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The objective of the study is to investigate the clinical characteristics and long-term prognosis including flares and organ damage in patients with giant cell arteritis (GCA) from a tertiary referral centre and compare these features in different subgroups. In this retrospective observational study, patients with GCA who were followed up in our vasculitis clinic between 1998 and 2018 were evaluated by a predefined protocol. Patients with and without cranial symptoms were compared for clinical and laboratory features, flares and permanent damage findings. Vasculitis Damage Index and Large Vessel Vasculitis Index of Damage were used for damage assessment. Records of 89 patients (median follow-up time 46 months) were analysed; mean time to diagnosis after initial symptom was longer in patients with acute vision loss (11 ± 4 vs. 4.8 ± 1.1 months p = 0.002). EGG (n = 19) was younger (63 ± 2 vs. 69 ± 1 years old p = 0.01); had higher mean CRP (141.8 ± 107.3 vs. 76.6 ± 67.9 mg/dL p = 0.023) and ESR (120.8 ± 25.1 vs. 99.3 ± 24.3 mm/h p = 0.004) at diagnosis. PET-CT detected large vessel vasculitis in 42/48 (87.5%) cases of the entire cohort. Thirty-one patients had flares and proportion of flared patients was significantly higher in patients with cranial symptoms. At least one damage item (DI) was present in 54 (60.7%) patients. The development of damage was found to be associated with flares. Evaluation of our cohort revealed the importance of early diagnosis for prevention of vision loss in GCA. Patients without cranial symptoms were younger, present with higher inflammatory response and for these, PET-CT was the main diagnostic tool. Relapse rate was higher in patients with cranial symptoms. Flares and accompanying corticosteroid treatment may contribute to organ damage in GCA.

Objective.Major salivary gland ultrasonography (SGUS) is a widely used imaging technique to evaluate salivary gland involvement in primary Sjögren syndrome (pSS). The aim of this study was to evaluate the relationship between SGUS, salivary flow rate (SFR) as an objective measure of the gland function, and oral health-related quality of life (OHRQOL) as a patient-reported outcome measure (PROM) in a pSS cohort.Methods.Sixty-six patients with pSS were examined by SGUS according to Hocevar and Milic scoring systems. Patients with inhomogeneity/hypoechoic areas with scores ≥ 2 in parotid and submandibular glands were classified separately as “severe glandular involvement.” Further, oral health, SFR, and Oral Health Impact Profile-14 (OHIP-14) for OHRQOL were assessed.ResultsBoth total Hocevar and Milic scores were higher in 21 pSS patients with low unstimulated whole salivary flow rate (U-WSFR) than 45 pSS patients without low U-WSFR (P = 0.001 and P < 0.0001, respectively). Increased scores of homogeneity, hypoechoic areas and glandular border visibility were observed in patients with low U-WSFR (P < 0.05). Among these variables, homogeneity score was found to be an independent risk factor for low U-WSFR in pSS according to logistic regression analysis (OR 1.586, P = 0.001). Moreover, a higher OHIP-14 score was observed in severe parotid involvement compared to nonsevere cases (23.26 ± 21.19 vs 8.32 ± 13.82, P = 0.004).Conclusion.High Milic and Hocevar SGUS scores are associated with reduced SFR and poor OHRQOL as a PROM. The inhomogeneity component of the SGUS score is associated with low U-WSFR and is an indicator of severely affected gland function.

We aimed to analyse the distribution of HLA Class 2 genotypes which were reported among the genetic risk factors for ANCA-associated vasculitis (AAV) among Turkish patients in comparison with healthy subjects and previously reported data of AAV cohorts. Ninety-eight patients (F/M: 47/51 and mean age: 49 ± 1.14) were enrolled in the study and records of gender and birthplace-matched 196 healthy kidney donors were used as the control group. Patients were classified according to the clinical subgroups and ANCA serotypes (MPO-AAV, PR3-AAV). DNA was isolated from venous blood from all patients, and high-resolution HLA Class 2 genotyping was carried out by using NGS-Omixon Holotype HLA Kit. The frequencies of HLA-DQB1*03:03, - *06:04, and -DPB1*13:01, -*16:01 and -*66:01:00 alleles were significantly higher, and the frequencies of HLA-DQB1*02:02, -DPB1*02:01 and -*04:01 alleles were lower in the PR3-AAV subgroup (n = 53) compared to the controls. Comparison of amino acid sequences of the associated HLA-DPB1 alleles revealed the sequence of D-E-A-V in risk alleles replaced with the G-G-P-M sequence in protective alleles between 84 and 87th positions. Structural analysis of the HLA-DPB1*02:01 showed that this shared position is in the contact area between HLA-DP α and β chains and within pocket 1 of the antigen-binding groove. First HLA genotyping analysis in Turkish AAV patients revealed a negative correlation between PR3-ANCA positivity and certain HLA-DPB1 alleles contradictory to the results reported from European cohorts. Known functional effects of D-E-A-V sequence on HLA-DPB1 support the importance of our finding, but further studies are needed to reveal its pathogenic mechanisms.

