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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.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.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.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.

Aim: The prognostic nutritional index (PNI), which includes serum albumin level and total lymphocyte count in the peripheral blood, is as an index that essentially reflects theimmunonutritional status of the patients. We investigate the role of PNI with SLEDAI-2K as an independent predictor of active disease in patients with systemic lupus erythematosus (SLE) in Turkey.Methods: PNI was calculated in 156 SLE patients according to the following formula: 10 x serum albumin value (g/dL) + 0.005 x peripheral lymphocyte count (/mm3).Spearman correlation analysis was used for analyzing correlations between PNI and laboratory variables. Logistic regression analyses were performed to assess the relationship between laboratory and clinical variables and SLE Disease Activity Index-2000 (SLEDAI-2 K).Results: Seventy-nine patients were defined as active SLE (SLEDAI-2K>10) and 77 as inactive SLE. Patients with active SLE have lower median PNI than those with inactive SLE (38 vs. 42, p, Amaç: Periferik kandaki serum albümin seviyesi ve lenfosit sayısı ile hesaplanan ve hastaların immün-beslenme durumu yansıtan prognostik nutrisyon indeks (PNI) ile Türkiye’deki SLE hastalarında hastalık aktivitesi (SLEDAI-2K) arasındaki ilişkiyi göstermeyi ve PNI’nın hastalık aktivitesinin bağımsız bir prediktörü olduğunu göstermeyi amaçladık.Yöntem: PNI, 156 SLE hastasında aşağıdaki formüle göre hesaplandı: 10 x serum albümin değeri (g / dL) + 0.005 x periferik lenfosit sayısı (/ mm3). PNI ile laboratuvar değişkenleri arasındaki ilişkilerin analizinde Spearman korelasyon analizi kullanıldı. Laboratuvar ve klinik değişkenler ile SLE Hastalık Aktivite İndeksi-2000 (SLEDAI-2 K) arasındaki ilişkiyi göstermek için lojistik regresyon analizleri yapıldı.Bulgular: 79’u hastalık aktivitesi yüksek (SLEDAI-2K>10)ve 77’si hastalık aktivitesi düşük toplam 156 SLE hastası çalışmaya alındı. Yüksek hastalık aktivitesine sahip SLE hastalarının medyan PNI'si, hastalık aktivitesi düşük olanlar ile kıyaslandığında daha düşük saptanmıştır (38'e karşı 42, p

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.

Objectives To evaluate the impact of familial Mediterranean fever (FMF) features on the clinical course and outcomes of coronavirus disease 2019 (COVID-19) and clinical course of FMF after COVID-19. Methods Consecutive FMF patients with COVID-19 were enrolled from three referral hospitals. Clinical features of FMF and detailed COVID-19 information were obtained from patient interviews and medical records. Results Seventy-three FMF patients were included in the study. 94.5% of patients had clinical symptoms of COVID-19. We found 24.7% hospitalization, 12.3% respiratory support, 4.1% intensive care unit admission, 6.8% complication, and 1.4% mortality rate in patients. The risk factors of hospitalization for respiratory support were male gender [OR: 7.167 (95% CI: 1.368–37.535)], greater age [OR: 1.067 (95% CI: 1.016–1.121)], and non-adherence to colchicine treatment before the infection [OR: 7.5 (95% CI: 1.348–41.722)]. One-third of patients had reported attacks after COVID-19. The patterns of triggered attacks were fever, peritonitis, pleuritis, transient arthritis, chronic knee mono-arthritis, and protracted febrile myalgia. Conclusions FMF characteristics were not associated with worse outcomes of COVID-19. Colchicine non-adherence was the risk factor of hospitalization for oxygen support. The rate of FMF attacks after COVID-19 is prominently increased, with some of them being protracted and destructive.

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.

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.

OBJECTIVE: Behçet’s disease (BD) is a chronic, multisystem disorder that can cause severe morbidity and mortality. Monitoring tools that measure disease activity are required for effective management of BD. We aimed to investigate the association of prognostic nutritional index (PNI) with disease activity in BD. METHODS: In this cross-sectional study, we enrolled 88 adult patients with BD and 51 healthy controls. The patients were divided into patients with active and inactive BD according to their disease activities. PNI was calculated using the following formula: 10×serum albumin (g/dL)+0.005×peripheral lymphocyte count (per mm3). To evaluate BD activity, the Behçet Disease Current Activity Form was used. The relations of BD activity with PNI, neutrophil-to-lymphocyte ratio, platelet-to-lymphocyte ratio, erythrocyte sedimentation rate, and C-reactive protein were investigated. A receiver operator characteristic curve analysis was used to define the optimum cutoff value of PNI for active BD. RESULTS: A total of 48 patients were classified as having active BD and 40 as having inactive BD. Patients with active BD had lower mean PNI than patients with inactive BD and healthy controls (51.8±4.2, 57.4±2.9, and 56.6±3.6, respectively; p

