frequency of microscopic polyangiitis was significantly higher (p=0.034) in non-pulmonary involvement cases. There were 67 cases with proteinase 3 Antineutrophil cytoplasmic antibodies and 39 cases with myeloperoxidase Antineutrophil cytoplasmic antibodies positivity. Most of the cases with proteinase 3 Antineutrophil cytoplasmic antibodies positivity were classified as granulomatosis with polyangiitis, this was statistically significant. Recovery was referenced for the outcome. Any of the variables were found statistically significant effective on outcome. Conclusions: Cases with pulmonary involvement were more than the cases without pulmonary involvement in our study. microscopic polyangiitis was significantly higher in non- pulmonary involvement cases. We studied on a large group, and these significant findings may have important implications for the investigation, pathogenesis, and treatment of Antineutrophil cytoplasmic antibodies-associated vasculitis.

Objective To characterize the systemic phenotype of primary Sjögren’s syndrome at diagnosis by analysing the EULAR-SS disease activity index (ESSDAI) scores. Methods The Sjögren Big Data Consortium is an international, multicentre registry based on worldwide data-sharing cooperative merging of pre-existing databases from leading centres in clinical research in Sjögren’s syndrome from the five continents. Results The cohort included 10 007 patients (9352 female, mean 53 years) with recorded ESSDAI scores available. At diagnosis, the mean total ESSDAI score was 6.1; 81.8% of patients had systemic activity (ESSDAI score ≥1). Males had a higher mean ESSDAI (8.1 vs 6.0, P 65 years, P Conclusion The systemic phenotype of primary Sjögren’s syndrome is strongly influenced by personal determinants such as age, gender, ethnicity and place of residence, which are key geoepidemiological players in driving the expression of systemic disease at diagnosis.

Objective Persistent inflammation is an insidious and less studied feature of FMF. We investigated clinical determinants of persistent inflammation and its associations with individual damage items. Methods This is a cross-sectional analysis of 917 FMF patients, who fulfilled the Tel Hashomer criteria and had at least 6 months’ follow-up. Patients were stratified based on whether they had persistent inflammation. We used logistic regression analysis to investigate independent predictors of persistent inflammation and the associated individual damage items. Results One hundred and forty-two (15%) patients had persistent inflammation. Active FMF (54%) was the most prominent reason for the persistent inflammation. Spondylarthritis (16%), other inflammatory arthritis (8%) and IBD (2%) were other frequent reasons. Male gender, history of exertional leg pain, inflammatory comorbidities, M694V homozygosity, colchicine resistance, lower education levels and musculoskeletal attack dominance were found to be the independent predictors of persistent inflammation. Earlier disease onset led to a tendency towards persistent inflammation. Patients with persistent inflammation were more likely to suffer damage. There is an increased risk of developing proteinuria, amyloidosis and renal insufficiency. Conclusion We identified, for the first time, the predictors of persistent inflammation in adult FMF patients and related individual damage items of the Autoinflammatory Disease Damage Index. Persistent inflammation is insidious and one of the chief causes of damage; therefore, especially patients with these predictors should be followed up more closely. If detected, underlying inflammatory comorbidities should be assessed meticulously as early detection and proper treatment strategies may favourably impact the natural history of the disease.

Connection of small generatingunits in the medium voltage distribution system is increasing substantially.Such units, called distributed generation, can have a significant impact on,load flow, shortcircuit, voltage, frequency, power quality, stability,islanding and synchronized running, protection and reliability. In this study,the stability impact of a private sector power plant in Thrace region havingsynchronous generators driven by gas engine and gas turbine has beeninvestigated in terms of fault ride through capability. Critical clearing timehas been determined, once the criteria of fault ride through capability wereexplored. Grid operators do limit penetration levels of such plants and canalso disconnect available units when grid fails. Stability is affected byvoltage dip much worsely than penetration levels. It has been seen that thedisconnection of power plants is not necessary, when the voltage dip restrictedto 0,5 pu. Also that the stability is positively affected when the power factoris decreased to 0,8 from 1,0. The results also show that even 100% penetratedsynchronous generator based power plants in distribution system do not affectstability badly in steady state operation.

Background/aim Colchicine is the mainstay of treatment in FMF. However, in daily practice it is not easy to maintain effective colchicine doses in a substantial number of patients due to its side effects. In this study, we aimed to investigate prevalence and risk factors for colchicine side effects that limit optimal drug dosing and cause permanent discontinuation. Materials and methods All patients were recruited from “FMF in Central Anatolia” (FiCA) cohort, 915 adults with a minimum follow- up time of 6 months during which they had obeyed all treatment instructions. Demographic and anthropometric data, FMF disease characteristics, disease severity, complications, and treatment features were recorded on a web-based registry. Prevalence of colchicine intolerance and characteristics of intolerant patients were analyzed. Results Effective colchicine doses cannot be maintained in 172 (18.7%) subjects. Main side effects that limit optimal dosing were as follows: diarrhea in 99 (10.8%), elevation in transaminases in 54 (5.9%), leukopenia in 10 (%1.1), renal impairment in 14 (1.3%), myopathy in five (0.5%), and allergic skin reaction in two. Colchicine had to be permanently ceased in 18 (2%) patients because of serious toxicity. Male sex and obesity were found to be associated with liver toxicity, and having a normal body weight was associated with diarrhea. Chronic inflammation and proteinuria were more common in colchicine-intolerant patients, and they had reported more frequent attacks compared to those tolerating optimal doses. Conclusion Colchicine intolerance is an important problem in daily clinical practice, mainly due to diarrhea and liver toxicity. Suboptimal colchicine dosing is associated with complications.