Background:The negative impact of COVID-19 in patients with ANCA associated vasculitis (AAV) and patients on rituximab (RTX) treatment have been reported (1). Risk factors for severe course of COVID-19 and increased mortality in these patients are unclear.Objectives:To evaluate the course of COVID-19 in our AAV cohort and identifying risk factors for mortality.Methods:Patients with AAV who were classified according to CHCC and whose scheduled last visit were after December 2019 were screened and evaluated for COVID-19 either by phone call or in the clinic. Records of patients with a history of hospital admission due to COVID-19 were evaluated. Cumulative clinical findings and treatment history were noted. Hypogammaglobulinemia (hIgG) was defined as IgG level below 700 mg/dl. All inpatients with a diagnosis of COVID-19 were screened for hIgG and IVIG was administered if necessary.Results:Eighty-nine patients (47.2% female, mean age 56 + 12.5 (28-81)) were included into the study. The diagnosis was GPA in 56 (62.9%) and MPA in 33 (37.1%) patients. Mean follow up time was 91 + 53.4 (26-272) months. Anti-PR3 and anti-MPO were positive in 46 (51.7%) and 32 (35.9%) patients, respectively. Lower respiratory tract (LRT) involvement was present in 72 (80.9%) and 10 patients had a history of diffuse alveolar haemorrhage (DAH). Sixty-one patients (68.2%) had a history of rapidly progressive glomerulonephritis (RPGN) and 21 (23.6%) had peripheral nervous system (PNS) involvement.Fifteen (16.9%) patients had COVID-19; 14 of them were PCR positive, one patient had symptoms and thorax CT findings compatible with COVID-19. Pulmonary infiltrates were observed in 13 patients (86.7%); 9 (60%) had severe pneumonia. Twelve patients (85.7%) were hospitalized, 6 patients (42.9%) needed ICU admission and 5 patients (35.7%) died. Tocilizumab and anakinra for hyperinflammation during COVID-19 were used in 1 (6.7%) and 4 (26.7%) patients, respectively.Four out of five deceased patients (3 on RTX treatment, 1 with renal transplant) were in remission at the time of COVID-19. COVID-19 was detected in a patient with disease flare and DAH, during treatment with high dose steroids and plasmapheresis. hIgG was detected in all deceased patients from COVID-19 during hospital admission (mean IgG: 495±113.2 mg/dL).Symptomatic COVID-19 was more frequent in patients with a history of DAH, RPGN and hIgG. hIgG during the follow-up was significantly associated with COVID-19 in multivariable analysis (p=0.01, OR=20,6 %95 CI (2-210). Comparison of patients who died of COVID-19 and survived showed that female sex, PNS involvement and hIgG during the clinical course and hospital admission were risk factors for increased mortality (Table 1). Age, smoking, treatments, history of flares or serious infections, remission status and chronic renal insufficiency did not differ between groups.Conclusion:The frequency and mortality from COVID-19 is found to be high in our AAV cohort compared to previous reports (1). Patients with serious lung or renal involvement are prone to symptomatic COVID-19. Previously reported severe outcomes on RTX therapy might be related to consequent hIgG. High dose IVIG treatment may not be sufficient in improving survival in AAV patients with severe COVID-19 and hIgG.References:[1]Severity of COVID-19 and survival in patients with rheumatic and inflammatory diseases: data from the French RMD COVID-19 cohort of 694 patients. 2020:annrheumdis-2020-218310.Table 1.Comparison of risk factors for CI and mortality in patients with AAVCOVID-19 (n=15)Non-infected (n=74)pORDeath (n=5)Survive (n=10)p2OR2Age53.4±11.956.6±12.6NS51.2±12.654.6±12.1NSSex (female)635NS420.03614 (0.9-207)LRT Involvement1458NS59NSDAH460.0384.1 (1-16.9)13NSRPGN15460.0048.5 (1-68.4)57NSPNS involvement318NS300.0059 (1.4 - 57)RTX treatment1033NS37NShIgG in outpatient visits670.026.3 (1.8-23.3)420.0216(1.5-234)hIgG during hospitalization due to CI----540.0252.5 (1.2-5.4)Flares≥1725NS43NSChronic Renal Insufficiency722NS43NSDisclosure of Interests:None declared

Background:Infection is a remarkable cause of morbidity and mortality in patients with SLE.Objectives:We aimed to determine the clinical course of COVID-19 infection in our patients with SLE and the factors affecting this courseMethods:SLE patients (2012 SLICC criteria) diagnosed with COVID-19 infection by a positive PCR test and/or typical findings of lung involvement in CT (computed tomography) imaging were included. Data regarding cumulative clinical and laboratory characteristics, histopathology results, autoantibody profiles, immunsuppressives and damage (SLICC damage index/SDI)) were retrieved from the existing database and revised. SLE Disease Activity Index (SLEDAI-2K) was determined at the time of infection.Results:Sixteen SLE patients with COVID-19 infection were identified. Most (87.5%) of these patients were female. Seventy % (n=11) had lupus nephritis. Twenty-five % had thrombotic antiphospholipid syndrome.PCR was positive in 70% (n=11) of the patients. Pulmonary parenchymal findings compatible with COVID-19 were observed in 56% (n=9) of those patients. Regarding complaints upon admission, 50% (n=8) had fever, 44% (n=7) cough, 44% (n=7) dyspnea, 19% (n=3) myalgia, 12.5% (n=2) headache, 12.5% (n=2) nausea /vomiting, 6% (n=1) diarrhea, and 6 % (n=1) had anosmia. Eight patients were hospitalized. Six of these patients needed oxygen therapy via nasal cannula. None needed a follow-up in the intensive care unit. The mean hospitalization duration was 14 ± 5 (8-25) days.Regarding disease activity at the time of infection, 9 had inactive disease with a SLEDAI-2K score of 0 whilst in 5 patients SLEDA-2K score was ≥4. The mean SLEDAI-2K score at the time of infection was 1.7 ± 2.3 (0-6). System/organwise, 1 patient with chronic thrombocytopenia presented with a worsening platelet count accompanied by serologic activity. This patient was a non-adherent to treatment who had stopped taking mycophenolic acid months before COVID19. Three patients 2 of whom had proliferative nephritis experienced nephritic flares.1 patient who had a history of cutaneous lupus and was in remission presented with oral ulcer, leukopenia and hypocomplementemia during infection. Of 16 patients, 7 had system damage at the time of infection. The mean SDI score of the patients was 1.4±1.8. Comparison of patients with and without damage revealed no significant differences in disease activity, symptoms associated with COVID, in the need for hospitalization, hospitalization duration, and the requirement for oxygen therapy. However,CT findings compatible with COVID19, were more common in patients with damage (87% vs.33%,p=0.04) and their mean CRP levels were higher at diagnosis (65 ± 47 vs.22 ± 48 mg/l;p=0.032).All patients received similar treatment for COVID-19 except active patients who required high dose steroids (2 with active renal, 1 with thrombocytopenia and 1 with oral ulcer, leukopenia and hypocomplementemia).The patient with thrombocytopenia also received intravenous immunoglobulin and 1 with cutaneous active disease received tocilizumab as she developed macrophage activation syndrome. Six patients (37.5%) had received rituximab (RTX) in the last 6 months before COVID. No significant difference, in terms of hospitalization and need for oxygen therapy due to COVID19 was found between patients who had received RTX vs who had not. No hypogammaglobulinemia was detected in patients who received RTX despite lower levels of IgG (998 ± 184 vs 1481± 51 mg/dl, p=0.02)Conclusion:Although half of the patients in our series of COVID19 infected SLE patients required hospitalization, there were no mortalities. More patients with damage (none pulmonary) displayed CT findings compatible with COVID19 and further follow up will reveal whether they will suffer from fibrotic lung disease. Patients can experience disease flares during COVID. But it is also important to consider that some manifestations such as thrombocytopenia may also be a sign of severe infection. Immunosupressive agents may not have a negative impact on the course of infection.*the first two authors contributed equallyDisclosure of Interests:None declared.

