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Anti-tumor necrosis factor (anti-TNF) agents are commonly used in treatment of axial spondyloarthritis (axSpA), but clinical and radiological improvement is not achieved in all patients. We aimed to investigate the impact of anti-TNFs on inflammatory and noninflammatory parameters in patients with axSpA.In this longitudinal study, 30 biologic naïve axSpA patients with high disease activity and 30 healthy controls were enrolled. All patients were treated with anti-TNF agents for 6 months. ASDAS-CRP, BASDAI, BASFI, BASMI, patient and physician global assessments were evaluated. C-reactive protein, COX2, TNF-α IL-6, IL-17, IL-22, IL-23, IL-33, sclerostin, dickkopf-1, and noggin levels were evaluated at baseline and at 6 months of anti-TNF treatment.At baseline, axSpA patients had significantly higher median (IQR) TNF-α levels, 34.4 (31.4-37.03) vs. 18.1 (12.1-28.4) pg/ml (p 0.001), and lower DKK1, 446.7 (356.9-529.3) vs. 1088.7 (951.7-1244.4) pg/ml, and sclerostin, 312.4 (140.8-412.7) vs. 412.3 (295.4-512.8) pg/ml, compared to healthy controls (all p 0.001). The median (IQR) serum levels of IL-17, IL-22, and IL-33 increased significantly after 6 months of anti-TNF treatment, from 93.3 (85.1-104.8) to 102.1 (86.6-114.6) pg/ml (p = 0.026), 159.2 (151.9-178.4) to 183.5 (156.3-304.6) pg/ml (p = 0.033), and 127.8 (106.6-186.1) to 147.06 (128.5-213.4) pg/ml (p = 0.016), respectively. Sclerostin and DKK-1 levels increased significantly after anti-TNF treatment from 312.4 (140.8-412.7) to 405.1 (276.3-452.5) pg/ml (p = 0.018) and 446.7 (356.9-529.3) to 881.3 (663.1-972.2) pg/ml (p 0.001), while there was no significant change in noggin level.Many inflammatory cytokines increase after anti-TNF treatment and noggin is not affected by anti-TNF treatment in AxSpA. Noggin might be a therapeutic target in patients with axSpA.• Anti-TNF therapy is not sufficient for complete blockage of the inflammatory process in axial spondyloarthritis. • The increase in IL-17, IL-22, and IL-33 may decrease the efficiency of anti-TNF therapy. • Noggin might be a therapeutic target as a complementary or alternative approach to anti-TNF therapy in axial spondyloarthritis.

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.

Background/Objective Core muscle endurance (CME), which is the ability of sustaining the activity of trunk muscles, has been shown to be lower in patients with ankylosing spondylitis (AS). The aim was to investigate the possible relationship between CME times and balance, fatigue, physical activity (PA) level, and thoracic kyphosis angle. Methods Fifty-one patients with AS with a mean age of 41.0 years (interquartile range, 25/75 years; 29.0/51.0 years) were included in the study. Core muscle endurance times were assessed by using trunk extension, trunk flexion, and side bridge tests. Overall stability index, anteroposterior stability index, mediolateral stability index, and limits of stability were evaluated with the Biodex Balance System. Fatigue and PA levels were surveyed using Fatigue Severity Scale and International Physical Activity Questionnaire, respectively. Thoracic kyphosis angle was measured by using a digital inclinometer. Additionally, CME times were compared for "high-fatigue" versus "low-fatigue" and as "low PA" versus "moderate/high PA" groups. Spearman correlation coefficients and Mann-Whitney U test were used for statistical analysis. Results Significant correlations were detected between overall stability index, anteroposterior stability index, Fatigue Severity Scale, International Physical Activity Questionnaire, and all CME tests (p < 0.05) and between mediolateral stability index and side bridge test (p < 0.05). Limits of stability correlated only with side bridge test (p < 0.05). Core muscle endurance significantly differed between high-fatigue and low-fatigue groups (p < 0.05), except trunk flexor test (p > 0.05). No significant differences were observed between low PA and moderate/high PA groups (p > 0.05), except side bridge test (p < 0.05). Conclusions Core muscle endurance times seem to be related to PA level, fatigue, and balance but not with thoracic kyphosis angle. Assessing CME in patients with AS might help in planning individualized exercise programs.