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., Union ChimiqueBelge (UCB); Turkish Society for Rheumatology (TRD), D. Solmaz had funding from Union ChimiqueBelge (UCB) for an axial spondyloarthritis fellowship. S. Bakirci had funding from the Turkish Society for Rheumatology (TRD). S.Z. Aydin received honoraria from Abbvie, Celgene, UCB, Novartis, Jannsen, and Sanofi.

OBJECTIVES: To analyse the clinical and laboratory factors associated with bamboo spine. METHODS: Data of patients fulfilling the 2009 ASAS classification criteria for axial spondyloarthritis, registered in the national, multicentre, longitudinal, and observational database of TReasure was analysed. Radiographs were assessed using the Bath Ankylosing Spondylitis Radiologic Index (BASRI). Data of patients with a bamboo spine (Group 1) was compared to data derived from patients with a longstanding disease of at least 15 years but no syndesmophytes (Group 2). RESULTS: Out of the 5060 patients, 1246 had eligible radiographs. There were 111 patients (8.9%) with a bamboo spine. Male sex was more common among patients with bamboo spine. The median BMI of 27.7 (25.8-31.1) in Group1 was higher than the BMI of 25.9 (22.9-29.2) in Group 2 (p

Mouth and genital ulcers with inflamed cartilage (MAGIC) syndrome is characterized by overlapping features of relapsing polychondritis (RP) and Behcet's disease (BD). To date, no studies have defined the clinical spectrum of disease in a cohort of patients with MAGIC syndrome.Adult patients within an ongoing prospective, observational cohort study in RP were clinically assessed for MAGIC syndrome. A systematic review was conducted to identify additional cases of MAGIC syndrome by searching four databases: PubMed (US National Library of Medicine), Embase (Elsevier), Scopus (Elsevier) and Web of Science: Core Collection (Clarivate Analytics). The inclusion criteria used were: [1] patients of any age or gender who were diagnosed with MAGIC syndrome, or both RP and BD; [2] case report or case series study; [3] published from 1985 - July 2020; and [4] in English language. Risk of bias was assessed using a checklist developed by the authors and based on the Consensus-based Clinical Case Reporting (CARE) Guidelines. Search results screening, article inclusion, data extraction and risk of bais assessment was performed independently by two investigators. Clinical characteristics, particularly BD-related features, were compared between patients with MAGIC syndrome and cases of non-MAGIC RP. The performance characteristics of different criteria to classify MAGIC syndrome were also evaluated.Out of 96 patients with RP, 13 (14%) patients were diagnosed with MAGIC syndrome. For the systematic review, 380 articles were retrieved of which 90 were screened at title and abstract levels. Of these screened, 60 were excluded and 30 proceeded to full text review where an additional 8 were excluded. Twenty-two articles were included in our review and from which 27 additional cases of MAGIC syndrome were identified. Pooling all 40 cases together and comparing them with non-MAGIC RP, there was a significantly higher prevalence of ocular involvement (28% vs 4%, p0.01), cutaneous involvement (35% vs 1%, p0.01), GI involvement (23% vs 4%, p0.01), and CNS involvement (8% vs 0, p = 0.04) in MAGIC syndrome. A higher prevalence of aortitis (23% vs 1%, p0.01), Raynaud's phenomenon (54% vs 11%, p0.01), and elevated anti-collagen II antibodies (50% vs 9%, p = 0.04) were observed in MAGIC syndrome. Fulfillment of either McAdam's or Damiani's Criteria for RP plus the International Criteria for Behçet's Disease had excellent sensitivity (98%) to classify cases of MAGIC syndrome.A substantial proportion of patients with RP can be clinically diagnosed with MAGIC syndrome. These patients have features of RP, BD, and other unique features including aortitis, Raynaud's phenomenon and elevated anti-collagen II antibodies.

Objective Because of concerns about malignancy risks, using biological disease-modifying antirheumatic drugs (bDMARDs) in patients with a history of malignancy remains a challenging issue in rheumatology practice. This study aimed to investigate bDMARD preferences of physicians when treating of rheumatoid arthritis (RA) and spondyloarthritis (SpA) patients with a history of malignancy. Methods The data for this cross-sectional study were gathered from the TReasure database using a date range of December 2017 and January 2020. Biological disease-modifying antirheumatic drug preferences were analyzed for 40 RA patients and 25 SpA patients with a history of malignancy. Results The most frequently prescribed bDMARD was rituximab, which was given to 28 RA patients (70%). For 25 patients (62.5%), the time between the diagnosis of malignancy and starting on a bDMARD regimen was less than 60 months, with a median interval of 43.5 months. Among SpA patients, the preferred bDMARDs were secukinumab and etanercept, which were each administered to 7 patients (28%). For 13 SpA patients (52%), the time between the diagnosis of malignancy and starting on bDMARDs was less than 60 months, with a median interval of 97 months. Conclusions The observed bDMARD preferences may be related to the therapeutic effects of rituximab on lymphoproliferative malignancies, the protective effects of secukinumab on tumor progression, and the short half-life of etanercept. Biological disease-modifying antirheumatic drugs should be used in RA and SpA patients with malignancy in case of high inflammatory activity.

