Assessment and comparison of the 2023 ACR/EULAR APS criteria with the revised Sapporo criteria.

OBJECTIVE: To analyze antiphospholipid antibody (aPL)-positive patients using the 2023 American College of Rheumatology/The European Alliance of Associations for Rheumatology (ACR/EULAR) antiphospholipid syndrome (APS) classification criteria and compare the revised Sapporo criteria and the 2023 ACR/EULAR criteria and evaluate whether the 2023 ACR/EULAR criteria provide added value over the revised Sapporo criteria. METHODS: In this descriptive study, 94 aPL-positive patients (with or without APS diagnosis) were identified from two hospital-based registries (Gazi and Hacettepe University). Patients were classified into four groups to compare both criteria sets. These four groups are as follows: (1) patients classified with only the revised Sapporo criteria; (2) patients classified with only the 2023 ACR/EULAR APS criteria; (3) patients classified with both two criteria sets; and (4) patients classified with neither two criteria set. RESULTS: Of the 94 patients, 11 were classified with only the revised Sapporo criteria; one with only the 2023 ACR/EULAR APS criteria; 52 with both criteria sets; and 30 with neither set of criteria. For these 94 patients, the operating characteristics of the 2023 ACR/EULAR APS criteria, using the revised Sapporo criteria as the gold standard, the 2023 ACR/EULAR APS entry criteria demonstrated 100% sensitivity, and the 2023 ACR/EULAR APS classification criteria demonstrated 98% specificity and 82.5% sensitivity. CONCLUSION: The study emphasizes the importance of recognizing differences in clinical manifestations, such as early pregnancy loss without severe preeclampsia (PEC) and/or severe placental insufficiency (PI) and calls for a nuanced discussion on anticardiolipin (aCL) and anti-beta 2-glycoprotein-I (anti-ß2GPI) immunoglobulin G (IgG) cutoff values.

The validity, reliability, and responsiveness of a new biopscyhosocial questionnaire for patients with systemic lupus erythematosus.

OBJECTIVE: In recent years, the importance of holistic care in individuals with systemic lupus erythematosus (SLE) has been emphasized, and therefore a measurement tool that evaluates biopsychosocial impact is needed. This study was conducted to determine the validity, reliability, and responsiveness of the Cognitive Exercise Therapy Approach-Biopsychosocial Questionnaire (BETY-BQ) in individuals with SLE. METHODS: Lupus Quality of Life (LupusQoL), Short Form-36 (SF-36), Health Assessment Questionnaire (HAQ), and Hospital Anxiety and Depression Scale (HADS) were used for the validity. For reliability, evaluations were repeated at 1 -week intervals and for responsiveness, the same scales were re-administered at 3-month intervals to 30 patients. RESULTS: BETY-BQ showed moderate to high correlations with LupusQoL subparameters (rho = -0.522 to -0.718, p < 0.001), moderate to very high correlations with SF-36 subparameters (r = -0.826 to rho = 0.594, p < 0.001), high correlations with HAQ and HADS-D (rho = 0.735, p < 0.001; rho = 0.622, p < 0.001, respectively) and a moderate correlation with HADS-A (rho = 0.571, p < 0.001). In the test-retest method the correlation between the responses was very high (r = 0.989, p < 0.001). The intraclass correlation coefficient (r = 0.944, p < 0.001) and Cronbach's alpha value were found to be excellent (0.952). BETY-BQ showed moderate correlations with LupusQoL subparameters (the body image r = -0.443, p = 0.014; planning rho = -0.529, p = 0.003; fatigue r = -0.484, p = 0.007; intimate relationships rho = -0.421, p = 0.02), SF-36 social function subparameter (rho = -0.427, p = 0.019) and anxiety and depression subparameter of HADS (rho = 0.418, p = 0.021; r = 0.507, p = 0.004, respectively). CONCLUSION: BETY-BQ which was found as a valid, reliable, and responsive scale in individuals with SLE, was presented to healthcare professionals for use in clinical and research studies as a scale that can be preferred in the evaluation of biopsychosocial status of individuals with SLE.

Transitioning from pediatric to adult-oriented healthcare in rheumatology: the pediatric side of the coin.