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.

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.

Aim Systemic sclerosis (SSc) is a chronic autoimmune disease of unknown etiology characterized by excessive collagen production, endothelial cell injury, microvascular obliteration, cutaneous fibrosis and progressive visceral disease. The hands are frequently involved during the progression of the disease, with symmetrical skin thickening as a prominent feature. Modified hand mobility in scleroderma (mHAMIS) test is a measurement method to assess hand mobility in patients with SSc. Knowing the inter-rater reliability of the instrument is important in order for the results from different examiners to be accurately interpreted. The aim of this study was to test inter-rater reliability of the mHAMIS test. Method Hand mobility for both hands was assessed in 25 female patients with SSc by 2 physiotherapists who have different years of experience. Patients who had flexion contracture in at least 1 finger and undergone hand surgery in the last year due to any injuries, were excluded from the study since hand mobility was prevented. Inter-rater reliability was determined using intra-class correlation coefficients (ICCs). Result The ICCs were excellent between raters for dominant and non-dominant hands. The values were 0.92 and 0.93, respectively. Conclusion The inter-rater reliability of the mHAMIS was found to be excellent. This research contributes to the literature by proving that the test can be used without causing bias in clinical trials.

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.

Angiotensin-converting enzyme (ACE)/Angiotensin (Ang) II pathway has crucial regulatory effects on circulatory hemostasis and immune responses. This pathway has a major role in the development of acute lung injury and acute respiratory distress syndrome (ARDS), which is a devastating complication of SARS-CoV-2 infection. The aim of this study is to investigate the serum ACE activity and its correlation with clinical features and the disease severity in patients with COVID-19. Patients with confirmed COVID-19 by detecting SARS-CoV-2 nucleic acid RT-PCR were included in the study. Demographic data, clinical features, laboratory and radiologic investigations were recorded. Patients were classified by disease severity; asymptomatic, mild, and severe pneumonia. The serum ACE activity was evaluated with an autoanalyzer based on a spectrophotometric method. Fifty-five patients (50.9% female) and 18 healthy subjects (33.3 % female) were enrolled in the study. The median age of patients was 40 years, ranging from 22 to 81 years. Eighteen healthy subjects were served as the control group. The baseline characteristics were comparable between groups. The median serum ACE activity of patients and controls (38.00 [IQR 21] U/L and 32.00 [IQR 24] U/L, respectively) and of between patients grouped by disease severity (38.5 [IQR 19], 36 [IQR 25], and 38 [IQR 22] U/L, asymptomatic, mild and severe pneumonia group, respectively) were similar. There was no correlation between the serum ACE activity and conventional inflammatory markers. In this study, we did not find an association between serum ACE activity and COVID-19 and serum ACE activity on admission did not reflect disease severity.