Objective Immunoglobulin G4-related disease (IgG4-RD) is a newly recognized fibro-inflammatory disease which affects many systems, as well as the cardiovascular system. Identifying the coronary involvement like periaortitis, coronary periarteritis and pericarditis is important, as they often cause unfavorable outcomes. Methods Eighty-one patients with IgG4-RD were retrospectively evaluated for symptomatic coronary artery involvement from Hacettepe University Vasculitis Research Center (HUVAC) database. The demographic, laboratory, radiologic and clinical characteristics of the patients were assessed. Results Among 81 patients with IgG4-RD, 6 patients (M/F:5/1) had coronary artery involvement. The patients' median age was 57 and serum IgG4 levels were above normal except for one case. All patients with coronary arteritis revealed an increased coronary vessel wall thickening and stenotic lesions. The coronary aneurysm and pericarditis were observed in half of the patients. Immunosuppressive treatments were given to all the patients and most of them followed in stable condition. Conclusion Coronary arteritis is a rare but notable manifestation of IgG4-RD. Although coronary periarteritis can cause significant morbidity and mortality, it seems better results can be achieved with early diagnosis and treatment.

Objective: To identify several clinical and/or laboratory parameters which can differentiate adult-onset Still's disease (AOSD) from other causes of fever of unknown origin (FUO) and create a clinician-friendly algorithm for this purpose. Methods: FUO patients hospitalized between March 2015 and September 2017 were recruited prospectively. AOSD patients diagnosed between 2001 and 2017 in our department were analyzed. Clinical and laboratory parameters were recorded for all patients. A multivariate analysis was performed to identify possible parameters related to the discrimination of AOSD from FUO. Results: We recruited 69 AOSD patients (51 females, 74%) and 87 patients (43 females, 49.4%) evaluated for FUO. Median ages were 45 (30-57) and 45 (30-62), respectively. Arthralgia, rash, sore throat, neutrophilia, serum ferritin level higher than 5 times of the upper limit, and elevated lactate dehydrogenase levels were associated with the likelihood of diagnosing AOSD; on the other hand, the number of daily fever peaks equal or greater than 3 was associated with the unlikelihood of diagnosing AOSD. After the clinical feasibility assessment of possible parameters derived from the multivariate analysis, in the setting of fever, two clinical (arthralgia, sore throat) and two laboratory (ferritin level, neutrophilia) parameters were selected to develop an algorithm for discrimination of AOSD and FUO. Conclusion: Presence of arthralgia, hyperferritinemia, sore throat, and neutrophilia suggests AOSD in patients presenting as FUO. This study proposes a clinician-friendly algorithm for the first time in current literature to discriminate AOSD from other causes of FUO.

WOS: 000463722100007, PubMed ID: 30489254, Objective: Familial Mediterranean fever (FMF) is the most common autoinflammatory disease. Most of the identified disease-causing mutations are located on exon 10. As the number of studies about the effect of the exonal location of the mutation and its phenotypic expression is limited, we aimed to investigate whether the exonic location of the Mediterranean fever (MEFV) mutation has an effect on the clinical manifestation in patients with FMF. Methods: Study population was derived from the main FMF registry that included 2246 patients from 15 different rheumatology clinics. We categorized the mutations according to their exon locations and retrieved the clinical and demographic information from the database. Results: Patients having the MEFV mutations on exon 2 or 10 (n: 1526) were divided into three subgroups according to the location of the MEFV mutations: Group 1 (exon 2 mutations), Group 2 (exon 10 mutations), and Group 3 (both exon 2 and exon 10 mutations). Group 2 patients were of a significantly younger age at onset, and erysipel-like erythema, arthritis, amyloidosis, and a family history of FMF were more common in this group. Conclusion: Patients with FMF and exon 10 mutations show more severe clinical symptoms and outcome. Exon 2 mutations tend to have a better outcome.

Objective: Pulmonary hypertension (PH) is a one of the major causes of death in patients with systemic sclerosis (SSc). In this study, we in- vestigated the impact of updated hemodynamic definition proposed by the 6th PH World Symposium (6th WSPH) on the frequency of PH and its subtypes in patients with SSc. Methods: Patients with SSc admitted between 2015 and 2019 and who underwent right heart catheterization (RHC) were included. The frequen- cy of PH and its subgroups based on the hemodynamic definitions of both 2015 European Society of Cardiology/European respiratory Society (ESC/ERS) PH guidelines and 6th WSPH was compared. Results: Of the 65 patients with SSc, 23 (35.4%) had normal mean pulmonary arterial pressure (mPAP), 20 (30.8%) had mildly elevated mPAP (21–24 mm Hg), and 22 (33.8%) had PH [pulmonary arterial hypertension (PAH) (n=16, 24.6%), group 2 PH (n=5, 7.7%), group 3 PH (n=1, 1.5%)] ac- cording to the 2015 ESC/ERS PH definition. Based on the updated criteria, 7 (10.8% of total cohort) additional patients were reclassified as having PH [PAH (n=3), group 2 PH (n=3), group 3 PH (n=1)]. Conclusion: The impact of the updated definition on the frequency of PH and PAH in our cohort was greater than previously reported, which may be caused by the difference in screening strategies for PAH.