OBJECTIVES: The transition of adolescents and young adults (AYAs) from pediatric to adult-oriented healthcare may be affected by many factors, including the personal and cultural settings. We aimed to analyze the transition readiness and the factors affecting the transition success in rheumatology. METHODS: Patients older than 12 years were included in this prospective study. All filled out the Transition Readiness Assessment Questionnaire (TRAQ) 5.0. AYAs were phone-interviewed after their transfer to adult-oriented healthcare. Drug adherence was evaluated with 4-item Morisky Medication Adherence Scale (MMAS-4). AYAs rated their transitional care experience with visual analogue scale (VAS 0-10; 0, the worst; 10, the best). RESULTS: A total of 504 TRAQs were filled out by 406 patients (F/M = 1.5). The total TRAQ score was positively correlated with age and higher in the forms filled out by girls than boys (4.2 vs 4.0, respectively; p= 0.005). The transition was successful for 78 (83.9%) out of 93 patients transferred to adult-oriented healthcare. The VAS for the transition process was lower and the post-transfer MMAS-4 score was worse (8 vs 9, p= 0.030 and 3 vs 4, p= 0.020; respectively) in patients whose transition was not successful when compared with the successfully-transitioned ones. The best-performing TRAQ cut-off value was >4.0 for predicting transfer readiness in rheumatology. CONCLUSION: A TRAQ score of > 4 could be used while deciding about the transfer readiness of AYAs in rheumatology. Improving the AYAs' experience of the transition process and closely monitoring medication adherence during transition are essential for a successful transition.

Hematologic Malignancy Risk in Inflammatory Arthritis Patients Treated with TNF Inhibitors: The Real-Life Data from the HUR-BIO Registry.

INTRODUCTION: This study aimed to assess the incidence of hematologic malignancy (HM) among inflammatory arthritis (IA) patients receiving tumor necrosis factor inhibitors (TNFi) compared with the general Turkish population. METHODS: HUR-BIO (Hacettepe University Rheumatology Biologic Registry) is a single-center biological disease-modifying anti-rheumatic drug (bDMARD) registry since 2005. Patients with IA, including rheumatoid arthritis, spondyloarthritis, or psoriatic arthritis who had at least one visit after the TNFi were screened from 2005 to November 2021. Standardized incidence rates (SIR) were calculated after adjustment for age and gender and compared with the 2017 Turkish National Cancer Registry (TNCR). RESULTS: Of the 6139 patients registered in the HUR-BIO, 5355 used any TNFi at least once. The median follow-up duration was 2.6 years for patients receiving TNFi. Thirteen patients developed a HM on follow-up. In these patients, the median age at the IA onset was 38 (range, 26-67), and the median age at the HM diagnosis was 55.5 (range, 38-76). Patients using TNFi had an increased HM incidence (SIR 4.23, 95% confidence interval (CI) 2.35-7.05). Ten patients with HM were under 65 years of age. In this group, there was a higher incidence of HM in both men (SIR 5.15, 95% CI 1.88-11.43) and women (SIR 4.76, 95% CI 1.74-10.55). CONCLUSIONS: The risk of HMs in inflammatory arthritis patients receiving TNFi was four times higher than in the general Turkish population.

Fertility, early menopause and pregnancy outcomes of patients with Takayasu's arteritis.