There has been a substantial improvement in classifying patients with primary Sjögren's syndrome (pSS), with the new 2016 ACR/EULAR classification criteria. It was aimed to investigate the potential role of parotid elastography in the classification of patients with pSS, as well as the clinical diagnosis of those who do not otherwise fulfil the criteria.This is a cross-sectional analysis of patients with pSS followed up in tertiary out-patient rheumatology clinic. Patients' medical records were retrospectively investigated whether or not clinically diagnosed pSS patients fulfil 2016 ACR/EULAR criteria sets. Elastographic evaluation of parotid and submandibular glands bilaterally was performed when presented for follow-up. Strain ratio, shear wave velocity and Pascal values of the glands were obtained.Clinical data on 179 patients with Sjögren's syndrome were investigated. Ninety-six patients with pSS and 30 gender and age-matched healthy controls were included in the study. Eighty-six percent of the clinically diagnosed patients satisfied the 2016 ACR /EULAR criteria and were considered 'criteria patients', and the remaining were considered 'non-criteria patients'. Both criteria and non-criteria patients had significantly higher parotid strain ratio and submandibular velocity compared with healthy controls (p 0.001 and p 0.001 for parotid strain ratio and p 0.001 and p = 0.016 for submandibular velocity, respectively). Replacing labial gland biopsy findings with parotid strain ratio in the new classification criteria resulted in similar sensitivity and lower specificity, 91.6% and 80%, respectively.Parotid shear elastography is an easy and noninvasive method and might be a useful tool for the classification of patients with pSS, especially when labial gland biopsy is not feasible. Key Points • Salivary gland elastography (SGE) is a useful tool for the classification of patients with pSS. • SGE could be performed instead of labial biopsy without changing the diagnostic power of classification criteria.

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.

Introduction Systemic lupus erythematosus (SLE) is associated with an increased risk of pulmonary infections, as well as a rare condition known as shrinking lung syndrome (SLS). The diaphragm has an important role to play in lung physiology and might also play a role in these adverse events. Here, we aimed to investigate whether SLE patients have impairment in their diaphragmatic muscle thickness and function with respect to another connective-tissue disease: primary Sjögren’s syndrome (pSS). Method Patients diagnosed with SLE who were in remission or who had minimal disease activity and had at least one year of follow-up were included in this study. Patients with known lung pathology and smokers were excluded. Patients with pSS constituted the second experimental group. Ultrasonographic evaluation of the diaphragmatic muscle was conducted by an experienced independent sonographer at three time points, diaphragmatic thickness during deep and quiet inspiration and maximum expiration being measured. Diaphragmatic muscle function was evaluated with maximum expiratory pressure (MEP) and maximum inspiratory pressure (MIP). Results A total of 115 patients were studied ( n = 39 SLE; n = 76 pSS). The mean ± standard deviation ( SD) thickness of the diaphragmatic muscles during quiet inspiration was significantly reduced in patients with SLE compared to patients with pSS (2.32 mm vs. 2.81 mm; p Conclusion SLE patients had reduced diaphragmatic muscle thickness compared to those with pSS, which was associated with impaired functional tests. Further prospective studies are needed to investigate whether structural and functional impairments in diaphragmatic muscle play a role in an increased risk of pulmonary infections and SLS in patients with SLE.

The aim of the present study was to investigate serum fetuin-A (Fet-A) levels in patients with Takayasu arteritis (TA) and granulomatous polyangiitis (GPA) and to analyze the relationship between serum Fet-A levels and disease activity scores.Thirty-two TA and 28 GPA patients presented to the rheumatology clinic at Gazi University and met the criteria of American College of Rheumatology 1990 and 2012 International Chapell Hill meeting, respectively, and 20 healthy control subjects were included in the present study. We collected data on serum C-reactive protein (CRP), albumin, calcium, and phosphate levels as well as erythrocyte sedimentation rates. Calcification risk index (CRI) was calculated for each patient. The Birmingham Vasculitis Activity Score (BVAS) and Indian Takayasu Clinical Activity Score (ITAS), were used to assess disease activity in GPA and TA patients respectively.Serum Fet-A levels were significantly lower in the overall vasculitis group compared to control group (p = 0.015). In subgroup analysis, Fet-A levels were significantly lower in those with active disease, compared to control group (p = 0.001, for active TA (n = 18) and GPA (n = 17), respectively). However, there was no significant difference in serum Fet-A levels in inactive cases versus control subjects (p = 0.061, for inactive TA (n = 14) and GPA (n = 11), respectively). Serum Fet-A levels negatively correlated with BVAS (r = - 0.675) and ITAS scores (r = - 0.385), as well as with CRP and CRI.Our results suggest that serum Fet-A level could be a novel biomarker for assessment of activity status in patients with GPA or TA. Key Points • Serum Fetuin-A is negative acute phase protein and systemic calcification inhibitor synthesized in hepatocytes and secreted by various inflammation. • Serum Fetuin-A was negatively correlated with CRP, BVAS, and ITAS scores and significantly decreased in vasculitis patients with high disease activity. • Serum Fetuin-A could be a promising and useful biomarker for the assessment of disease activity for vasculitis, also that it might also be a predictor of long-term cardiovascular progression.