OBJECTIVES: To analyse how the main components of the disease phenotype (sicca symptoms, diagnostic tests, immunological markers and systemic disease) can be driven by the age at diagnosis of primary Sjögren's syndrome (pSS). METHODS: By January 2021, the participant centres had included 12,753 patients from 25 countries that fulfilled the 2002/2016 classification criteria for pSS. The age at diagnosis was defined as the time when the attending physician confirmed fulfilment of the criteria. Patients were clustered according to age at diagnosis. 50 clusters with more than 100 observations (from 27 to 76 years) were used to study the influence of the age at diagnosis in the disease expression. RESULTS: There was a consistent increase in the frequency of oral dryness according to the age at diagnosis, with a frequency of 95% in those diagnosed at the oldest ages. The smooth curves that best fitted a linear model were the frequency of dry mouth (adjusted R2 0.87) and the frequency of abnormal oral tests (adjusted R2 0.72). Therefore, for each 1-year increase in the age at diagnosis, the frequency of dry mouth increased by 0.13%, and the frequency of abnormal oral diagnostic tests by 0.11%. There was a consistent year-by-year decrease in the frequency of all autoantibodies and immunological markers except for cryoglobulins. According to the linear models, for each 1-year increase in the age at diagnosis, the frequency of a positive result decreased by 0.57% (for anti-Ro antibodies), 0.47% (for RF) and 0.42% (for anti-La antibodies). The ESSDAI domains which showed a more consistent decrease were glandular and lymph node involvement (for each 1-year increase in the age at diagnosis, the frequency of activity decreased by 0.18%), and constitutional, cutaneous, and haematological involvements (the frequency decreased by 0.09% for each 1-year increase). In contrast, other domains showed an ascending pattern, especially pulmonary involvement (for each 1-year increase in the age at diagnosis, the frequency of activity increased by 0.22%), and peripheral nerve involvement (the frequency increased by 0.09% for each 1-year increase). CONCLUSIONS: The influence of the age at diagnosis on the key phenotypic features of pSS is strong, and should be considered critical not only for designing a personalised diagnostic approach, but also to be carefully considered when analysing the results of diagnostic tests and immunological parameters, and when internal organ involvement is suspected at diagnosis.

Background: Several societies published recommendations for latent tuberculosis infection (LTBI) screening before biological and targeted synthetic disease-modifying anti-rheumatic drugs (DMARDs) in rheumatoid arthritis (RA) but not for other inflammatory arthritides such as spondyloarthritis (SpA). Using RA guidelines could result in overestimating Tuberculin Skin Test (TST) positivity in SpA. This study aimed to compare the distribution of TST results in SpA and RA patients along with comparison in terms of QuantiFERON®-TB Gold In-Tube (QFT-GIT) test in a Bacillus Calmette-Guérin-vaccinated population.Methods: Adult RA (n=206) and SpA (n=392) patients from the TReasure database who had both TST and QFT-GIT prior to initiation of biological and targeted synthetic DMARDs were included in the study. Demographic and disease characteristics along with pre-biologic DMARD and steroid use were recorded. The distribution of TST and performance with respect to QFT-GIT were compared between RA and SpA groups.Results: Pre-biologic conventional DMARD and steroid use was higher in the RA group. TST positivity rates were 44.2% in RA and 69.1% in SpA for a 5 mm cut-off (pConclusions: TST positivity was more pronounced in SpA compared to that in RA and this was not explainable by pre-biologic DMARD and steroid use. The agreement of TST with QFT-GIT was poor in both groups. Using a 5 mm TST cut-off for both diseases could result in overestimating LTBI in SpA.

Background/aim: Peritonitis attacks of Familial Mediterranean Fever (FMF) usually requires emergency medical admissions and it's hard to distinguish a typical abdominal attack from surgical causes of acute abdomen. Therefore, history of abdominal surgery, particularly appendectomy, is very common in patients with FMF. However, history of appendectomy might also give some clues about the course of FMF in the adulthood. This study was to determine whether the history of appendectomy help to anticipate disease course of FMF in the adulthood. Materials and methods: All patients recruited from FMF in Central Anatolia (FiCA) cohort, comprising 971 adult subjects. All patients fulfilled the Tel Hashomer criteria. Demographic data, FMF disease characteristics, co-morbid conditions, past medical history, surgical history and disease complications were meticulously questioned and laboratory features and genotype data (if available) were recruited from patient files. Results: Appendectomy history was evident in 240 (24.7%) subjects. Disease onset was earlier and peritonitis is strikingly more prevalent (97.1% vs. 89.6%, p < 0.001) in appendectomized patients. These patients had reported almost two fold more frequent attacks in the last year compared to appendix intact patients (median 3.5 vs. 2 attacks, p = 0.001) without a difference in frequency of musculoskeletal and skin attacks. Severe disease was more common (10% vs. 5.9%, p = 0.038) due to involvement of more attack sites throughout the life and more frequent attacks. Appendectomy patients had used higher daily doses of colchicine to control disease (1.43 +/- 0.6 mg vs. 1.27 +/- 0.52 mg, p = 0.002) but colchicine resistance was also more common in these patients, 15% vs. 6.7% respectively, p < 0.001. Conclusion: Appendectomy history is common in FMF patients and associated with frequent serositis attacks in adulthood. These patients require higher colchicine doses with a lower rate of response and more need for Interleukin-1 antagonist therapies.