OBJECTIVES: The reproductive health of patients with Takayasu's arteritis (TA) is an important issue. Systemic inflammation and treatments used in TA may affect female reproductive organs, leading to infertility, maternal and fetal morbidity, and early menopause. This study aimed to evaluate fertility, early menopause and pregnancy outcomes of patients before and after TA diagnosis. In addition, pregnancy morbidities following TA diagnosis were analysed for each cluster. STUDY DESIGN: Two hundred and two patients with TA (184 females) who met the 1990 American College of Rheumatology criteria were registered in the prospective database of Hacettepe University Vasculitis Research Centre by the end of February 2020. Demographic and clinical features, comorbidities, distribution of vascular involvement, obstetric histories and outcomes were evaluated retrospectively. Patients with TA were classified according to novel proposed disease clusters. Early menopause was defined as menopause before 45 years of age. RESULTS: One hundred and twenty-one female patients with TA, for whom gynaecological records and marriage status could be obtained, were included in the study. Ninety-seven patients were married, of whom 12 (12.5 %) patients were infertile. In total, there were 238 pregnancies in 83 female patients with TA: 203 before TA diagnosis, 35 after TA diagnosis, and two patients were diagnosed during pregnancy. Compared with the pre-diagnosis group, maternal complications were significantly more common in the post-diagnosis group [23 (11.3 %) vs 9 (25.7 %); p = 0.048]. The most common maternal complication was gestational hypertension (12.1 %). Fetal complications were common in both groups (21.5 % pre-diagnosis vs 34.2 % post-diagnosis; p = 0.18), and included prematurity, intrauterine growth retardation and low birth weight. According to novel disease subsets, post-diagnosis patients were classified as C1 (n = 3, 15.7 %), C2 (n = 9, 47.3 %) or C3 (n = 6, 31.5 %). One patient could not be classified. There was no difference in obstetric outcomes between these subgroups. Early menopause was observed in 20 (16.5 %) of 121 patients, but some patients had not yet reached 45 years of age. CONCLUSION: Infertility was higher in patients with TA in comparison with the general population in Turkey (12.5% vs 8.6%), and the early menopause rate was lower in patients with TA (16.7% vs 36.1%). Pregnancies following a diagnosis of TA had more maternal complications than pre-diagnosis pregnancies. Fetal complications were more common both pre- and post-diagnosis. Chronic inflammation before TA diagnosis may lead to increased infertility and fetal complications.

Association between oxygen delivery and digital ulcers in systemic sclerosis.

OBJECTIVE: 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. METHODS: 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). RESULTS: 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/m2 vs 549 mL/min/m2, p = 0.03). There was a strong positive correlation between cardiac output and DO2 calculated by using the index-SpO2 (r = 0.903; p < 0.001). Of the SSc patients, 46 (41.4 %) had DUs within the last 12 months. Patients with DUs had higher mean mRSS, lover mean FVC and more frequently diffuse disease, interstitial lung disease, anti-SCL70 antibody positivity (p < 0.05 for all). No difference was observed in DO2 among DU positive or DU negative groups by any calculation (p > 0.05 for all). CONCLUSIONS: DO2 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.

Drug retention rate and predictive factors of drug survival for secukinumab in radiographic axial spondyloarthritis.

The drug retention rate has been an important indicator for evaluating the treatment tolerance. Unfortunately, our knowledge of the secukinumab retention rate in radiographic axial spondyloarthritis (r-AxSpA) is limited. The objective of this study was to assess the retention rate of secukinumab and evaluate predictive factors of drug survival based on data from a real-life cohort. We retrospectively assessed 147 r-AxSpA patients between May 2018 and January 2020 from the HUR-BIO database. Secukinumab retention rates were analyzed using the Kaplan-Meier method and Cox proportional hazard model was used for predictors factors. The global retention rate of secukinumab was 55% at 12 months. r-AxSpA patients with obesity had a lower frequency of secukinumab discontinuation (29% vs. 50%, p = 0.013) in comparison r-AxSpA patients without obesity. In multivariate analysis, multiple TNFi usage had a higher risk of secukinumab discontinuation [HR 1.99 (1.09-3.62), p = 0.024]; on the other hand, obesity had a lower risk [HR 0.45 (0.27-0.90), p = 0.008]. Except for not using multiple TNFi, this real-life analysis showed for the first time that obesity is not an adverse risk factor for secukinumab drug retention in r-AxSpA. Secukinumab, which is an interleukin-17A inhibitor, could act via a different pathway than tumor necrosis factor inhibitors (TNFi). The identification of predictive factors such as obesity that may affect the individual drug selection may provide more appropriate biologic treatment strategies for r-AxSpA.

Anxiety levels before biologic initiation and changes with treatment in patients with psoriatic arthritis: HUR-BIO biologic registry results.