Objectives The 6-minute stepper test (6MST) is a submaximal test that requires little space to assess exercise capacity compared to the 6-minute walk test (6MWT). The study aims to investigate the test-retest reliability and convergent validity of 6MST and to compare physiological responses, dyspnea, fatigue perception with 6MST and 6MWT in patients with ankylosing spondylitis (AS). Methods To test the convergent validity of 6MST, 65 patients performed both 6MWT and 6MST on the first day and correlation between two tests were assessed with Pearson correlation test. In order to investigate the test-retest reliability of the 6MST, 32 of the 65 patients performed 6MST one week later and intraclass correlation coefficients (ICC) were calculated. Dyspnea and fatigue perception were analyzed with using Wilcoxon signed-rank test, physiological responses were analyzed using paired sample t-test. Results Excellent test-retest reliability was observed for 6MST (ICC: 0.988). There was a significant correlation between 6MST and 6MWT (r: 0.725, p < 0.001). Dyspnea and leg fatigue perception were significantly higher in 6MST (p < 0.05). Physiological responses and fatigue perception were similar in both 6MST and 6MWT (p > 0.05). Conclusion This study demonstrated that the 6MST is reliable and valid method to evaluate exercise capacity in patients with AS. 6MST can be used to evaluate exercise capacity of patients with AS.

Coronaviruses are enveloped, positivepolarity, single-stranded RNA viruses that can cause respiratory and gastrointestinal tract infections, less likely to cause infections with hepatic, neurological and nephrotic involvement. A novel coronavirus termed as severe acute respiratory syndrome coronavirus-2 (SARS-CoV-2) emerged in the city of Wuhan, China, and caused an outbreak of unusual viral pneumonia at the end of 2019. This study aimed to reveal the relationship between systemic immune-inflammation index (SII), C-reactive protein (CRP) and interleukin-6 (IL-6) and viral dynamics in COVID-19 patients. This retrospective, single-center study was conducted in Ankara City Hospital from April 1 to May 31, 2020. A total of 338 hospitalized patients who had positive results in SARS-CoV-2 reverse transcrytase polymerase chain reaction (RT-PCR) test from nasopharyngeal and oropharyngeal samples during their hospital admission were included in this study. Patients were divided into three groups according to their ward/intensive care unit, intubation and mortality situation and their clinical data were evaluated. Correlation analysis was performed to determine the relationship between viral dynamics and laboratory parameters such as SII, the neutrophil-to-lymphocyte ratio (NLR), the lymphocyte-to-CRP ratio (LCR), the lymphocyte-to-monocyte ratio (LMR), the platelet-to-lymphocyte ratio (PLR), CRP, IL-6 ferritin, albumin levels and lymphocyte count. Advanced age, low Ct value, increase in IL-6, increase in SII, decrease in albumin, increase in ferritin, decrease in lymphocyte count, increase in NLR, decrease in LCR, decrease in LMR, increase in PLR and increase in CRP levels were found statistically significantly different in all three groups (p

PubMedID: 31651120 Background/aim: Rheumatoid arthritis (RA) is an autoimmune disease characterized by synovial inflammation. The study aimed to assess serum 14-3-3eta, anti-CarP, and anti-Sa in seronegative RA (SNRA) patients who were treatment-naïve as well as in healthy subjects. This is the first study in the literature to examine these autoantibodies together in SNRA patients. Materials and methods: Forty-five treatment-naïve SNRA patients and 45 healthy subjects were recruited. Drugs change the levels of autoantibodies; therefore, patients who took any medication had been excluded from our study. Anti-carbamylated protein, anti-Sa, and 14-3-3eta were measured by using three different ELISA kits. Results: Median serum concentration of healthy controls in 14-3-3eta was 0.02 (0.02–0.27) ng/mL. Median serum concentration of SNRA patients in 14-3-3eta was 1.00 (0.48–1.28) ng/mL. Data were analyzed with Mann–Whitney U tests; the P-value was