Familial Mediterranean fever (FMF) is characterized by recurrent attacks of fever, serositis, and arthritis. Some patients suffer from associated inflammatory conditions and damage related to FMF that may potentially impair work productivity which have not been studied to date.Consecutive FMF patients who were attending a tertiary referral center and age-and sex-matched healthy subjects enrolled into the study. Disease activity was assessed with autoinflammatory disease activity index (AIDAI) and patient global assessment. Damage was evaluated using Autoinflammatory Disease Damage Index (ADDI). Quality of life (QoL) and work productivity were determined with 36-Item Short Form Health Survey (SF-36) and Work Productivity and Activity Impairment Specific Health Problem v2.0 (WPAI:SHP), respectively.There were 111 FMF patients, 60 female (54%), mean age 32.7±8.7 years. There were significant impairments in all domains of the SF-36 QoL in FMF patients. Of the 111 patients enrolled, 65 (58.6%) were employed in a paid work. Mean% ±SD impairment in work productivity both assessed as absenteeism (9.3±23.2% vs. 0.7±2.6, p=0.013) and presenteeism (35.2±32.6% vs. 9.6±14.7, p0.001) were significantly higher in FMF patients compared to healthy subjects. Impairment in work productivity was correlated with the number of attacks, disease activity, colchicine resistance, and disease-associated damage. Impairment was most significant in colchicine-resistant FMF patients but lower in those on interleukin (IL)-1 antagonist treatments.FMF causes significant work impairment and reduced QoL which is associated with disease activity and damage. The use of IL-1 antagonists may help to improve work productivity and QoL in FMF patients with frequent attacks. Key points • Work productivity is impaired in patients with FMF. • Disease activity was an independent predictor for impaired work productivity. • IL-1 antagonists may improve work productivity and quality of life in FMF patients with frequent attacks.

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, …

To assess, through both objective and subjective methods, the complaints of dysphonia among adults with rheumatoid arthritis (RA). The secondary purpose of the study is to determine whether complaints of dysphonia are related to depression and disease activity.This is a prospective cohort study.Eighty subjects (38 RA and 42 healthy volunteers aged 18-65 years old) were included in the study. Participants were evaluated using the Voice Handicap Index-10 (VHI-10) to assess voice complaints. Laryngeal findings of participants with RA were performed by videolaryngoscopy. Maximum phonation time (MPT) measurements and acoustic voice analysis (PRAAT software) were performed to evaluate the presence of objective dysphonia. Disease activity of individuals was calculated by using Disease Activity Score-28 (DAS-28) scale. Beck Depression Inventory (BDI) was applied to evaluate the symptoms of depression in participants.The prevalence of laryngeal symptoms of participants with RA was %42.1. According to the cut-off score of VHI-10, 15.8% of the participants in the study group had voice complaints. Comparing the MPT and acoustic voice analyses values of the study and control group, the MPT of the RA participants were statistically lower (P0.05). Perturbation parameters of male participants in the study and control groups were statistically different. 15.8% of participants in RA group had symptoms of depression. However, there was no statistically significant difference between BDI and acoustic voice parameters.RA may be associated with voice disorders. Male patients with RA had worse jitter parameters, but the number of participants was low. Dysphonia may not be associated with depression and disease activity in RA patients.

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.

Supplemental material, sj-pdf-1-lup-10.1177_0961203320919848 for Diaphragmatic muscle thickness and diaphragmatic function are reduced in patients with systemic lupus erythematosus compared to those with primary Sjögren’s syndrome by Hasan Satış, Emetullah Cindil, Reyhan B Salman, Dilek Yapar, Esra Temel, Nur B Demir, Hakan Babaoğlu, Onur Gündoğdu, Nuh Ataş, Halit Şendur, Aslıhan Avanoğlu Güler, Hazan Karadeniz, Abdurrahman Tufan, Mehmet A Öztürk, Şeminur Haznedaroğlu and Berna Göker in Lupus

In the Wuhan Province of China, in December 2019, the novel coronavirus 2019 COVID-19 has caused a severe involvement of the lower respiratory tract leading to an acute respiratory syndrome. Subsequently, coronavirus 2 SARS-CoV-2 provoked a pandemic which is considered a life-threatening disease. The SARS-CoV-2, a family member of betacoronaviruses, possesses singlestranded positive-sense RNA with typical structural proteins, involving the envelope, membrane, nucleocapsid and spike proteins that are responsible for the viral infectivity, and nonstructural proteins. The effectual host immune response including innate and adaptive immunity against SARS-Cov-2 seems crucial to control and resolve the viral infection. However, the severity and outcome of the COVID-19 might be associated with the excessive production of proinflammatory cytokines "cytokine storm" leading to an acute respiratory distress syndrome. Regretfully, the exact pathophysiology and treatment, especially for the severe COVID-19, is still uncertain. The results of preliminary studies have shown that immune-modulatory or immune-suppressive treatments such as hydroxychloroquine, interleukin IL -6 and IL-1 antagonists, commonly used in rheumatology, might be considered as treatment choices for COVID-19, particularly in severe disease. In this review, to gain better information about appropriate anti-inflammatory treatments, mostly used in rheumatology for COVID-19, we have focused the attention on the structural features of SARS-CoV-2, the host immune response against SARS-CoV-2 and its association with the cytokine storm.

