Search

Aim: This study examines treatment patterns and preferences among patients diagnosed with Axial Spondyloarthritis (AxSpA) across different age groups. Material and Method: Ankara Bilkent City Hospital registry enabled a comprehensive cross-sectional analysis of 2,811 patients stratified into three age groups: 18-40, 41-55, and over 55 years. These groups were compared in terms of their treatments. Results: Our findings indicate an increasing prevalence of female patients and comorbidities with age. Medication usage patterns showed a trend towards increased use of Methotrexate and Colchicine with age, while Sulfasalazine and Leflunomide were more commonly prescribed in older age groups. Notably, the use of biologic Disease-Modifying Anti-Rheumatic Drugs (bDMARDs), including anti-Tumor Necrosis Factor (anti-TNF)", "anti-Interleukin (anti-IL) agents, demonstrated a declining trend with advancing age, though not reaching statistical significance. This trend was also reflected in gender-specific treatment distributions, where no significant difference was found in bDMARDs administration among patients over 55 years, contrasting with a higher usage rate in younger male patients. Conclusion: Our study highlights a shift towards more conservative treatment approaches, such as increased conventional synthetic Disease-Modifying Anti-Rheumatic Drugs (csDMARDs) use in older patients, likely due to their safety profile and the specific challenges associated with treating older adults, including higher comorbidity rates and medication side effects. These findings emphasize the need for personalized treatment strategies and suggest potential adjustments in clinical practices to better accommodate the aging population, advocating for ongoing research to optimize treatment efficacy and safety for elderly patients with AxSpA. [ABSTRACT FROM AUTHOR]

BACKGROUND: Long-COVID refers to a variety of symptoms that continue for at least 4 weeks following the onset of acute COVID-19 infection. "Microclots/microvasculopathy" is a potential cutting-edge theory. Nailfold capillaroscopy is a non-invasive method used to assess microvascularity. In this study, we aimed to compare baseline characteristics and capillaroscopic findings of patients with and without long-COVID syndrome. METHODS: Baseline clinical characteristics of 53 patients who tested positive for SARS-CoV-2 were recorded. At the time of COVID-19 diagnosis, patients underwent nailfold capillaroscopy. One year later, patients were rescreened for long-COVID symptoms. Comparisons were made between patients with and without long-COVID syndrome in terms of their baseline characteristics and capillaroscopic findings. RESULTS: There were 35 individuals (66%) with long-COVID syndrome. The most common symptoms related to long-COVID were fatigue (43.4%), myalgia (34%), arthralgia (20.8%), dyspnea (20.8%). In total, 22 patients (41.5%) had abnormal capillaroscopy findings. Like other baseline characteristics, the proportion of patients with abnormal capillaroscopic findings (40% vs 44%, p=0.76) was similar between patients with and without long-COVID syndrome. CONCLUSION: Microvasculopathy and microthrombotic vascular damage are among the strongest hypotheses discussed in this regard. Our results may suggest that factors, rather than baseline microvasculopathy, may drive pathophysiological mechanism underlying the poorly understood long-COVID syndrome [ABSTRACT FROM AUTHOR]

Rheumatoid arthritis (RA) is a chronic inflammatory disease with a higher incidence in older adults. We aimed to analyze the differences in the choice of RA treatment regimens across various age groups. A retrospective cohort study using data from the Ankara Bilkent City Hospital Registry was conducted, involving 3,475 RA patients categorized into three age groups: 18-49, 50-64, and over 65 years. Key variables examined included demographics, comorbidities, and utilization of various treatment modalities, including disease-modifying antirheumatic drugs (DMARDs), tumor necrosis factor-alpha inhibitors (TNFi), targeted synthetic DMARDs (tsDMARDs), and other biological treatments. Statistical analysis was done with Jamovi software. The study found significant differences in drug prescriptions among age groups. Older patients (>65 years) were less likely to be prescribed methotrexate and sulfasalazine but more likely to receive leflunomide and colchicine. A decline in the use of TNFi and tsDMARDs with age was noted, while other biologics were consistently used across all ages. Our findings underscore the impact of age on treatment choices in RA, revealing a tendency towards less aggressive treatment approaches in older patients. We emphasize the importance of re-evaluating current treatment protocols to better accommodate the health needs of the aging RA population and enhance their quality of life. [ABSTRACT FROM AUTHOR]