OBJECTIVES: Psoriatic arthritis (PsA) is an inflammatory musculoskeletal disease related to several comorbidities. Anxiety is an important comorbidity in PsA and the data is scarce. We aimed to understand the rates before biologic agents and change in anxiety with the treatment. METHODS: PsA patients from the Hacettepe University biologic database (HUR-BIO) were assessed for the high anxiety level (score ≥ 4) using the patient self-reported measure of anxiety on a 0-10 numerical scale, included in the Psoriatic Arthritis Impact of Disease questionnaire (PSAID-12). The rate and scores of anxiety were determined before starting biologic agents, at the first visit within 6 months. Changes in anxiety scores were assessed according to favorable treatment responses, and the correlation was evaluated by Spearman correlation analysis. RESULTS: From 520 patients registered, 147 [mean (SD) age 43.3 (12.4) years, 70.7% female] had anxiety score both at baseline and first visit within 6 months. Both the frequency of high anxiety level and mean (SD) scores decreased at the first visit [63.9% vs. 41.4%, 4.8 (3.4) vs. 3.2 (3.1) respectively, p < 0.001 for both] after a mean (SD) follow-up of 105.7 (22.2) days. There was also a positive correlation between the change in anxiety scores and all parameters tested for treatment response: pain, PGA, BASDAI, HAQ-DI, DAS-28, and also PsAID-12. CONCLUSION: Anxiety is a more frequent problem at biologic initiation than rates observed in the general PsA population. The rates show a decreasing trend and correlates with treatment response but is still high within 6 months under treatment. KEY POINTS: • As high as 65% of patients had a high anxiety levels before the initiation of bDMARDs. • The disease activity control is essential in reducing anxiety; however, rates are still high within 6 months. • Decreased anxiety scores and rates of the high anxiety level are linked to better outcomes.

The clinical features and treatment of eosinophilic granulomatosis with polyangiitis (EGPA) in Turkey: one or two distinct diseases?

Eosinophilic granulomatosis with polyangiitis (EGPA) is defined the disease as having two subgroups, ANCA (+) and ANCA (-). We aimed to compare EGPA subgroups in terms of clinical features, outcomes, and treatments. A multidisciplinary team was established under our vasculitis centre since October 2014. Totally 50 EGPA patients were enrolled. Clinical features, treatments, and outcomes (FFS, VDI, relapse) were reviewed. For relapse-free survival analysis, time to first relapse was compared according to ANCA phenotype by Kaplan-Meier survival analysis and log-rank test. 17 (34%) patients were in ANCA (+), 33 (66%) patients were in ANCA (-) group. ANCA (-) patients were significantly younger at the diagnosis time (37.9 ± 14.3 vs 53.8 ± 16.3; p = 0.001) and had more nasal polyposis (45.5% vs 11.8%; p = 0.017). ANCA (+) patients had higher BVAS (17[13] vs 9[4]; p = 0.002), renal involvement and peripheral neuropathy were more common in this group, while cardiac involvement was seen only in ANCA (-) group (n = 3). Biological agents (mepolizumab or rituximab) were prescribed to nine patients in ANCA (-) and two patients in ANCA (+) group. The median duration of follow-up was 47 (IQR 69.9) months. ~ 40% of patients had at least one relapse, but relapse-free survival rate was similar between the groups. However, the predictor of first relapse was elevated Ig E level [OR (95% CI): 6.5 (1.09-38.63) p = 0.04]. Consequently, both clinical features, disease activity, and treatments appear to be significantly different between EGPA subgroups. The relapse risk was similar although clinical features and treatment strategies were different. Also, elevated Ig E levels may be a precursor for the relapse.

QuantiFERON®-TB Gold In-Tube test can be used for screening latent tuberculosis before biological treatment in a Bacille Calmette-Guérin (BCG)-vaccinated country: the HUR-BIO single-center real-life results.

OBJECTIVES: 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. METHODS: 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. RESULTS: 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). CONCLUSIONS: 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.

Pregnancy outcomes in partners of male ankylosing spondylitis patients treated with anti-tumour necrosis factor-α biologics: real-life results from a single-centre cross-sectional study.

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.

Do ANCA-associated vasculitides and IgG4-related disease really overlap or not?