Spondyloarthritis (SpA) is a group of diseases with overlapping skeletal and extra-articular features. Acute anterior uveitis (AAU) is the most common extra-articular manifestation of SpA. The relation between AAU and SpA is well defined in the current literature. Our study aims to analyze the frequency and factors associated with AAU in different forms of SpA in a large nationwide cohort of Turkish SpA patients.Retrospective cohort study.The data were obtained from the TReasure database, which compiles data from records of the web-based Rheumatoid Arthritis (RA) and SpA patients treated with biological disease-modifying anti-rheumatismal drugs from different regions of Turkey. The clinical characteristics of SpA and uveitis are recorded.Data of the 4,297 SpA patients were included in the study. Overall, 475 of 4,297 patients (11.0%) had experienced 1 or more episodes of uveitis. SpA patients with older age (P.001), a smoking history (P = .004), delayed diagnosis (P = .001), longer disease duration (P.001), arthritis (P.001), positive HLA-B27 (P.001), a family history of SpA (P.001), and radiographic damage (presence of sacroiliitis, syndesmophytes, bamboo spine, hip involvement) (P.001 for all) more commonly had uveitis. On the other hand, uveitis was less prevalent in patients with psoriasis and psoriatic arthritis (P.001 for both).Uveitis may be the key feature leading to SpA diagnosis. Patients with radiographic damage and long disease duration have an increased risk for uveitis in both male and female SpA patients. Patients with uveitis should be referred to a rheumatologist for a thorough evaluation of SpA.

The Turkish population is vaccinated with Bacille Calmette-Guérin (BCG), and the BCG vaccination decreases the specificity of the tuberculin skin test (TST). The purpose of this study was to investigate the incidence of active tuberculosis (TBC) among rheumatic patients who were screened only with the QuantiFERON®-TB Gold In-Tube (QFT-GIT) test for latent TBC prior to biological treatment.The Hacettepe University Biological Database (HUR-BIO) was used for latent TBC assessment. Consecutive patients were evaluated from July 2015 to October 2016 by a questionnaire that included the patients' demographic characteristics, treatment history, and symptoms of active TBC. A total of 664 patients were interviewed by physicians. TBC statuses of the 671 non-interviewed patients were checked from the Turkish National Tuberculosis Registry records. Mean TBC incidence per year was calculated for anti-tumor necrosis factor-alpha (TNF-α) agents.A total of 1335 (58.2% female) patients with the mean age of 44.2 ± 12.9 years were included. Of the patients, 836 (62.6%) had spondyloarthropathy, 432 (32.4%) had rheumatoid arthritis, and 67 (5%) had other rheumatologic diseases. The total biological drug exposure was 2292 patient-years (2043 patient-years for anti-TNF-α, 249 patient-years for non-TNF-α inhibitors). Positive and indeterminate QFT-GIT results were found in 258 (19.3%) and 23 (1.7%) patients, respectively. The median follow-up time after the onset of biological agent was 19.4 months (IQR = 29.5). Pulmonary TBC was found in 3 (0.2%) of the 1335 patients. The annual incidence of TBC was 147/100,000 patient-years for all TNF-α inhibitors (249/100,000 and 123/100,000 patient-years for QFT-GIT-positive and negative patients, respectively).TBC incidence increased by nearly seven times the Turkish national TBC incidence. The QFT-GIT Test appears acceptable to determine latent TBC before biological agent use. Consequently, the QFT-GIT Test can be appropriately used in BCG-vaccinated countries. Key Points • Our study contributes to filling the gap in the literature by reflecting real-life data about TBC frequency after QFT-GIT use in patients receiving biological agents. • The frequency of active TBC will remain within acceptable limits when only QFT-GIT is used in the screening of latent TBC prior to the use of biological agents in a population where the majority are vaccinated with BCG. • Using the QFT-GIT alone for latent TBC screening prior to biologic treatment in countries with high BCG vaccination rates reduces the number of patients needing isoniazid (INH) treatment.