Background:Retinol binding protein 4 (RBP4) is a plasma retinol transporter that transports retinol from liver to periphery. RBP4 has been studied as a biomarker in metabolic and neoplastic conditions, however its association with inflammation is not clear. Serum amyloid A (SAA), another retinol binding protein, has been known as a sensitive biomarker of inflammation in familial Mediterranean fever (FMF) and other autoinflammatory disorders. C-reactive protein (CRP), erythrocyte sedimentation rate (ESR) and SAA are commonly used as acute phase reactants, but they are not successful in differentiating non-infectious inflammatory conditions from infections.Objectives:We aimed to evaluate the potential of serum RBP4 as a biomarker of acute phase response and to determine its performance in differentiation of inflammation of patients with FMF and AA amyloidosis from infections.Methods:A total of 169 participants in 5 groups, consisting of FMF (n = 60), FMF with AA amyloidosis (n = 58), non-FMF AA amyloidosis (n = 23), infections (n = 10, 3 pneumonia, 3 sepsis, 1 pyelonephritis, 1 fungal infection, 1 cellulitis, 1 disseminated zoster), and healthy controls (HC) (n = 18), were included and evaluated cross sectionally. Hemogram and serum CRP, ESR, SAA, ferritin, creatinine, AST, ALT, albumin levels were recorded from the patient charts. FMF and FMF + amyloidosis patients were evaluated during attack-free period. Serum RBP4 levels were investigated by ELISA (Elabscience, USA). Mean values and relative changes compared to healthy controls were evaluated for SAA, CRP, RBP4 levels in all groups.Results:Serum RBP4 level was found to be higher in FMF group compared to the patients with infection (p = 0.002) and HC (p Table 1.Demographic features and laboratory findings of the participantsVariablesFMF(n=60)FMF- Amyloidosis(n=58)Non-FMF-AA Amyloidosis(n=23)Infection(n=10)Healthy control(n=18)Female/Male46/1433/258/153/78/10Age (SD)*38±13(18-74)43±11(21-69)53±1365±1533±9Creatinine (mg/dL)*0,8±0,21,7±1,72,0±1,61,7±1,00,7±0,2Albumin(mg/dL)*4,7±0,44,3±0,63,3±0,93,0±0,94,8±0,2Ferritin (ng/mL)*70±94245±315139±168554±3883±72RBP4 (ng/mL)*772±183671±214666±256512±204524±117RBP4 (median)770(434-1142)653(227-1259)645(331-1214)487(226-876)498(566-738)CRP (mg/L)*16±47,112,8±32,825,7±36,469±36,81,2±1,2SAA (mg/dL)*10,3±31,45,0±13,97,1±14,140,2±18,50,3±0,1ESR*15±1319±1641±2945±427±5Relative RBP4 increase1,47±0,351,28±0,411,27±0,490,98±0,39Relative CRP increase13,4±39,210,6±27,321,4±30,357,7±30,6Relative SAA increase34,5±104,816,0±45,723,7±47,1133,9±61,7*mean, RBP4 (Retinol Binding Protein 4), C-Reactive Protein (CRP), Erythrocyte Sedimentation Rate (ESR), Serum Amyloid A (SAA).Conclusion:This preliminary study showed that RBP4 levels may be increased about 1.5 times in FMF and to lesser extent in AA amyloidosis patients despite no significant change during acute phase response of different infections. Patients with infections show strong CRP and SAA response, and the differential response of RBP4 in FMF patients warrants further analysis in larger group of patients with different clinical characteristics.Disclosure of Interests:None declared

Background:COVID-19 runs a variable course resulting in acute respiratory distress syndrome and death in a subset of patients. The entry of SARS-CoV-2 into the cell stimulates innate immunity including NLRP3 inflammasome and lead to development of adaptive immunity later. Hyperinflammatory response with the release of proinflammatory cytokines including IL-1β and IL-6 results in cytokine storm in some patients with a worse outcome. Colchicine acts on NLRP3 inflammasome and inhibits and IL-1 mediated inflammatory attacks in gout and familial Mediterranean fever (FMF) patients. Patients with inadequate response to colchicine may benefit from anti-IL-1 biologic agents such as anakinra and canakinumab. Recently, favourable effects of anakinra have been observed in COVID-19 patients with findings of cytokine storm.Objectives:We aimed to evaluate the impact of COVID-19 among refractory FMF patients followed-up in tertiary referral with the treatment of biologic agents and also document the course of COVID-19 in these patients.Methods:We searched out database of FMF patients to identify those using biologic agents (anti-IL-1, anti-IL-6 or anti-TNF) for colchicine-refractory FMF. We interviewed the patients using a standard questionnaire by phone call for symptomatic COVID-19 and evaluated those patients who described findings of COVID-19 further by their hospital records or inviting them to the hospital for additional investigations.Results:We identified 183 patients and contacted 106 of them by phone in May-October 2020. A history of symptomatic COVID-19 was documented in 7 FMF patients who were on a biologic agent. Six were on anti-IL-1 and one was on anti-TNF, and one of the patients was not taking his biologic agents for 1 year. All of 7 patients had a favourable outcome. All but 1 patient followed at home and none of them developed findings of cytokine storm, thromboembolism and secondary bacterial infection. Hospitalized patient did not require intensive care unit (ICU) support or mechanical ventilation, and he was not given additional anti-inflammatory medications.Conclusion:This series of refractory FMF patients with potentially higher inflammatory characteristics showed COVID-19 did not result in a worse outcome in those patients during the first phase of the pandemic, and none developed findings of cytokine storm. Observations in these patients supports further that biologic agents blocking IL-1 and possibly TNF may contribute to the uneventful course of COVID-19 by preventing the development of hyperinflammatory response. Data collection from a larger group of patients, especially those with amyloidosis, will clarify the protective effects of colchicine and contribution of anti-IL-1 treatments on the favourable disease course during the second phase of the pandemic.Patient 1Patient 2Patient 3Patient 4Patient 5Patient 6Patient 7Age (years)45483953323731MEFV variantsUnknownM694V/M680IUnknownM694V/ M694VM694V/ M694VM694V/ M694VM694V/ M694VAmyloidosisNoNoNoNoNoNoNoBiologic agentsAnakinra100 mg/dayNot takenfor 1 yearAdalimu-mabCanakinumab150 mg/monthAnakinra100 mg/dayCanakinumab150 mg/monthAnakinra100 mg/dayPrednisone (mg/day)5NoNoNoNoNoNoColchicine(mg/day)21,51,51,51,52,02,0RT-PCR positivityYesYesYesYesYesYesYesChest CT signsYesYesNot doneNot doneNot doneNot doneNot doneHospitalisationNoNoNoYesNoNoNoAntiviral therapyOseltamivirOseltamivirNoFavipravirFavipravirFavipravirFavipravirHCQ useYesYesYesYesNoNoNoNew FMF attackduring COVID-19NoNoNoNoNoNoNoDisclosure of Interests:None declared