BACKGROUND: Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) and immunoglobulin G4-related disease (IgG4-RD) have some common features. The co-occurrence/concurrence of AAV and IgG4-RD was recently published by the collaborative European Vasculitis Study Group. First, we aimed to investigate ANCA positivity of our IgG4-RD cohort. Second, a literature review of co-occurrence/concurrence of AAV and IgG4-RD was done. METHODS: Data of 62 patients with IgG4-RD in Hacettepe Vasculitis Center Database were used. Patient dataset was designed to include demographic data, clinical characteristics, imaging and IgG4-RD, AAV and ANCA test results. At the next step, we performed a systematic literature review in PUBMED database covering the time period from 1976 until April 2018. Relevant publications were searched using these MeSH terms ''IgG4-related disease and Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis'', "IgG4-related disease and Eosinophilic Granulomatosis with Polyangiitis", "IgG4-related disease and Microscopic Polyangiitis" and "IgG4-related disease and Granulomatosis with Polyangiitis". RESULTS: Three (10.3%) of 29 patients had low titer ANCA positivity. These three patients didn't have any findings of vasculitis and no granuloma was seen in biopsy. In the literature review, we found 17 cases had features of both IgG4-RD and AAV. These cases were re-evaluated according to the Comprehensive Diagnostic Criteria for IgG4-RD. ANCA were positive in 15 of 17 patients (88%). CONCLUSION: None of our IgG4-RD patients overlapped with AAV. Only two patients in the literature review seemed to be fully compatible with both diseases. Even though AAV and IgG4-RD share similar clinical features, we think this might be a co-occurrence instead of a histopathological link.

Proposal for a simple algorithm to differentiate adult-onset Still's disease with other fever of unknown origin causes: a longitudinal prospective study.

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.

Correction to: Proposal for a simple algorithm to differentiate adult-onset Still's disease with other fever of unknown origin causes: a longitudinal prospective study.

The author regrets that the original version of this article contained error. Figure 1 was shown in the wrong version, thus corrected figure is shown in this article.

Change in PsAID-12 scores in patients continuing or discontinuing anti-TNF treatments in psoriatic arthritis: results from the HUR-BIO biologic registry.

OBJECTIVE: Psoriatic Arthritis Impact of Disease 12-item questionnaire (PsAID-12) has been developed to be used in psoriatic arthritis in daily practice. The aim of the present study was to assess the change values of PsAID-12 in PsA patients continuing or discontinuing anti-TNF treatment. METHODS: We recruited patients from the Hacettepe University biological database (HUR-BIO). Overall, 70 PsA patients had PsAID-12 score before the initiation of the first anti-TNF treatment. Stopping or switching the anti-TNF treatment due to inefficacy was definitely considered a negative response. Changes were evaluated by the comparison with the baseline PsAID-12 score in compliance with the favorable and unfavorable responses to anti-TNF treatments. The standardized response mean (SRM) was used for determining the response. RESULTS: Seventy (78.6% female) patients were analyzed and their mean age was 45.5 years (12.0 years). The mean follow-up duration was 18.3 months (12.6 months). At baseline, the mean PsAID-12 score was 6.6 (1.5). Physicians stopped or switched the treatment in 28 patients (40.0%) due to the inefficacy of anti-TNF treatment. The ΔPsAID-12 score was 0.25 (1.71) in the patients discontinuing anti-TNF treatment and 3.52 (2.31) in the patients continuing their anti-TNF treatment (p < 0.001). The SRM scores higher for PsAID-12, particularly in the well response to anti-TNF treatments. CONCLUSION: A decrease of 3.5 units in PsAID-12 score shows a favorable response to anti-TNF treatment. Changes in PsAID-12 score had well discrimination capacity for anti-TNF treatments.

Frequency of Behçet's disease among a group of visually impaired adults.