Artificial intelligence (AI) technology is revolutionizing the healthcare industry and medical research. AI offers possibilities for faster and more accurate diagnosis of diseases, personalized treatments, and the discovery of new drugs. AI-driven medical imaging can enhance healthcare quality by identifying conditions at early stages, reducing pressure on doctors and improving diagnostic procedures. AI also has the potential to significantly improve the drug development process, simplify clinical trials, and recommend personalized treatments. However, the integration of AI into medical research requires interdisciplinary collaboration, ethical considerations, and patient safety. AI is also shaping the future of healthcare through robotic surgical systems and telemedicine. While there are potential benefits, it is important to adhere to academic ethics and research standards to maintain integrity and trust in the scientific community. [Extracted from the article]

Objectives: To investigate the association between vascular inflammation, as detected by positron emission tomography (PET) imaging and interleukin-6 (IL-6), pentraxin3, and B-cell-activating factor (BAFF) in subjects with LVV. Methods: The study included newly diagnosed giant cell arteritis (GCA, n=27) or Takayasu arteritis (n=9) patients and healthy control (HC, n=31) subjects. PET scan and blood samples were obtained before the introduction of treatments. IL-6, PTX3, and BAFF levels were determined quantitatively by enzyme-linked immunosorbent assay kits. Results: Thirty-six patients with LVV (20 females, 16 males; age 64.5±16.6 years) and 31 HC (14 females, 17 males; age 37.1±9.6 years) were included. Serum levels of IL-6, PTX3, and BAFF were increased in patients with newly diagnosed LVV compared with healthy control subjects. In receiver operating characteristics (ROC) analysis, serum IL-6 and BAFF provided excellent discrimination of newly diagnosed LVV patients from HC (area under the ROC curve of >0.90 and >0.80, respectively). None of the inflammatory markers correlated with vascular inflammatory activity determined by PET scanning. Conclusions: Our results suggest that IL-6 and BAFF may serve as markers of large vessel vasculitis, while PTX3 is not useful. None of the inflammatory markers correlated with PET assessed vasculitis activity. [ABSTRACT FROM AUTHOR]

Copyright of Van Tip Dergisi is the property of Yuzuncu Yil University, Faculty of Medicine and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

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. [ABSTRACT FROM AUTHOR]

Objective: The objective of this study was to assess the impact of Familial Mediterranean Fever (FMF) attacks on daily physical activity and detect FMF attacks using a connected activity tracker. Methods: Patients with FMF according to the Tel-Hashomer criteria were included in this prospective observational study. Attack-related data were collected weekly via phone call to avoid memory bias. Median steps in attack and attack-free days were calculated and compared using the Wilcoxon rank test. Sensitivity and specificity threshold for capturing attacks was set to two thirds of median steps per day in the whole observation period. Results: Twelve patients participated in the study. The median age of patients was 26 (18–32) years, and 7 (58.3%) of them were male. Patients with attacks (n = 10) walked a median of 6990 (4552–11,531) steps per day in attack-free days, whereas this number decreased to a median of 1841 (590–4783) steps in attack days (p = 0.005). The activity tracker captured 42 of 45 attack days and 312 of 361 attack-free days. The cutoff value had 93% sensitivity and 86% specificity for capturing attacks. Conclusions: FMF attacks significantly impair the physical activity of patients. Activity tracking may be a reasonable method to document FMF attacks. This might prevent errors due to memory bias and help accurately identify and treat patients with FMF. [ABSTRACT FROM AUTHOR]

Introduction: Behçet disease is a chronic vasculitis that generally affects young adults in the most productive period of their life. The purpose of this study is to evaluate patients' work productivity and daily activity impairment, and their relationship with disease activity and quality of life. Method: In this study, 55 patients with Behçet disease who are currently working and 50 healthy controls were included. To evaluate quality of life, a 36-item short form health survey was used. For the evaluation of Behçet disease activity and work productivity, the Behçet Disease Current Activity Form and Work Productivity and Activity Impairment questionnaire were used, respectively. Quality of life and work productivity between patients and healthy controls were compared. Results: Scores of all domains of the health survey were significantly worse in patients with Behçet disease (range, p = 0.006 to p < 0.001). The mean Work Productivity and Activity Impairment absenteeism, presenteeism and activity impairment scores were higher in patients with Behçet disease (p = 0.005, p < 0.001 and p < 0.001, respectively). There was a significant correlation between Behçet Disease Current Activity Form score and absenteeism (r = 0.32, p = 0.016). Moreover, there was significant correlation between Work Productivity and Activity Impairment and most domains of the health survey (range, r = −0.27 to –0.64, range, p = 0.047 to p < 0.001). Conclusion: Our results showed remarkable impairment in work productivity and health-related quality of life in Behçet disease. There is a strong correlation between work productivity and quality of life. To improve work productivity, more effective therapeutic approaches and improvements in working conditions should be provided. [ABSTRACT FROM AUTHOR]