Background:Positron emission tomography (PET/CT) has gained importance in the diagnosis and assessment of large vessel vasculitis (LVV) recently.Objectives:We aimed to investigate the diagnostic importance and clinical significance of PET/CT findings in giant cell arteritis (GCA).Methods:Data of the patients who underwent PET/CT to investigate large vessel involvement and who had at least 6 months of follow-up with a clinical diagnosis of GCA were retrospectively evaluated. PET/CT images were assessed by an experienced nuclear medicine specialist, regions of interest were drawn for major vascular territories and standardized maximum uptake values (SUVmax) of these areas were recorded.Results:Twenty-nine consecutive patients (median age 68 (50-83), mean follow-up time 37.1 ± 48.8 (6-242)) were included into the study. All patients were over 50 years old and had erythrocyte sedimentation rate (ESR) over 50 mm/h at the time of imaging. Twenty patients (68.9%) met the ACR 1990 Classification criteria (ACR (+) group). The number of patients who had hypermetabolism in the aorta and its major branches in favour of LVV in PET/CT was 23 (79.3%) (PET-CT (+) group). Thoracic and abdominal aorta involvement were detected in 22 (75.8%) and 16 (55.2%) patients, respectively. There was positive correlation between SUVmax in thoracic and abdominal aorta on PET/CT and ESR at diagnosis (r = 0.63 p = 0.002 and r = 0.77 p Conclusion:PET/CT is increasingly used in the diagnosis and assessment of GCA in our center. The level of FDG uptake of the vessel wall in PET/CT correlates with the acute phase response. Flare was rarely observed in PET/CT (+) patients at diagnosis. Axillary artery involvement detected on PET/CT may be associated with the classical GCA clinic in ACR(+) patients (1). PET/CT (+) patients who does not met ACR criteria seems to have a diverse clinic features like young age and rare presence of PMR. PET/CT findings may be helpful in recognizing subgroups and predicting prognosis of GCA although prospective studies with follow-up scans are warranted.References:[1]Grayson PC, Maksimowicz-McKinnon K, Clark TM, Tomasson G, Cuthbertson D, Carette S, et al. Distribution of arterial lesions in Takayasu’s arteritis and giant cell arteritis. Annals of the rheumatic diseases. 2012;71(8):1329-34.Table 1.Comparison of patients who fulfilled and not fulfilled ACR 1990 classification criteria among PET/CT (+) patients.ACR (+) PET/CT (+)(n=14)ACR (-) PET/CT (+)(n=9)pOR (%95 CI)Age at diagnosis68,8±4,563.3±9,20.004PMR1020.0212.5 (1 – 6.1)History of flare41NSCRP at diagnosis75,1±30,6130,8±93,40.024ESR at diagnosis93,9±28,1112,5±21,2NSBrachiocephalic artery96NSRight subclavian85NSLef subclavian95NSRight carotid85NSLeft carotid96NSRight axillary700.0112 (1.18 – 3.3)Bilateral axillary600.0221.75 (1.1-2.7)Thoracic aorta SUVmax (mean)3,9±1,14,6±1,3NSAbdominal aorta SUVmax (mean)4,5±1,25,3±1,8NSDisclosure of Interests:None declared

Background:The prevalence of SLE pulmonary involvement varies depending on several factors, including diagnostic methods [1].Objectives:We aimed to determine the frequency of involvement with different diagnostic methods in a single center cohort.Methods:300 SLE patients were included. Chest x-ray (CXR), lung spirometry, carbonmonoxide diffusion test (DLCOc) and echocardiography were performed. High resolution thorax computed tomography (HRCT) was done for a definite diagnosis of interstitial lung disease (ILD) whilst diagram electromyography (EMG), ultrasonography (USG) and magnetic resonance imaging (MR) were utilized to diagnose shrinking lung syndrome (SLS).Results:The mean age and follow-up time were 43 and 11,5 years respectively. Of 300 patients, 16% had ILD, 6,7% had pulmonary hypertension (PHT), 3% had SLS, 0,3% had pulmonary infarction. At the start of the study, patients’ records showed that 4% had ILD, 5% PHT, 0,3% SLS and 0,3% pulmonary infarction. The median age, mean duration of disease and follow-up time were significantly higher and longer in patients with ILD compared to patients without (pConclusion:Interstitial lung disease is common in patients with SLE and considerable number of patients have SLS [2]. Spirometry, DLCOc and CXR are simple but valuable to diagnose pulmonary involvement in SLE patients. Diaphragm MR, USG and EMG are complementary methods for definite diagnosis in SLS [2]. Considering the significant difference of prevalence between the start and the end of the study, one of the possibbilities is the underrecognition of SLE pulmonary disease due to its being part of a multisystemic presentation. Higher usage of immunosuppressives in these patients may support a multisystemic active disease. Although drug effect is another concern, it is hard to establish a causal relationship due to the study’s cross-sectional design. HCQ may have a role in ILD prevention.References:[1]Keane MP, Lynch JP. Pleuropulmonary manifestations of systemic lupus erythematosus. Thorax 2000;55:159-166.[2]Singh R, Huang W, Menon Y, Espinoza LR. Shrinking lung syndrome in systemic lupus erythematosus and Sjogren’s syndrome. J Clin Rheumatol. 2002 Dec;8(6):340-5.Table 1.Spirometry; DLCO; diaphragm EMG, USG and MRI results of patients with SLS.Patient/Age/Sex1/44/F2/57/F3/39/F4/38/M5/23/F6/60/F7/58/F8/37/F9/66/F10/28/FFEV1 (%)47655963676271537039FVC (%)56735962797072556237DLCO (%)45504465535547656245TLC (%)61716566786454636245USG deep inspiration (L)4,704,742,593,013,063,065,734,172,121,59USG deep inspiration (R)3,721,982,262,242,772,174,672,834,622,49USG diaphragm thickness (L)4,823,002,482,842,551,592,093,181,621,44USG diaphragm thickness R(R)1,231,041,841,802,191,311,791,972,081,63MR high sideRRRRRRRLLMR height difference4,926,192,872,641,672,451,730,950,78EMG resting AMP (R)0,20,30,60,50,60,40,10,80,90,7EMG resting LAT (R)76,47,166,786,154,7565,8EMG resting AMP (L)0,40,50,80,71,20,60,31,20,50,5EMG resting LAT (L)6,355,756,855,66,05664,355,26,15Disclosure of Interests:None declared