Background:Takayasu’s arteritis (TA) is commonly seen in women of childbearing ages; therefore, reproductive health of TA patients is an important issue.Objectives:We aimed to evaluate the fertility and pregnancy (PG) outcomes of TA patients before and after diagnosis.Methods:In the prospective database of the Hacettepe University Vasculitis Research Centre (HUVAC), 202 TA patients (female =184) meeting the 1990 ACR criteria were registered by the end of February 2020. 120 patients who could be reached out gynaecological records and marriage status, were included in the study. We identified 233 PG in 82 of included these 120 TA patients (Figure 1). Demographic and clinical features, comorbidities, distribution of vascular involvement, obstetrical histories and outcomes were retrospectively evaluated. Patients were classified according to a novel proposed disease clusters (C1: Abdominal Predominant, C2: Aortic Arch Predominant, C3: Focal Disease) using the defined decision tree.Results:12 of 96 (12.5%) married TA patients had infertility being defined as not being able to get pregnant after one year (or longer) of unprotected sex. 20 (16.7%) women had menopause before the age of 45, being defined as early menopause (EM). Compared to normal population, infertility (12.5% vs. 8.1%) and EM ratios (16.7% vs. 7.6%) seem to be increased in TA patients. (1, 2)200 PG had occurred before TA diagnosis (bTA) in 71 women, and 33 PG were observed after TA diagnosis (aTA) diagnosis in 19 women (two patients diagnosed during PG). According to novel disease subsets, aTA patients classified into C1 (n=3; 15.7%), C2 (n=9; 47.3%) and C3 (n=6; 31.5%). One patient could not be classified.GHT was seen in 4 (12.1%) patients being the most frequent maternal complication in aTA group. Most common fetal complications were prematurity, IUGR and LBW in both groups (Table 1). Even fetal complications were more frequent in aTA group, it was not statistically significant [11 (33.3%) vs. 43 (21.5%), p=0.18 respectively]. However, maternal complications were significantly more common in aTA group [22 (11.0%) vs. 8 (24.2%), p=0.048]. There was no difference for the obstetrical outcomes in terms of novel TA classification.Conclusion:This study showed increased infertility and EM ratios in TA patients. PG of aTA had more complications in terms of maternal complications. Fetal complications were more frequent in PG of aTA but not statistically significant. Larger cohort data is required.References:[1]doi:10.1017/s0021932017000244.[2]doi:10.1016/j.maturitas.2019.03.008.PregnanciespBefore TA (n=200)After TA (n=33)Number of Patients7119NPAge at TA diagnosis, years mean (SD)38.2 (13.1)24.0 (6.6)Age at first pregnancy21.7 (5.0)27.6 (5.0)Comorbidities/CV Risk factors n(%)n (%)p- Smoking22 (31)3 (15.8)>0.05- Dyslipidemia14 (19.7)1 (5.3)- Hypertension29 (40.8)9 (47.4)- Diabetes mellitus8 (11.3)0 (0)Maternal Complicationsn (%)Numbers of pregnancies with any maternal complications22 (11.0)8 (24.2)0.048- Gestational hypertension6 (3.0)4 (12,1)NP- PROM6 (3.0)1 (3.0)- GDM0 (0)0 (0)- Bleeding (Antepartum/postpatum)6 (3.0)2 (6.1)- Preeclampsia0 (0.0)0 (0.0)- İnfection5 (2.5)1 (3.0)- Placenta previa0 (0)1 (3.0)Fetal Complicationsn (%)pNumbers of pregnancies with any fetal complications43 (21.5)11 (33.3)0.180- LBW16 (8.0)6 (18.2)NP- IUGR20 (1.0)5 (15.2)- Preterm birth13 (6.5)7 (21.2)- CNS complications2 (1.0)1 (3.0)- Cardiovascular complications3 (1.5)0 (0)- RDS_BPD4 (2.0)0 (0)- NICU admission12 (6.0)1 (3.0)- Other5 (2.5)0 (0)- Neonatal death5 (2.5)0 (0)- Retinopathy of prematurity1 (0.5)0 (0)- Stillbirth7 (3.5)0 (0)Deliveryn (%)PVaginal delivery128 (64)6 (18.2)Cesarean24 (12)13 (39.4)Spontaneous abortus21 (10.5)7 (21.2)0.088Termination of pregnancy18 (9.0)7 (21.2)0.061Abbreviations: BPD: Bronchopulmonary dysplasia, CNS: Central nervous system, GDM: Gestational diabetes mellitus, IUGR: Intrauterin growth restriction, LBW: Low birth weight, NICU: Neonatal Intensive Care Unit, NP: Not performed PROM: Premature rupture of membranes, RDS: Respiratory distress syndromeDisclosure of Interests:None declared

The temporal relationship between the onset of skin psoriasis (Pso) and psoriatic arthritis (PsA) is a long and well-known feature of psoriatic disease. Psoriatic skin lesions emerge years before the development of musculoskeletal manifestations in nearly 75% of PsA patients. While skin and joint manifestations develop within one year in 15-20% of the patients, joint manifestations precede the skin involvement by a year or more in about 10% of the patients (1).

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., Union Chimique Belge (UCB); Scientific and Technological Research Council of Turkey (TUBITAK)Turkiye Bilimsel ve Teknolojik Arastirma Kurumu (TUBITAK), This work was supported by the Union Chimique Belge (UCB) (to Dilek Solmaz for axial spondyloarthritis fellowship) and the Scientific and Technological Research Council of Turkey (TUBITAK) (to Sibel Bakirci).

The COVID-19 pandemic raises many alarms in the rheumatological era. Gianfrancesco et al reported the answer of an important question: the characteristics associated with hospitalization for COVID-19 in people with rheumatic disease.1 The data suggested that patients with rheumatic disease on prednisone dose of ≥10 mg/day were associated with higher odds of hospitalization, and vasculitis was the fourth common rheumatic disease among all of these patients. Another essential concept is to be careful in the differential diagnosis of the patients presenting with symptoms and signs of COVID-19. Physicians should keep in mind the other infectious and inflammatory diseases during diagnostic procedures of these critical patients. Inevitably, COVID-19 ranks first in differential diagnosis of all patients with respiratory symptoms and signs in current pandemic days. The standard of reference for confirming COVID-19 relies on microbiological tests, such as real-time reverse transcription polymerase chain reaction (RT-PCR).2 A systematic review of the accuracy of COVID-19 tests reported false-negative rates up to 29% (equating to a sensitivity of 71–98%), based on negative RT-PCR tests which could turn out positive on repeated testing.3 Chest CT can be used as an auxiliary to RT-PCR for diagnosing COVID-19 pneumonia in the current pandemic context.4 The main CT feature of COVID-19 pneumonia is the bilateral patchy ground-glass opacities (GGOs) with peripheral predominance.5 On the other hand, GGO has many causes and one of these is eosinophilic granulomatosis with polyangiitis (EGPA). Herein, we presented two patients who have been hospitalized with preliminary diagnosis of COVID-19 but diagnosed as EGPA in hospitalisation period for COVID-19. A male patient in his 20s was admitted to the emergency department with complaints of shortness of breath, cough and sputum. In the medical history of the patient, he had asthma for 3 years but he did not receive any asthma …