Background: Familial Mediterranean fever (FMF) patients suffer from chronic complications of disease such as AA amyloidosis, chronic arthritis, and spondylitis. Reduced quality of life (QoL) is a feature of chronic diseases but it is also impaired in patients with FMF. Despite the regular use of colchicine at a maximal dose, about 10% of patients do not respond well or resistant to colchicine (crFMF). IL-1 inhibitors have been shown to be effective in controlling attacks in crFMF patients. Herein, we aimed to investigate QoL changes of crFMF patients with IL-1 inhibitors.Methods: All patients were prospectively monitored for the frequency, duration, severity of attacks, patient global assessments (Visual Analog Scale; VAS), and laboratory features. Either anakinra or canakinumab was used as IL-1 antagonist treatments. Demographic information, MEFV gene mutations, attack characteristics, and previous treatments were registered. Short form-36 (SF-36) quality of life scale was implemented by the interviewer for evaluating the QoL before and 3 months after the treatment.Results: A total of 44 patients were included in this study. Striking improvements were detected in frequency, duration, and VAS severity of attacks (p < 0.001). In the comparison of pre- and post-treatment, SF-36 sub-components significant improvements were observed on physical function, role limitation due to physical difficulty, role limitation due to emotional problem, energy, emotional well-being, social function, pain, general health, and health change.Conclusions: In conclusion, IL-1 antagonists prevent attacks and improve QoL of crFMF. [ABSTRACT FROM AUTHOR]

Background: Colchicine is the mainstay of the treatment of familial Mediterranean fever (FMF). However, 10% of FMF patients do not respond well to colchicine. Efficacy of interleukin (IL)-1 inhibitors in reducing attacks have been demonstrated in colchicine-resistant FMF (crFMF) patients recently. Colchicine is still the only approved drug for the prevention of amyloidosis in FMF and utility of IL-1 inhibitors in crFMF cases who already has amyloidosis remain to be elucidated. Herein, we evaluated efficacy and safety of IL-1 inhibitors in patients with crFMF-associated AA amyloidosis in a relatively large single center study. Methods: Medical records of FMF patients complicated with AA amyloidosis in our dedicated FMF center were retrospectively reviewed and those patients who ever treated with IL-1 inhibitors were enrolled into the study. Patient global, physician global assessments (on 0-10cm visual analog scale), C-reactive protein (CRP), erythrocyte sedimentation rate (ESR), serum creatinine and 24-h urinary protein excretion values for each visit were recruited from computer-based hospital records. Treatment response of patients were assessed with clinical symptoms, serum albumin, CRP and ESR values. Renal outcome parameters were analyzed on those not receiving renal replacement therapy. Results: Seventeen patients were identified with crFMF-amyloidosis that ever treated with IL-1 inhibitors. Background colchicine therapy was continued in all patients in maximal-tolerated dose along with IL-1 inhibitors. All patients benefit from IL-1 antagonists assessed by patient and physician global assessments. Inflammatory markers, CRP and ESR, were significantly reduced in all and normalized in 12 out of 17 patients. More importantly, the amount of proteinuria was remarkably improved following IL-1 inhibitor therapy (1606mg/day to 519 mg/day, p=.008). Both anakinra and canakinumab were well-tolerated without severe side effects. All patients were initially treated with anakinra but switched to canakinumab in seven patients (one leukopenia, four injection site reaction, two inefficacy). Conclusion: We evaluated the clinical and laboratory responses to IL-1 inhibitors in crFMF-associated amyloidosis patients. We found significant decreases in CRP, ESR and proteinuria after IL-1 inhibitor therapy. This study confirmed that IL-1 inhibitors are effective for controlling attacks and inflammatory activity in FMF patients complicated with AA amyloidosis. Moreover, they reduce or stabilize amount of proteinuria and preserve renal function in short-term follow-up. Prolonged prospective clinical trials are warranted to assess their long-term efficacy in this particular patient group. [ABSTRACT FROM AUTHOR]