Familial Mediterranean fever is characterized by self-limited attacks of serositis and arthritis. However, substantial number of patients suffer from chronic complications of this disease, primarily involving musculoskeletal system. Treatment for these complications is challenging due to limited evidence. Interleukin-1 (IL-1) antagonists, tocilizumab and anti-tumor necrosis factor (anti-TNF) agents are off-label treatment options for the management of chronic manifestations of FMF, such as secondary (AA) amyloidosis, chronic arthritis and sacroiliitis. This paper presents a case series of four FMF patients who are refractory to IL-1 antagonists, anti-TNF agents and tocilizumab, who responded well to tofacitinib. The authors also conducted a comprehensive literature search for studies investigating tofacitinib use in FMF patients. Although still limited, current data suggest that tofacitinib could be a useful treatment option for FMF patients with associated inflammatory comorbid conditions and chronic manifestations of disease.

Familial Mediterranean fever (FMF) is characterized by recurrent short-lived/self-limiting inflammatory attacks. Besides these, a substantial number of patients with FMF present with a variety of other inflammatory diseases; however, this issue has not been systematically studied previously. Hence, we aimed to investigate the frequency of inflammatory comorbid diseases in a large FMF cohort. All patients were recruited from "FMF in Central Anatolia (FiCA) Cohort", comprising 971 (mean age 35.3 +/- 12 years, 61.5% female) adult subjects. All patients fulfilled Tel Hashomer criteria. Demographic data, FMF disease characteristics, MEFV gene mutations, and comorbid inflammatory diseases were meticulously questioned, and laboratory features and genotype data were retrieved from hospital records. There were comorbid inflammatory diseases in 205 (21.1%) patients. The most common inflammatory disease was spondyloarthritis (12.9%). Other remarkable inflammatory disorders were psoriasis, immunoglobulin A vasculitis/Henoch-Schonlein purpura, Behcet's disease and inflammatory bowel diseases. Cryptogenic organizing pneumonia is a newly defined entity in our cohort which is seemed to be associated with FMF (0.3%). Number of patients with persistent inflammation was higher in those with comorbid diseases (p < 0.001). Our results suggest that FMF is commonly associated with other inflammatory diseases. Therefore, clinicians should be cautious about comorbid inflammatory diseases in FMF patients, particularly in those with persistent inflammation. Identification of pathogenic pathways linking FMF to these diseases warrants further investigations.