Background/aim Takayasu’s arteritis (TA) is a rare, large-vessel vasculitis of unknown etiology, affecting aortic arch, and its main branches. Noninvasive imaging methods are frequently used in diagnosis and follow-up in Takayasu’s arteritis. Studies investigating optimal timing of follow up imaging are rare. This study is aimed to investigate the radiologic changes in vascular involvements of Takayasu’s arteritis patients one year after diagnosis. Materials and methods Database of our Vasculitis Center was analyzed retrospectively and 97 patients were included into the study. Demographic, clinical, radiological, and therapeutic findings of patients were recorded. Patients with follow-up imaging after approximately one year of diagnosis were recruited into further analysis. Radiological changes and the effect of different immunosuppressive agents on vascular involvements were investigated. Results Mean age and disease duration of patients were 43.0 and 9.0 years. The most commonly used imaging methods/modalities for the diagnosis of TA were computer tomography-angiography (CT-Ang) (58.8%), magnetic resonance-angiography (MR-Ang) (29.9%), and doppler ultrasonography (11.3%). Subclavian and common carotid arteries were the most frequently involved vessels. Fifty-three patients underwent follow-up imaging after one year of diagnosis and, in 64% of patients, same imaging method had been used. MR- Ang (62.3%) and CT-Ang (35.9%) were the most preferred follow-up imaging studies. Sixty-eight percent of patients had stable vascular involvement, 28% had progression, and 4% had regression. No difference was found in radiological changes regarding patients with usage of different immunosuppressive agents (P = 0.634). There was no association between the change in serum acute phase reactants and radiological disease activity. Conclusion The most commonly used imaging modality for the diagnosis of TA was CT-Ang, whereas MR-Ang was the most preferred for follow-up. Almost 30% of TA patients in our Vasculitis Center had progression at around one year concordant with previous literature. A follow-up imaging at around one year of treatment seems feasible in management of TA.

Most patients with inflammatory arthritis are at their reproductive ages. Use of anti-tumour necrosis factor alpha (anti-TNF-α) agents, one of the important treatment options for inflammatory arthritis, can cause foetal morbidity and mortality. While most studies on the effects of anti-TNF-α agents on pregnancy outcomes are about maternal exposure, the number of studies on the risks related to paternal exposure is insufficient. This study aimed to assess pregnancy periods and outcomes of the partners of male ankylosing spondylitis (AS) patients receiving anti-TNF-α treatment during the preconception period. Totally, 163 male AS patients using anti-TNF-α agents were identified from the Hacettepe University Biological Registry. Of these patients, 45 (27.6%) who declared that their partners got pregnant after initiation on anti-TNF-α agents were included. Data regarding demographics and drug exposure and pregnancy and infant outcomes were evaluated. Of 45 pregnancies, 39 (86.7%) resulted in healthy live births, 3 (6.7%) resulted in spontaneous abortion, and 3 (6.7%) were terminated with curettage. Of 39 live births, 34 (87.2%) were term and 5 (12.8%) were preterm, 30 (76.9%) had normal birth weight, 6 (15.4%) had low birth weight, and 3 (7.7%) had fetal macrosomia. No congenital malformations related to paternal exposure were observed. This study is valuable as being one of the studies providing pregnancy outcomes of partners of male AS patients receiving anti-TNF-α agents with its relatively high number of patients. The results suggested that paternal exposure to anti-TNF-α agents during preconception period could be safe on pregnancy outcomes.

Defining predictors of damage would improve patient care. We applied damage indexes to patients with familial Mediterranean fever (FMF) and identified the predictors of damage.This is a cross-sectional analysis of 926 FMF patients, who fulfilled the Tel-Hashomer criteria and had at least six months of follow-up. Patients were stratified according to their damage status (damage vs. no damage) defined with autoinflammatory disease damage index (ADDI) and modified ADDI (excluding musculoskeletal pain). We used logistic regression analysis to investigate independent predictors of damage for both indexes.Mean disease duration was 21.6±11.9 years. 527 patients (57%) had damage according to ADDI. Median ADDI score was 1 (0-11). Most common FMF-related damages were observed in musculoskeletal, reproductive and kidney domains. Female gender, inflammatory comorbidity, colchicine resistance, colchicine nonadherence, musculoskeletal attack dominance, diagnostic delay, follow-up time, and smoking history remained independent predictors of damage according to ADDI score. The rate of patients with damage defined by modified ADDI was only to 23%. M694V/M694V homozygosity, female gender, musculoskeletal attack dominance, colchicine resistance, persistent inflammation, follow up time and family history of amyloidosis were found to be predictors of damage according to modified ADDI score.Our study is the first to apply comprehensive damage indexes to FMF patients and identified predictors of damage. Factors linked to a severe FMF phenotype, including M694V homozygosity and persistent inflammation, were associated with only modified ADDI. Our findings justify the concerns about musculoskeletal pain and might point to the need for re-evaluation of ADDI for FMF patients.

Introduction/objectives Familial Mediterranean fever (FMF) is characterized by recurrent attacks of fever, serositis, and arthritis, but some patients may experience long-term complications of disease such as infertility/subfertility. The published data about FMF-associated infertility is still limited. The aim of this study is to investigate the frequency and to determine potential factors for FMF-associated infertility/subfertility. Methods We enrolled 971 adult patients with FMF. We defined infertility as the failure to conceive after 12 months of regular, unprotected intercourse. All patients fulfilled Tel Hashomer criteria. Demographic data, FMF disease characteristics and genotype data (if available), disease complications, laboratory parameters, and treatment features were recorded. Results There were 582 subjects eligible for the present study (mean age 41.05 +/- 10.6 years, 65.8% female). MEFV mutations were available in 482 subjects, and 74.9% of subjects were harboring M694 V mutation (25.1% homozygous for M694 V). Infertility was present in 64 patients (14.6% of females and 4% of males). Multivariate analysis showed female sex [odds ratio (OR), 4.47; 95% confidence interval (CI95%) 1.75-11.42; p = 0.002], FMF disease onset < 20 years [OR, 2.99; (CI95% 1.04-8.61);p = 0.04], disease severity (ISSF) [OR, 4.81; (CI95% 2.28-10.17); p < 0.001], and colchicine nonresponse [OR, 2.80; (CI95% 1.17-6.74) p = 0.021] were the independent predictors of infertility. We also observed reversal of infertility in five patients who were treated with IL-1 beta antagonists. Conclusion Disease severity, FMF disease onset < 20 years, colchicine nonresponse, and female sex were found to be the independent predictors of infertility. The value of effective therapeutic interventions must be determined to treat infertility in these patients.center dot The prevalence of infertility increased in female patients with FMF.center dot Female sex, FMF disease onset < 20 years, disease severity, and colchicine nonresponse were risk factors for FMF-associated infertility.center dot With effective treatment of FMF, reversal of infertility was observed in five patients.