BACKGROUND: Behçet's disease (BD) is one of the reasons of acquired visual impairment among young adults. Ocular involvement is a significant cause of disability in BD. The objective of this study is to assess the prevalence of BD among a group of adults who has visual impairment. METHODS: Ankara Metropolitan Municipality Education and Technology Center is one of the official institutions which records and follows the demographic data of visually impaired people in Turkey. In November 2014, there were 675 visually impaired people recorded at this center. Medical history was taken from 294 adults by phone in November and December of 2014. Participants were asked if the visual impairment had been either acquired or congenital. If the patients had BD or suspicious BD, they were recalled for detailed investigation which would be carried out by an internist, a rheumatologist and an ophthalmologist. RESULTS: Two hundred thirteen of 294 (72.4%) visually impaired adults were male. One hundred nine of 294 (37.1%) had acquired visual impairment. Six (5.5%) of those 109 patients had BD. Overall prevalence of BD among study group was 2.04%.The median age of people with BD was 35 years. The median age at BD diagnosis was 16.5 years and the median duration from diagnosis to visual loss was 2.5 years. CONCLUSION: BD is still one of the causes of acquired visual impairment in Turkey. In this study, BD prevalence among a visually impaired adult group was 2.04%. BD accounted for 5.5% among adults who had acquired visual impairment. In a study of 1965, BD prevalence among people with acquired blindness was 12%. However, this study was conducted at pre-immunosuppressive period. Our prevalence is obviously lower than those studies. Extended population-based studies are needed for population estimations.

Polyarteritis nodosa: lessons from 25 years of experience.

OBJECTIVES: Polyarteritis nodosa is a necrotising vasculitis of predominantly medium size vessels. The present study aimed to summarise the characteristics of PAN patients, and also analyse the trend of decreasing PAN frequency in the last 25 years. METHODS: PAN patients followed up between 1990 and 2015 were included. The demographics, clinical findings and outcomes were retrospectively evaluated. RESULTS: One hundred thirty-three patients, including 66 children, were enrolled in the study. Among 133 patients, 86 (64.7%) had fever, 108 (81.2%) had skin involvement, 54 (40.6%) had renal involvement, 43 (32.3%) had neurological involvement, 32 (24.1%) had gastrointestinal involvement, 10 (7.5%) had cardiac involvement, 6 (4.5%) had pulmonary involvement. The median (minimum-maximum) leukocyte count, erythrocyte sedimentation rate and C-reactive protein levels at the time of diagnosis were 10400 (6100-32000)/mm3, 58 (2-132) mm/h and 5.22 (0-46) mg/dL, respectively. All patients were ANCA negative. Hepatitis serology was analysed in 121 patients and found positive in 13 of them. MEFV mutations were screened among 65 patients, 24 of them had mutations in at least one allele. Biopsy was performed in 109 patients and angiography was performed in 92 patients. The number of PAN patients declined significantly after 2010. 9 patients were re-categorised as DADA2 after 2014 and no patient were diagnosed with FMF+PAN after 2008. CONCLUSIONS: Our results suggest a decrease in PAN in our country which may be due to improved healthcare and dissecting mimicking diseases. Further prospective studies with prolonged follow-up could help us to better understand the disease characteristics.

Which definition should be used to determine colchicine resistance among patients with familial Mediterranean fever?

OBJECTIVES: Colchicine is the main therapy for familial Mediterranean fever (FMF); however, 5-10% of patients are colchicine-resistant. There is no standard and validated definition for colchicine resistance. We aimed to compare the existing definitions for colchicine resistance in both adult and paediatric FMF patients to find out the best definition to determine colchicine-resistant patients. METHODS: 385 FMF patients were evaluated and patients receiving anti-interleukin-1 treatment were included. The anti-IL-1 therapy had been initiated by the experts in the past based on their experience. Eleven different definitions (found out after PubMed search for colchicine resistance in FMF) were applied to all patients. Results were re-analysed after excluding the patients who had no clinical attacks but persistently high acute phase reactants (APRs) and/or amyloidosis. RESULTS: Sixty patients (40 adults/20 children) who had been using anti-IL-1 therapy were included into this study as colchicine-resistant patients. The highest percentage of patients fulfilled definition 5 (93.3%). Definition 9 had the poorest performance (26%). Significantly, a higher percentage of adult patients met definitions 4 and 6 than paediatric patients (87.5% vs. 50%, p=0.002; 75% vs. 40%, p=0.008, respectively). After excluding patients without clinical attacks, the highest percentage of patients fulfilled definition 2 (94.4%). We combined the attack frequency (>1 typical episode/3 months) in definition 2 and presence of amyloidosis/APR increase (increase in ≥2/3 APRs) in definition 5 to create a new definition which was met by 59 (98.3%) colchicine-resistant FMF patients. CONCLUSIONS: Definition of colchicine resistance is still controversial. Definitions with both clinical and laboratory criteria were met by a higher percentage of resistant patients than those without laboratory criteria. However, the proper definitions for the attack-free period and persistence of APRs are still lacking.