Background:Pulmonary arterial hypertension (PAH) is a severe vasculopathic complication for systemic sclerosis (SSc) patients. The availability of oral-specific vasodilator therapies has provided better outcomes.Objectives:We aimed to analyze the characteristics of SSc-PAH patients and factors associated with mortality.Methods:Medical records of 291 SSc patients fulfilling ACR/EULAR criteria (2013) and followed–up during 2008-2020 years were screened and the patients who diagnosed PAH evaluated by right heart catheterization (mean PAB≥20 mmHg) (n=26, 8,9%) were included into this retrospective cross-sectional study.Results:The characteristics of 26 SSc-PAH(24 females) patients were summarised in Table 1.Table 1.Characteristics of SSc patients with PAHSSc-PAH(n=26)DemographicsAge(yrs)53.6±8.9Duration of Raynaud’s(yrs)16.1±11.8Duration of Non-Raynaud’s(yrs)9.2±6.6Raynaud’s to Diagnosis of PAH(yrs)10.8±6.7Clinical Characteristics (%)LcSSc9 (34.6)DcSSc18 (65.4)Digital ulcer14 (53.8)Gastrointestinal20 (69.0)Synovitis5(19.2)Flex contractures7 (26.9)Tendon friction rubs3(11.5)Renal crisis1 (3.8)Pulmonary fibrosis19 (73.1)Auto-antibodies (%)ANA23 (88.5)Anti-centromere6(23.1)Anti-Scl7013(50)Treatment (%)Specific vasodilatorERA (bosentan/macicentan/ambricentan)15 (51.7)PDE5-i (sildenafil/tadalafil)17 (58.6)Prostacyclin-analog (İloprost/treprostenil/selexipag)15 (51.7) Riociguat5 (17.2)Immunosuppressives21(80.8)Steroids16(61.5)Twenty-four (92,3%) of the SSc-PAH patients had PAH-related symptoms at the time of diagnosis, 2 (7,7%) were asymptomatic and diagnosed by screening. RHC and treatment details were stated in Table 2.Table 2.Right Heart Catheterization (RHC) ve treatment details of SSc-PAH patientsn=26Initial RHC -mean PAB30,4±7,9 (median 28, 20-53mmHg) -PVR5,1±2,4 (median 4, 3-9 woods) -PCWP10,8±5,5 (median 10, 0-15 mmHg)Initial treatment -monotherapy5 (19,2%) -combination10 (38,5%) -add-on combination12 (46,2%)Eleven out of 26 patients (42,3 %) were deceased after a mean follow up of 43,7±24,6 (median 48,1-84) from PAH diagnosis and 15,1±9,9 (median 13, 0,6-34) years after SSc diagnosis. Deceased patients were younger and had younger age at disease onset (49,1± 8,8 vs 56,9±7,7, p=0.032 and 30,8±13,0 vs 42.0 ±10,9, 0.027). All deceased patients had associated pulmonary fibrosis (100 vs 53.3%, p=0.01). No significant difference was observed for initial RHC parameters between deceased and survived SSc-PAH patients. Specific monotherapy was found to be more frequent in deceased patients (45,5 vs 0%, p=0,007).Conclusion:The prevalence of SSc-PAH was found to be 8.9% with increased mortality in our cohort. SSc-PAH patients predominantly had diffuse cutaneous involvement with digital vasculopathy, pulmonary fibrosis, and anti-Scl70 positivity. PAH was diagnosed after a median of 10 years of the Raynaud’s, mainly in symptomatic patients. Mortality in PAH-SSc patients was associated with early onset of disease, pulmonary fibrosis, and monotherapy. Initial RHC parameters were not found to be related to mortality.Disclosure of Interests:None declared.

Background:Psoriatic arthritis (PsA) is a heterogeneous disease and GRAPPA have proposed Minimal disease activity (MDA) as a composite outcome measure and has been validated in PsA.Objectives:In this study, we aimed to evaluate the characteristics, MDA frequencies, first biological disease modifying antirheumatic drugs (b-DMARD) continuation rate and associated factors in our PsA cohort.Methods:PsA patients who fulfilled the CASPAR classification criteria and had at least six months of follow-up data were evaluated cross-sectionally for MDA.Clinical data were collected from patient charts with standard forms.b-DMARD treatment was initiated in patients who did not respond to at least one conventional synthetic (cs) DMARD for at least three months. Only anti-TNFs were used as a first line b-DMARD therefore secukinumab (Secu) was used after first line b-DMARD treatment. Adalimumab, certolizumab, etanercept, golimumab were grouped as subcutaneous (s.c) anti-TNFs. MDA was defined as meeting five out of seven criteria during follow-up [1].Results:One hundred seventy-two patients (61% female) were included into the analysis. The mean follow-up time was 105.4±76 (6-444) months and the mean age was 50.2±13.3 (16-81) years. Mean age of onset for PsA was 38±11.9 (11-79) years; mean PsA and PsO duration were 140±90.7 (7.9-528) and 253±138 (0-756) months, respectively. Methotrexate was the most commonly used (88 %) cs-DMARD and biological DMARDs were used in 74 patients (43.3%)Overall, 95 patients (55.2 %) were observed at MDA which was significantly lower in b-DMARD users compared to only cs-DMARD users (45.9 % vs 61.9 %; p=0.038, OR: 4.3). MDA did not differ according to PsA subtypes. The addition of cs-DMARD treatment to b-DMARDs did not affect MDA frequency In univariate analysis; higher MDA frequency was associated with older age (p=0.002), longer PsO duration (p=0.036), late onset of PsA (p=0.007) and continuation of first b-DMARD (OR:13.9 pConclusion:Although frequency of MDA in our cohort was consistent with previous reports, a significant number of patients could not achieve MDA. Frequency of MDA was found to be lower in b-DMARD users compared to cs-DMARD users, possibly resulted from initiation of b-DMARD in patients with higher disease activity. Higher MDA rate was associated with higher continuation rate at first line b-DMARD treatment (TNF-inhibitor) and decreased gradually after b-DMARD switches. Although combined use of cs-DMARD with b-DMARDs did not increase the frequency of MDA, it was associated with higher b-DMARD retention. MDA is a useful outcome measure in daily follow-up of PsA patients and the importance of reaching sustained MDA for prognosis should be investigated further.References:[1]Coates, L.C., J. Fransen, and P.S. Helliwell, Defining minimal disease activity in psoriatic arthritis: a proposed objective target for treatment. Ann Rheum Dis, 2010. 69(1): p. 48-53.Table 1.b-DMARD responses, continuation rate and frequency of achieving MDA in patients with PsAb-DMARD treatmentMean (median) duration (month)Continuation rate n, (%)Primary inefficacy n, (%)Secondary inefficacy n, (%)MDAn, (%)First line b-DMARD (n=74)50.4 (36)37 (50)9 (24.3)17 (46)34 (45.9) *s.c TNF inhibitors (n=62; 83.8 %)50.8 (35.5)32 (51.7)8 (26.7)9 (30)31 (50) Infliximab (n=12; 16.2 %)13.8 (11)3 (25)1 (11.1)7 (77.8)3 (25)Second line b-DMARD (n=29)28.4 (13.5)15 (51.7)5 (35.7)3 (21.4)8 (27.6) *s.c TNF inhibitors (n=22; 75.9 %)28.6 (15)11 (50)4 (36.4)2 (18.2)5 (22.7) Infliximab (n=5; 17.2 %)35.2 (36)3 (60)--2 (40) Secukinumab (n=2; 6.9 %)9 (9)1 (50)1 (50)-1 (50)s.c:subcutaneousFigure 1.Comparison of b-DMARD retention according to MDA status in patients with ongoing first line b-DMARD treatment Log rank: p=0.001Disclosure of Interests:None declared