Background: Despite the improvements in classifying patients with primary Sjogren’s syndrome with usage of the new 2016 ACR/EULAR classification criteria, there remains to be a need for improvement and investigating the value of new modalities in diagnosing as well as classifiying these patients. Objectives: to investigate potential role of parotid elastography in diagnosis and classification of patients with primary Sjogren’s syndrome (pSs) Methods: This is a cross sectional analysis of patients with primary Sjogren’s syndrome followed up in our out-patient Rheumatology clinic. We performed chart reviews and retrospectively investigated the available data on files to search whether or not our clinically diagnosed patients satisfied the 2002 AEG and/or 2016 ACR/EULAR criteria sets. Ultrasonographic and elastographic evaluation of parotid and submandibular glands bilaterally were performed on consecutive patients with clinical diagnosis of pSs and contributions of these findings to classification criteria sets were interpereted Results: There were 95 pSs patients and 30 healthy gender and age matched controls. Strain ratio, shearwave velocity and Pascal values of the glands were examined. Parotid strain ratio, submandibular velocity and submandibular pascal values were statistically significantly different compared to healthy controls (Table).%86 of patients considered clinically as Sjogren syndrome satisfied 2016 ACR/EULAR (criteria patients) and%84 satisfied 2002 AER classification criteria. We grouped patients with respect to parotid strain ratio taking 1.0875 as the cut-off value. Those patients who did not satisfy 2016 ACR/EULAR criteria, but clinically diagnosed as pSs (non criteria pSs patients), also had significantly higher parotid strain ratio and submandibular velocity compared to healthy controls (p:0,016 and p Conclusion: Parotid shear elastography is an easy and nonivasive method and can be a useful tool for the diagnosis and classification of patients with pSs (1). References [1] Mossel E, Delli K, van Nimwegen JF, Stel AJ, Kroese FGM, Spijkervet FKL, Vissink A, Arends S, Bootsma H; EULAR US-pSS Study Group. Ultrasonography of major salivary glands compared with parotid and labial gland biopsy and classification criteria in patients with clinically suspected primary Sjogren’s syndrome Ann Rheum Dis. 2017 Nov;76(11):1883-1889. doi: 10.1136/annrheumdis-2017-211250 Disclosure of Interests: None declared

Background Underlying causes of inflammation cannot be established sometimes, despite meticulous medical history and physical examination, laboratory tests including cultures, immunologic and serologic tests and widely used radiologic procedures such as plain x-rays, echocardiography, ultrasonography and computed tomography. Rheumatologists quite oftenly face with these patients which is termed as inflammation of unknown origin (UIO). Differential diagnosis of UIO is diverse and investigation of such cases are challenging and time consuming. Objectives: to assess diagnostic utility of PET CT in the diagnosis of patients with UIO Methods Study comprised 68 (36 male, mean age 58.7±14.8, range 19-87 years) adult IUO subjects without a previous diagnosis of an inflammatory or malignant disease. Patients were screened with PET CT after 8 hours fasting, if a specific diagnosis could not be established with comprehensive evaluation including; meticulous history and physical examination, pertinent microbiologic cultures, brucella agglutination, Mantoux test, serum protein electrophoresis, echocardiography, plain x-rays, computed tomography of thorax and abdomen/pelvis. Results Final diagnosis were established in follow up were inflammatory diseases in 37 (54.4%), malignant disorders in 16 (23.5%) and infections in 5 (7.4%), whereas a final diagnosis cannot be made in 10 (14.7%). PET CT aided diagnosis in 40 (58.8%) patients but was ineffective in 28 (41.2%). All three PET CT positive subjects with a final diagnosis of infection had tuberculosis (tb). On of two PET negative subjects had EBV and one other also had tb. PET CT was positive in 24 of 37 (64.9%) subjects with a final diagnosis of inflammatory rheumatic disease. Final inflammatory diseases were large vessel vasculitis 15, polymyalgia rheumatica 5, seronegative arthritis 4, and other rare miscellaneous diseases, such as small vessel vasculitis, inflammatory myositis, polychondritis, sarcoidosis and IgG4 related disease. PET was positive in 11 of 15 (73.3%) large vessel vasculitis patients and 2 of 5 (40%) PMR patients. Because of small number of miscellaneous rheumatic diseases, diagnostic value of PET cannot be evaluated in these. Conclusion Investigation of underlying etiology of UIO is time and effort consuming. PET CT may help to identify final diagnosis more quickly by directing an obscure inflammatory site. PET CT may also have advantages like reducing number of unnecessary biopsies, diagnostic time, anxiety, work loss, morbidity and mortality References [1] Kan Y, Wang W, Liu J, Yang J, Wang Z. Contribution of 18F-FDG PET/CT in a case-mix of fever of unknown origin and inflammation of unknown origin: a meta-analysis. Acta Radiologica. 2018:0284185118799512. Disclosure of Interests None declared