Yildiz, Fatih/0000-0003-3628-8870; Cinar, Muhammet/0000-0002-6150-3539 WOS: 000537230800003 PubMed ID: 31922480 Objective: Our aim is to test the validity of the Psoriasis Symptom Inventory (PSI), a patient-reported outcome, to assess the psoriasis severity within the scope of rheumatology. Methods: Within the PsA international database (PSART-ID), 571 patients had PSI, while 322 of these also showed body surface area (BSA). Correlations between PSI, BSA, and other patient- and physician-reported outcomes were investigated. Results: There was a good correlation between PSI and BSA (r=0.546, p

Background/aim: Tumour necrosis factor inhibitors and anti-interleukin-6 (anti-IL-6) therapies are increasingly being used in Takayasu’s arteritis (TA) patients who are unresponsive to corticosteroids ± conventional immunosuppressive agents. The aim of this study is to assess the efficacy and safety of anti-IL-6 (tocilizumab) therapy in refractory TA patients in real life. Materials and methods: Fifteen TA patients (86.7% were female) who received at least 3 cycles of tocilizumab therapy were retrospectively assessed by clinical, laboratory, and radiological evaluations before and after tocilizumab therapy. Results: The median (min–max) age of the patients at evaluation was 35 (20–58) years and the median disease duration from diagnosis was 24 (12–168) months. The median (min.–max.) duration of follow-up after tocilizumab was 15 (3–42) months. There was a significant decrease in erythrocyte sedimentation rate (ESR), C-reactive protein (CRP), and patient global visual analogue scale (VAS) scores of patients after tocilizumab therapy. The median (min.–max.) ESR was 26 (5–119) vs. 3 (2–49) mm/h, P = 0.02; CRP was 39.8 (2.4–149.0) vs. 7.9 (0–92.9) mg/L, P = 0.017; and patient global VAS was 50 (0–90) vs. 30 (0–60), P = 0.027, respectively. In 8 patients, ESR and CRP levels were in the normal range in the last control. Imaging modality results after tocilizumab were available for 9 patients; 8 patients were radiologically stable and regression was seen in 1 patient. Comparable imaging modality results before and after tocilizumab were available for 5 patients; 4 patients were radiologically stable and regression was seen in 1 patient. Radiological findings were consistent with laboratory responses. Glucocorticoid dosages decreased from a mean dosage of 16.2 (9.1) mg/day at baseline to 7.1 (3.8) mg/day (P = 0.001) at the last follow-up visit. There was no increase in the steroid dosage in any of the patients. All patients tolerated tocilizumab well. Conclusion: Based on retrospective real life data, anti-IL-6 (tocilizumab) appears to be an effective and tolerable treatment option in refractory TA patients.

WOS: 000505281300007, PubMed: 30680951, 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., Turkish Society for Rheumatology; Scientific and Technological Research Council of TurkeyTurkiye Bilimsel ve Teknolojik Arastirma Kurumu (TUBITAK), Supported by the Turkish Society for Rheumatology. Dr. Bakirci's work was supported by the Scientific and Technological Research Council of Turkey and the Turkish Society for Rheumatology.

Objectives: Behcet’s disease (BD) is a multi-systemic inflammatory disorder which can affect all types and sizes of vessels.The usage of TNF-α antagonists is increasing in different involvements of...

Aim To determine the frequency and define the causes of pulmonary hypertension (PH) in patients with Takayasu arteritis (TA). Method Sixty-four TA patients were evaluated by transthoracic echocardiography (TTE). Having an estimated systolic pulmonary arterial pressure (sPAP) ≥40 mm Hg by echocardiography or if performed, mean PAP ≥25 mm Hg in right heart catheterization was defined as PH. Clinical, imaging and laboratory results of the TA patients were obtained from hospital files. Result In total, seven (10.9%) patients had PH. Four patients had PH due to left-sided heart disease (group 2 PH), three patients due to pulmonary arterial involvement (PAI; group 4 PH) and one patient due to atrial septal defect (group 1 PH). In one patient, combination of PAI, aortic insufficiency and pulmonary venous return anomaly was present and he was considered to have both group 2 and group 4 PH. PAI was more frequent (42.9% vs 15.7%) in patients with PH but the difference was not statistically significant. The percentage of patients treated with cyclophosphamide and/or biologics was higher in the group with PH as compared to the group without PH (P = 0.015). One patient with group 4 PH had been on pulmonary arterial hypertension (PAH)-specific agents for 8 years. Conclusion Pulmonary hypertension is not infrequent in TA patients and all the potential causes of PH should be carefully evaluated. Patients with severe or treatment-resistant disease are prone to have PH. PAH-specific agents may be effective in patients with group 4 PH.