Therapeutic plasma exchange for refractory SLE: A comparison of outcomes between different sub-phenotypes.

OBJECTIVE: Therapeutic plasma exchange (TPE) offers an alternative therapeutic modality for patients with systemic lupus erythematosus (SLE) and primary antiphospholipid syndrome (APS). However, there is conflicting evidence regarding its efficacy in different sub-phenotypes. This study aimed to investigate the main clinical characteristics and outcomes of patients with different phenotypes of SLE and APS treated with TPE at a tertiary care center. METHODS: The database of the Blood and Apheresis Unit between 2001 and 2013 was screened for patients with SLE and primary APS. SLE disease activity index (SELENA-SLEDAI), the indications for treatment, complications, and outcomes were obtained from a review of medical records and phone calls. A total of 24 patients (SLE: 20, APS: 4) were recruited for the study. RESULTS: Mean ages of SLE (M/F: 1/19) and primary APS (PAPS) patients (M/F: 2/2) were 32.4±12.89 and 52.0±10.7 years, respectively. The main indications for TPE were hematologic, neurologic, and pulmonary involvement and APS-related symptoms. TPE was preferred in eight patients because of leucopenia and co-infection. SLEDAI was significantly decreased after TPE (16.7±8.3 before vs. 8.8±3.1 after, p=0.001). Both primary APS and SLE-related catastrophic APS (CAPS) patients had completely responded to TPE. The success rate of TPE in patients with thrombocytopenia was lower than patients with hemolytic anemia. The median (IQR 25%-75%) number of TPE sessions was 6.5 (5-10.5). In total, five patients experienced TPE-related major adverse events (catheter infections in three patients, bleeding in one patient, and hypotension in one patient). The median (IQR 25%-75%) follow-up time was 33.5 (6.75-81.25) months. In total, four patients died during follow up, of which three died during the period of TPE administration. CONCLUSION: Our data suggest that CAPS and other APS-related problems respond well to the TPE treatment. TPE should be kept in mind for the treatment of patients with other features of SLE, especially those resistant to other agents and in the presence of leucopenia.

Starting of biological disease modifying antirheumatic drugs may be postponed in rheumatoid arthritis patients with multimorbidity: Single center real life results.

The objective of this study was to assess the frequency of comorbidities and multimorbidities in rheumatoid arthritis (RA) patients under biologic therapy and their effects on biological disease modifying antirheumatic drugs (DMARDs) choice, timing, and response.Hacettepe University Biologic Registry (HUR-BIO) is single center biological DMARD registry. Cardiovascular, infectious, cancer, and other comorbidities were recorded with face to face interviews. Multimorbidity is defined as >1 comorbidity. Disease duration, initial date of biological DMARDs, initial and overall biological DMARD choice were recorded. Disease activity score-28 (DAS-28) responses were compared to comorbidity presence and multimorbidity.Total of 998 RA patients were enrolled into the study. The mean age was 53.1 (12.5) and mean disease duration (standard deviation [SD]) was 11.7 (7.5) years. At least 1 comorbidity was detected in 689 (69.1%) patients, 375 (37.9%) patients had multimorbidity. Patients had mean 1.36 ±â€Š1.32 comorbidity. The median durations of first biological DMARDs prescription were 60 (3-552) months after RA diagnosis. For multimorbidity patients, the median first biological prescription duration was longer than the duration for patients without multimorbidity (72 [3-552] vs 60 [3-396] months, P < .001). The physicians prescribe tumor necrosis factor inhibitor (TNFi) biological drugs less frequently than other biological DMARDs in patients with at least 1 comorbidity (66.2% vs 74.5%, P = .007) or multimorbidity (34.6% vs 43.5%, P = .006). Patients with comorbidities and multimorbidity achieved DAS-28 remission less frequently than patients without comorbidity (31.6% vs 42.6%, P = .012 and 27.2% vs 39.7%, P = .001, respectively).In real life, physicians may postpone to prescribe biological DMARDs and less frequently choose TNFi biological drugs in patients with multimorbidity. Furthermore, comorbidity may have a negative effect on the treatment response.