Background:Systemic AA amyloidosis is a serious and life-threatening complication of chronic inflammatory diseases such as rheumatoid arthritis, spondyloarthritis (SpA), and periodic fever syndromes. While most common cause of AA amyloidosis is Familial Mediterranean Fever; Ankylosing Spondylitis (AS) is another frequent cause of AA amyloidosis in Turkey.Objectives:We aimed to evaluate the response of secukinumab (SEC) treatment in three patients with AS and AA amyloidosis (AS-AA) in our tertiary referral centre.Methods:We retrospectively evaluated three AA amyloidosis patients who fulfilled Modified New York Criteria for diagnosis of AS in our AA amyloidosis cohort with 163 patients. Diagnosis of AA amyloidosis was confirmed by Congo red stain and by monoclonal AA-specific antibodies.Results:Patient 1: 61-year-old male patient with inflammatory back pain (IBP) and peripheral arthritis for 14 years was evaluated in our clinic. After methotrexate (MTX) failure, he used adalimumab (ADA), etanercept (ETA) and certolizumab (CZP). Nephrotic range proteinuria was detected when he was on CZP, and rectum biopsy documented AA amyloidosis 3 years ago. After the diagnosis, CZP treatment was switched to infliximab (IFX). IFX was ineffective in controlling inflammatory findings. SEC was started 15 months ago and he responded partially. The dose of SEC was increased to 300 mg monthly, which resulted in a sustained improvement in clinical and laboratory findings.Patient 2: 69-year-old woman was admitted to our clinic with peripheral arthritis in addition to the history of IBP for 19 years in 2005. MTX, NSAID and prednisolone were started. Because of inefficacy to conventional treatments and development of nephrotic range proteinuria, ETA was added to treatment. The patient responded to ETA and was followed-up for 13 years without symptoms of AS and proteinuria. ETA was switched to IFX due to secondary inefficacy two years ago. On the third month of IFX treatment, she developed demyelinating polyneuropathy. IFX treatment was switched to SEC and she is still being followed-up on SEC without any findings of AS and proteinuria.Patient 3: 49-year-old woman who was on sulphasalasine for 24 years for treatment of ulcerative colitis (UC) was evaluated for recent onset IBP and peripheral arthritis in 2007. After failure of MTX, she started to receive IFX. She did not respond to first IFX and then ADA and CZP, and she developed nephrotic range proteinuria when she was on anti-TNF. Her serum creatinine increased progressively, and haemodialysis (H/D) was started six months later. Due to ongoing IBP and elevated acute phase response with CZP treatment, SEC was started. Significant improvement was observed in both clinical and laboratory findings with no worsening of UC.Table 1.Clinical characteristics, laboratory findings and treatment responses of patientsPatient 1Patient 2Patient 3Age (year)616949SexMaleFemaleFemaleAge of AS onset (year)473525Age of amyloidosis diagnosis (year)575046Amyloidosis duration (months)4216830 Family historyNoAS and amyloidosis in siblingsNoHLA-B 27 statusPositivePositiveNot available MEFV statusNegativeNegativeM694V heterozygousOrgan involvement of amyloidosisGIS and kidneyKidney, liver, heart, bone marrowGIS, kidneySecukinumab duration (months)151824 CRP (mg/L) Before271523 After2.44.52.8Creatinine (mg/dl) Before0.51.6H/D After0.51.3H/DProteinuria (g/day) Before4.25.5H/DAfter2.40.9H/DAlbumin (g/dL)Before2.72.53.1After3.14.34.2ASDASBefore4.11.64.6After1.81.11.7GIS: Gastrointestinal System, ASDAS: Ankylosing Spondylitis Disease Activity ScoreConclusion:AA amyloidosis is a rare complication of SpA, and SEC treatment was found to be safe and effective in our three patients with AS-AA. Although anti-TNF agents have previously used successfully in treatment of AS-AA, SEC may be a new option especially in patients who are resistant or intolerant to anti-TNFs.Disclosure of Interests:None declared