To evaluate the efficacy of on-demand use of anakinra in patients with crFMF. The Gazi FMF cohort was established in the year 2010, and from that date, 689 patients with FMF diagnosed according to the Tel Hashomer criteria were registered. Attack type, duration, severity, and their impact on life were collected either by disease diaries or a mobile phone application (FMF AIDD, AppStore, and Playstore). A retrospective cohort analysis was made from records of patients who have ever been treated with IL-1 inhibitors. A total of 78 patients were treated with IL-1 inhibitors in our cohort. Among these, 15 patients were identified who received on-demand anakinra. Rationale for on-demand use was prominent prodrome or trigger for attacks and patient's personal claim. Six patients were switched from regular use and nine were directly started as on-demand use. All were using background colchicine in maximum tolerated doses. None of the patients had evidence of persistently elevated acute phase reactants or proteinuria. The median duration of on-demand anakinra use was 6 (3-36) months. Pre- and post- on-demand anakinra periods were compared. Patient reported attack severity (p = 0.002), duration (p = 0.001), frequency (p = 0.001), absenteeism (p = 0.002), and presenteeism (p = 0.002) were significantly improved. On-demand anakinra prevented progression of prodromes to full-blown attacks which was demonstrated by decrease in the rate of attack/prodrome ratio (p = 0.02). On-demand anakinra can be continued in ten subjects on long-term. On-demand anakinra significantly improved FMF attacks in certain patients which suggest this approach would be of benefit in daily practice in selected patients. [ABSTRACT FROM AUTHOR]

The article presents a case study of a 33‐year-old male patient with left leg pain and edema. It notes that diagnosis of Behçet syndrome (BS) with deep vein thrombosis (DVT) and reports immunosuppressive treatment with ultrasound‐accelerated catheter‐directed thrombolysis (UACDT) using the Ekosonic Endovascular System (EKOS.).

Background: Long-COVID refers to a variety of symptoms that continue for at least 4 weeks following the onset of acute COVID-19 infection. "Microclots/microvasculopathy" is a potential cutting-edge theory. Nailfold capillaroscopy is a non-invasive method used to assess microvascularity. In this study, we aimed to compare baseline characteristics and capillaroscopic findings of patients with and without long-COVID syndrome., Methods: Baseline clinical characteristics of 53 patients who tested positive for SARS-CoV-2 were recorded. At the time of COVID-19 diagnosis, patients underwent nailfold capillaroscopy. One year later, patients were rescreened for long-COVID symptoms. Comparisons were made between patients with and without long-COVID syndrome in terms of their baseline characteristics and capillaroscopic findings., Results: There were 35 individuals (66%) with long-COVID syndrome. The most common symptoms related to long-COVID were fatigue (43.4%), myalgia (34%), arthralgia (20.8%), dyspnea (20.8%). In total, 22 patients (41.5%) had abnormal capillaroscopy findings. Like other baseline characteristics, the proportion of patients with abnormal capillaroscopic findings (40% vs 44%, p=0.76) was similar between patients with and without long-COVID syndrome., Conclusion: Microvasculopathy and microthrombotic vascular damage are among the strongest hypotheses discussed in this regard. Our results may suggest that factors, rather than baseline microvasculopathy, may drive pathophysiological mechanism underlying the poorly understood long-COVID syndrome (Tab. 2, Ref. 35).

Objectives: To investigate the association between vascular inflammation, as detected by positron emission tomography (PET) imaging and interleukin-6 (IL-6), pentraxin3, and B-cell-activating factor (BAFF) in subjects with LVV., Methods: The study included newly diagnosed giant cell arteritis (GCA, n = 27) or Takayasu arteritis (n = 9) patients and healthy control (HC, n = 31) subjects. PET scan and blood samples were obtained before the introduction of treatments. IL-6, PTX3, and BAFF levels were determined quantitatively by enzyme-linked immunosorbent assay kits., Results: Thirty-six patients with LVV (20 females, 16 males; age 64.5 ± 16.6 years) and 31 HC (14 females, 17 males; age 37.1 ± 9.6 years) were included. Serum levels of IL-6, PTX3, and BAFF were increased in patients with newly diagnosed LVV compared with healthy control subjects. In receiver operating characteristics (ROC) analysis, serum IL-6 and BAFF provided excellent discrimination of newly diagnosed LVV patients from HC (area under the ROC curve of >0.90 and >0.80, respectively). None of the inflammatory markers correlated with vascular inflammatory activity determined by PET scanning., Conclusions: Our results suggest that IL-6 and BAFF may serve as markers of large vessel vasculitis, while PTX3 is not useful. None of the inflammatory markers correlated with PET assessed vasculitis activity., (© Japan College of Rheumatology 2021. Published by Oxford University Press. All rights reserved. For permissions, please e-mail: journals.permissions@oup.com.)