Objectives The 6-minute stepper test (6MST) is a submaximal test that requires little space to assess exercise capacity compared to the 6-minute walk test (6MWT). The study aims to investigate the test-retest reliability and convergent validity of 6MST and to compare physiological responses, dyspnea, fatigue perception with 6MST and 6MWT in patients with ankylosing spondylitis (AS). Methods To test the convergent validity of 6MST, 65 patients performed both 6MWT and 6MST on the first day and correlation between two tests were assessed with Pearson correlation test. In order to investigate the test-retest reliability of the 6MST, 32 of the 65 patients performed 6MST one week later and intraclass correlation coefficients (ICC) were calculated. Dyspnea and fatigue perception were analyzed with using Wilcoxon signed-rank test, physiological responses were analyzed using paired sample t-test. Results Excellent test-retest reliability was observed for 6MST (ICC: 0.988). There was a significant correlation between 6MST and 6MWT (r: 0.725, p 0.05). Conclusion This study demonstrated that the 6MST is reliable and valid method to evaluate exercise capacity in patients with AS. 6MST can be used to evaluate exercise capacity of patients with AS.

Objectives: Treatment of connective tissue disease-related interstitial lung disease (CTD-ILD) remains challenging. The literature related to the immunosuppressive drugs is very limited in most CTDs and there is no previously reported study comparing induction regimens in patients with newly defined ‘interstitial pneumonia with autoimmune features (IPAF), We aimed to investigate the efficacy of azathioprine (AZA) used in induction regimens for interstitial lung disease in a variety of connective tissue disorders including IPAF, and compare it with cyclophosphamide (CYC) Methods. In a retrospective study, all patients presented with interstitial lung disease to the rheumatology and/or pulmonology departments in a tertiary referral centre, between 2009 and 2019. Five major CTD groups were defined; systemic sclerosis, rheumatoid arthritis (RA), primary Sjögren syndrome (pSS), inflammatory myositis (IMS) and interstitial pneumonia with autoimmune features (IPAF). All patients who satisfied one of these and received AZA or CYC for induction therapy for at least 6 months were included in the study. Treatment responses at six months and side effects were analyzed. Results: There were 1351 patients diagnosed with ILD. Of these, 328 patients were identified as CTD-ILD, satisfying the classification criteria of one of the CTDs. Among these, 57 patients received AZA and 79 patients received CYC for induction therapy. CYC treatment resulted in a 2.41 % increase in FVC, however, AZA resulted in a 1.44% decline in FVC predicted (p=0.041). Propensity score matching was used to reduce selection bias, AZA treatment was related to increased risk of progression (50% vs 13.5%, p:0.002). Conclusion: CYC is superior to AZA in induction therapy of CTD-ILD.

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.

We read with great interest the article ‘Comorbidity and Long-Term Outcome in Patients with Congenital Heart Block and Their Siblings Exposed to Ro/SSA Autoantibodies In Utero’ by Mofors et al .1 A large and long-term follow-up population consisting of patients with congenital heart block (CHB) as a result of in utero Ro/SSA autoantibody exposure were studied. It contributes valuable information to our knowledge; however, …

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 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.

ObjectiveThe 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.MethodsPatients 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.ResultsTwelve 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.ConclusionsFMF 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.

Aim: To investigate the relationship between dietary fat content and adiposity and markers of metabolic syndrome in smokers and non-smokers.Methods: 211 participants were enrolled and anthropometric measures were recorded. Food consumption was assessed through the 24-hour dietary recall method and the amount of fat consumed by a single dietician is calculated as a low-fat diet and high-fat diet.Results: Smokers with high-fat diets were younger than those with low-fat diets (p < 0.001). When the WHR of the smokers was compared, it was found that it was higher among women with a low-fat diet compared to other groups (p = 0.03). Fasting blood sugar, Hs-CRP, and systolic blood pressure was lower in patients with a high-fat diet compared to smokers with a low-fat diet (p = 0.01, p = 0.02, p = 0.03, respectively) In our study, smoking was associated with an increased risk of dyslipidaemia (Risk Ratio = 2.76 (CI 95% 1.23, 6.56), p = 0.01). Smoking and a high-fat diet decreased the risk of dyslipidaemia (Risk Ratio = 0.22 (CI 95% 0.70, 0.74), p = 0.01).Conclusion: In addition to the current literature, our study showed that there was no relationship between a high-fat diet and components of MetS such as obesity, DM, HT, and dyslipidaemia. Only being smoker was associated with an increased risk of dyslipidaemia but a high-fat diet decreased the risk of dyslipidaemia in the smoker.

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.