Background:Recently developed EULAR/ACR classification criteria for systemic lupus erythematosus (SLE) have important differences compared to the 2012 Systemic Lupus International Collaborating Clinics (SLICC) SLE classification criteria and the revised 1997 American College of Rheumatology (ACR) criteria: The obligatory entry criterion of antinuclear antibody (ANA) positivity is introduced and a “weighted” approach is used1. Sensitivity and specificity of these three criteria have been debated and may vary in different populations and clinical settings.Objectives:We aim to compare the performances of three criteria sets/rules in a large cohort of patients and relevant diseased controls from a reference center with dedicated clinics for SLE and other autoimmune/inflammatory connective tissue diseases from Turkey.Methods:We reviewed the medical records of SLE patients and diseased controls for clinical and laboratory features relevant to all sets of criteria. Criteria sets/rules were analysed based on sensitivity, positive predictive value, specificity and negative predictive value, using clinical diagnosis with at least 6 months of follow-up as the gold standard. A subgroup analysis was performed in ANA positive patients for both SLE patients and diseased controls. SLE patients that did not fulfil 2012 SLICC criteria and 2019 EULAR/ACR criteria and diseased controls that fulfilled these criteria were evaluated.Results:A total of 392 SLE patients and 294 non-SLE diseased controls (48 undifferentiated connective tissue disease, 51 Sjögren’s syndrome, 43 idiopathic inflammatory myopathy, 50 systemic sclerosis, 52 primary antiphospholipid syndrome, 15 rheumatoid arthritis, 15 psoriatic arthritis and 20 ANCA associated vasculitis) were included into the study. Hundred and fourteen patients (16.6%) were ANA negative.Sensitivity was more than 90% for 2012 SLICC criteria and 2019 EULAR/ACR criteria and positive predictive value was more than 90% for all three criteria (Table 1). Specificity was the highest for 1997 ACR criteria. Negative predictive value was 76.9% for ACR criteria, 88.4% for SLICC criteria and 91.7% for EULAR/ACR criteria.In only ANA positive patients, sensitivity was 79.6% for 1997 ACR criteria, 92.2% for 2012 SLICC criteria and 96.1% for 2019 EULAR/ACR criteria. Specificity was 92.6% for ACR criteria, 87.8% for SLICC criteria 85.2% for EULAR/ACR criteria.Eleven clinically diagnosed SLE patients had insufficient number of items for both 2012 SLICC and 2019 EULAR/ACR criteria. Both criteria were fulfilled by 16 diseased controls: 9 with Sjögren’s syndrome, 5 with antiphospholipid syndrome, one with dermatomyositis and one with systemic sclerosis.Table 1.Sensitivity, positive predictive value, specificity and negative predictive value of 1997 ACR, 2012 SLICC and 2019 EULAR/ACR classification criteriaSLE (+)SLE (-)Sensitivity (%)Positive Predictive Value (%)Specificity (%)Negative Predictive Value (%)1997 ACR(+) 308(-) 841527978.695.494.976.92012 SLICC(+) 357(-) 352626891.193.291.288.42019 EULAR/ACR(+) 368(-) 242826693.892.990.591.7Conclusion:In this cohort, although all three criteria have sufficient specificity, sensitivity and negative predictive value of 1997 ACR criteria are the lowest. Overall, 2019 EULAR/ACR and 2012 SLICC criteria have a comparable performance, but if only ANA positive cases and controls are analysed, the specificity of both criteria decrease to less than 90%. Some SLE patients with a clinical diagnosis lacked sufficient number of criteria. Mostly, patients with Sjögren’s syndrome or antiphospholipid syndrome are prone to misclassification by both recent criteria.References:[1]Aringer M, Costenbader K, Daikh D, et al. 2019 European League Against Rheumatism/American College of Rheumatology classification criteria for systemic lupus erythematosus. Ann Rheum Dis 2019;78:1151-1159.Disclosure of Interests:None declared

Background:Antiphospholipid syndrome (APS) is a chronic autoimmune disease with significant morbidity and mortality. The recently developed damage index for APS (DIAPS) considers thrombotic APS specific damage.Objectives:Herein we aimed to identify disease clusters based on clinical characteristics and compare DIAPS between these clusters in a single center cohort of patients with APS ± systemic lupus erythematosus (SLE).Methods:This retrospective study included 237 consecutive patients with APS [114 primary APS (PAPS) and 123 SLE+APS]. Data regarding demographics, clinical and laboratory characteristics and cardiovascular risk factors were retrieved from the existing database and revised. Two-step cluster analysis was performed. Cumulative damage was calculated for all patients by applying DIAPS as described previously.Results:237 patients were classified into 4 subgroups by cluster analysis. Cluster 1 (n=74) consisted of older patients with arterial-predominant VT, livedo reticularis and increased cardiovascular risk, cluster 2 (n=70) of SLE+APS patients with thrombocytopenia and heart valve disease, cluster 3 (n=59) of patients with venous-predominant VT, less extra-criteria manifestations and cluster 4 (n=34) of patients with only PM with a lower frequency of extra-criteria features and cardiovascular risk (table 1).Table 1.Demographic, clinical and laboratory characteristics of clustersAll (n=237)Cluster 1 (n=74)Cluster 2 (n=70)Cluster 3(n=59)Cluster 4 (n=34)PAge (years), median (range)43 (20-81)51 (20-81)40 (27-72)42 (24-69)40.5 (26-65)Duration of disease (years), median (range)9.5 (1-37.7)13.1 (1-37.7)10.4 (1-28.7)8.5 (1-32.8)7 (1-22.4)0.028Female, n (%)198 (83.5)56 (75.7)61 (87.1)47 (79.7)34 (100)SLE, n (%)123 (51.9)31 (41.9)46 (65.7)32 (54.2)14 (41.2)Vascular thrombosis, n (%)191 (80.6)73 (98.6)59 (84.3)59 (100)0 (0)Arterial thrombosis, n (%)109 (46)50 (67.6)31 (44.3)28 (47.5)0 (0)Venous thrombosis, n (%)112 (47.3)36 (48.6)37 (52.9)39 (66.1)0 (0)Pregnancy morbidity, n (%)117 (49.4)22 (29.7)46 (65.7)15 (25.4)34 (100)Livedo reticularis, n (%)38 (16)21 (28.4)10 (14.3)5 (8.5)2 (5.9)Thrombocytopenia, n (%)81 (34.2)4 (5.4)65 (92.9)4 (6.8)8 (23.5)Heart valve disease, n (%)92 (38.8)32 (43.2)46 (65.7)8 (13.6)6 (17.6)Arterial hypertension, n (%)101 (42.6)49 (66.2)34 (48.6)18 (30.5)0 (0)Hyperlipidemia, n (%)103 (43.5)69 (93.2)26 (37.1)0 (0)8 (23.5)Smoking, n (%)58 (24.5)31 (41.9)7 (10)17 (28.8)3 (8.8)Lupus anticoagulant, n (%)156 (65.8)53 (71.6)48 (68.6)35 (59.3)20 (58.8)0.36Anticardiolipin IgG/IgM, n (%)155 (65.4)46 (62.2)46 (65.7)38 (64.4)25 (73.5)0.71Anti-β2-glycoprotein I IgG/IgM, n (%)93 (39.2)25 (33.8)33 (47.1)26 (44.1)9 (26.5)0.13Triple aPL positivity, n (%)45 (19)12 (16.2)16 (22.9)13 (22)4 (11.8)0.46Cluster 2 had the highest cumulative damage (mean DIAPS 2.48 ± 1.67) followed by cluster 1 (2.24 ± 1.44), cluster 3 (1.69 ± 1.27) and cluster 4 (0.32 ± 0.68). Comparison of DIAPS (total and major domains) between the clusters is shown in figure 1.Patients with SLE+APS had a higher mean DIAPS compared to those with PAPS (2.10 ± 1.61 vs 1.69 ± 1.47, P=0.046). Cardiovascular domain was the most frequently affected DIAPS domain in both groups. Proteinuria and avascular necrosis were significantly more frequent in SLE+APS (9.8% vs 2.2%, P=0.02 and 5.7% vs 0%, P=0.009, respectively). DIAPS was positively correlated with disease duration (r=0.192, P=0.003).Conclusion:Elder APS patients with arterial thrombosis and increased cardiovascular risk and SLE+APS patients with extra-criteria manifestations had higher cumulative DIAPS. Longer disease duration, higher frequency of major organ involvement and higher immunosuppressive usage may have contributed to this difference. Therefore, control of cardiovascular risk factors, prevention and effective treatment of SLE flares may help to reduce damage in these subgroups.Figure 1.Comparison of mean DIAPS (total and major domains) between the clustersDisclosure of Interests:None declared