Background: Follow-up is crucial to detect asymptomatic complications of familial Mediterranean fever (FMF). The current European League Against Rheumatism recommendations state that patients with FMF should be evaluated at least every 6 months to monitor attacks, acute phase response, and proteinuria., Objectives: This study aimed to assess compliance of FMF patients with regular follow-up visits and the associated factors., Methods: Adult patients with a diagnosis of FMF who had their initial visit at least over 1 year ago were included. Demographic and socioeconomic data, family history, and comorbid diseases were obtained from medical records. The International Severity Score for FMF and the Autoinflammatory Disease Damage Index scores were calculated. We defined patients as "compliant with follow-up visits" both if they had at least 2 visits during the previous year and a compatible physician's assessment. The characteristics of the compliant and noncompliant patients were compared, and multivariable logistic regression analysis was used to determine the factors influencing visit compliance., Results: Four hundred seventy-four patients with FMF were included. Two hundred thirty (48.5%) were compliant, and 244 (51.5%) were noncompliant with follow-up visits. A family history of FMF in parents, the absence of a family history of FMF in siblings, treatment with biologic agents, concomitant medication use, multisite involvement during FMF attacks, and treatment satisfaction were independent predictors of visit compliance., Conclusions: Only half of the patients with FMF were compliant with follow-up visits. Better strategies should be implemented to increase the compliance of FMF patients. Identifying independent predictors would help to build one., Competing Interests: The authors declare no conflict of interest., (Copyright © 2020 Wolters Kluwer Health, Inc. All rights reserved.)

Objectives: Anakinra and canakinumab are the most commonly used agents in colchicine resistant/intolerant patients. In this study we investigated long-term efficacy and safety of anakinra and canakinumab., Methods: In this retrospective study, we enrolled 101 adult patients with familial Mediterranean fever (FMF). Clinical and laboratory parameters before and after treatment with anakinra/canakinumab and the side effects observed during the treatment were recorded. All patients received anakinra initially and switched to canakinumab, in case of inadequate response/intolerance., Results: The median (IQR) duration of treatment with anti-IL-1 agents was 35 (24-47.5) months. 101 patients were treated with anakinra and 27 patients with canakinumab. The autoinflammatory diseases activity and attacks decreased with both anakinra and canakinumab. Anakinra was effective in decreasing proteinuria and canakinumab was not effective in decreasing proteinuria in anakinra unresponsive patients. The modified FMF score was achieved in 76.2% of anakinra and 88.9% of canakinumab group. Injection site reactions (ISRs, n:15) was the most common reason of discontinuation of anakinra and most of ISRs developed in first 3 months of treatment. One severe skin rash, two anaphylactic reactions and one severe neutropenia were observed with anakinra; in the first, eighth, twelfth and fiftieth months, respectively. No severe side effects or side effect-related discontinuation of canakinumab were observed., Conclusions: Anakinra and canakinumab seem to be effective in long-term management of FMF patients. Canakinumab had a favourable safety/tolerability profile. Anakinra is also generally safe, but the serious side effects that may be observed in the short and long-term use should be taken into account.

Background: Sometimes, the underlying causes of inflammation cannot be established despite meticulous investigation, including medical history, physical examination, laboratory tests, and radiologic procedures. Rheumatologists are often faced with patients whose condition is known as inflammation of unknown origin (IUO). Differential diagnosis of IUO is diverse, and investigation of these cases is challenging and time-consuming., Objective: The study aimed to assess the diagnostic role of positron emission tomography/computed tomography (PET/CT) in the evaluation of patients with IUO., Methods: The study sample consisted of 97 adult patients with IUO who have not been previously diagnosed with an infectious, inflammatory, or malignant disease. The necessary data were collected from January 2015 to June 2018 with a 6-month follow-up period. The patients were screened using PET/CT after a specific diagnosis could not be established with detailed laboratory and radiologic evaluations., Results: A final diagnosis was established at follow-up, and 47 (54%) of the 97 patients had inflammatory diseases, 30 (34.4%) had malignancies, and 10 (11.4%) had infections. Despite meticulous investigation, 10 patients were left undiagnosed in the follow-up. PET/CT aided diagnosis in 59 patients (60.8%), but it was not helpful in 38 patients (39.2%). PET/CT was positive in 30 (63%) of the 47 patients with inflammatory diseases, whose final diagnosis was inflammatory rheumatic disease, as follows: large-vessel vasculitis in 19 patients, polymyalgia rheumatica in 7 patients, and seronegative arthritis or other rare miscellaneous diseases in 4 patients. The sensitivity of PET/CT was 67% with a specificity and diagnostic accuracy of 100% and 71%, respectively., Conclusions: Investigation of the underlying etiology of IUO is time-consuming and challenging. PET/CT may help identify the final diagnosis more quickly by locating an obscure inflammatory site; thus, it may reduce the number of unnecessary biopsies, diagnostic time, anxiety, work loss, morbidity, and mortality., Competing Interests: All authors declare no conflict of interest., (Copyright © 2020 Wolters Kluwer Health, Inc. All rights reserved.)

Competing Interests: The authors declare no conflict of interest.

Objectives: 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., Methods: 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., Results: 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., Conclusions: 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.

Objective: We aimed to present our single-center real-life experience of canakinumab use in adult patients with familial Mediterranean fever (FMF)., Methods: Data were derived from the Gazi FMF cohort, which was established in 2010. From that year, all patients with FMF were registered. The impact of FMF on their lives was tracked by either an FMF diary or mobile phone application (FMF-AIDD, free to download in App Store and Google Play). The records of patients who were treated with canakinumab were reviewed., Results: Twenty-three adult patients with FMF (65% female) were enrolled in this study. The median age was 32 years (min-max, 24-58 years), and the disease duration was 26 years (14-59 years). A total of 86% of patients harbored homozygous or compound heterozygous exon 10 MEFV mutations. Indications for interleukin 1 inhibitor use were colchicine resistance (n = 12) or intolerance (n = 2), amyloidosis (n = 7), and chronic manifestations of the disease (n = 2). All patients used anakinra before. The median duration of canakinumab use was 7 months (min-max, 1-44 months). Pre- and post-canakinumab periods were compared. Attack severity, duration, frequency, C-reactive protein level, and erythrocyte sedimentation rate were significantly improved (p ≤ 0.01), whereas serum creatinine and alanine aminotransferase levels remained the same (p = 0.27 and p = 0.74, respectively). Canakinumab achieved complete disease remission in 14 patients (60%). Canakinumab was discontinued in 7 patients. The reasons for discontinuation were pregnancy (n = 2), dominance of axial spondyloarthropathy (n = 2), inflammatory bowel disease, patient's refusal, and weight gain., Conclusions: Canakinumab is effective in the prevention of FMF bouts without severe adverse effects. Treatment with canakinumab in an individualized dose and interval may be a reasonable choice for colchicine- and anakinra-resistant or -intolerant adult patients with FMF and those with chronic manifestations of disease.

Aim: To evaluate the relationship between serum adenosine deaminase (ADA) levels and histological features in patients with autoimmune hepatitis (AIH)., Methods: A total of 80 subjects (52 AIH cases and 28 healthy controls) were included in the study. Patients were diagnosed according to the simplified criteria suggested by the International Autoimmune Hepatitis Group. All of the cases had been diagnosed with AIH between 2010-2015 at Hacettepe University, Department of Gastroenterology. Serum blood samples were collected and stored at -80 °C until the biochemical estimation of ADA activity. The diagnosis of patients was confirmed by liver biopsy. Serum ADA > 20 U/L was considered to be high level., Results: Mean serum ADA levels were significantly higher in AIH patients than those in healthy controls (25.4 ± 9.6 U/L vs 12.8 ± 2.2 U/L, P < 0.001). Serum ADA levels > 20 U/L were found in 63.5% AIH patients and in 0% healthy controls ( P < 0.001). Mean serum ADA levels were significantly increased in each stage of histological activity: 15.2 ± 3.5 U/L for patients with mild interface hepatitis, 23.1 ± 10.0 U/L for patients with moderate interface hepatitis and 30.9 ± 7.0 U/L for patients with severe interface hepatitis ( P < 0.001). Correlation analysis showed that there was a positive association between serum ADA levels and histological activity ( r = 0.71, P < 0.001). Receiver operating characteristic analysis suggested that 24.5 U/L was the optimum cut-off point of ADA level for severe interface hepatitis (sensitivity 88%, specificity 85.2%, area under the curve: 0.88)., Conclusion: Because of the positive correlation with inflammatory activity, serum ADA level may be a potential biomarker for predicting or monitoring histological activity in patients with AIH., Competing Interests: Conflict-of-interest statement: We have no financial relationships to disclose.