Background:The rates of hospitalization in patients with SLE is around 10% per year.1Objectives:In this study, we aimed to examine the hospitalization data of patients with SLE in the last 5 years at our center and determine the factors that affect hospitalization.Methods:Hospitalization data of patients with SLE (2012 SLICC classification) admitted to our rheumatology ward between January 2015 and 2020 were analyzed. Cumulative clinical and laboratory findings were retrieved from the existing SLE database and revised. SLICC SLE damage index (SDI), and the disease activity at admission were determined (SLEDAI-2K).Results:Eighty-six % (n=138) of 161 hospitalized patients were female. The mean age of the patients was 38 ± 13 years whilst mean duration of disease was 97.3 ±96.9 months. Thirty-eight% of the patients were hospitalized more than once and the mean number of hospitalizations was 1.8±1.5 The mean hospitalization duration covering all stays for each patient was 25±27 days. Active disease followed by infection and damage-related complications ranked the first three among all causes of hospitalization.Compared to patients hospitalized for active disease or other reasons, patients hospitalized for infection had a significantly higher number of readmissions (p) and their total duration of hospitalization was longer (p). Duration of disease was significantly shorter in patients hospitalized for active disease compared to patients hospitalized for infection and damage related causes (pThe frequency of patients with damage and the mean SDI score was significantly lower in the group with active disease (68% and 1.9 ± 2) compared to patients hospitalized for infection (90% and 2.7±1.6) and other causes (96% and 3±1.7) (pp) as well as the number of hospitalizations (r=0.393, p). Regarding disease activity at the time of admission, the mean score of patients hospitalized for active disease was 11.0 ± 6.1 whilst was 3.2 ± 2.8 in patients hospitalized for infection and 2.9 ± 3.3 in patients hospitalized for other reasons (p). Renal active disease was the most common (44%), followed by hematological (34.8%), articular (21.7%) and mucocutaneous (21%) activity. Ten% of the patients all of whom had damage were admitted to intensive care unit (ICU). Total hospitalization duration (p=0.012), mean SDI (p=0.008), antiphospholipid syndrome (p=0.033), lupus anticoagulant (p=0.010), thrombocytopenia (p=0.015), serositis (p=0.034), pulmonary hypertension (p=0.021), history of alveolar haemorrhage (pcardiac valve involvement (p=0.002) were associated with ICU hospitalization.Conclusion:Disease activity, infections and damage are the leading causes of hospitalization in patients with SLE. Damage increases the frequency of hospitalizations, prolongs the duration of stay, and increases the need for follow-up in the ICU. Tight control of disease activity with rational use of immunosuppressive treatment is important to reduce damage and hospitalizations.Graphic 1.Distribution of damage according to organs/systems in hospitalized patientsReferences:[1]Gu K, Gladman DD, Su J, Urowitz MB. Hospitalizations in patients with systemic lupus erythematosus in an academic health science center. The Journal of rheumatology 2017;44:1173-8.Disclosure of Interests:None declared

Background:Familial Mediterranean fever (FMF) is a hereditary autoinflammatory disorder caused by the MEFV gene variants. Although association between FMF and spondyloarthritis (SpA) has previously been reported, clinical and laboratory features of patients with FMF and SpA have not been defined in detail.Objectives:We aimed to evaluate clinical and laboratory characteristics, disease outcome and biologic responses of patients with FMF+SpA compared to patients with only SpA who were followed-up in our tertiary referral center.Methods:Database of FMF Clinic was screened for FMF patients with coexistent SpA and 113 patients were identified fulfilling Tel Hashomer and ASAS criteria for FMF and SpA, respectively. A group of patients with SpA without FMF matched for age, gender and disease duration were selected as the control group.Results:Thirteen patients were excluded because of missing data, and 100 patients (F/M: 52/48) were included into the analysis. Mean follow-up time was 93.6 ± 77 (range[r]: 3-324) months and mean patient age was 43.3 ± 12 (r: 20-87) years. Mean age of onset for FMF was 12.5 ± 8 (r: 1-36) and for SpA was 25 ± 11 (r: 7-72) years. SpA findings was classified as axial in 35.4%, axial and peripheral in 47.9% and only peripheral in 16.7% in FMF+SpA group. Half (49%) of the patients had hip involvement (70% bilaterally), and 21.5% of them needed total hip joint replacement (TJR), which were significantly more frequent compared to control group. Two exon 10 MEFV variants were found in 69.4%, and most (69.8%) had homozygous M694V. Hip involvement was more frequent in patients with two exon 10 variants (p=0.036; OR=4.4) compared to those with one variant; and TJR was more frequent in those with homozygous M694V compared to other exon 10 variants (p=0.001; OR=10). Radiographic sacroiliitis was less frequent in patients with homozygous M694V (p=0.019; OR=5.48). HLA-B27 positivity was not associated with hip or axial involvement in patients with FMF+SpA.Biologics were used in 60 patients (anti-TNF in 43, secukinumab in 1, and tocilizumab in 2). Anti-IL-1 drugs were used in 23 patients for refractory FMF. In 9 patients, anti-TNF and anti-IL-1 drugs were tried for refractory joint involvement: 5 switched to anti-TNFs from anti-IL-1, 4 patients switched to anti-IL-1 from anti-TNFs. Biologic DMARD requirement was more frequent in patients with two exon 10 variants (p=0.006; OR=7.4), especially in those with homozygous M694V (p=0.006; OR=7.6). Although anti-IL-1 usage did not differ among MEFV variants, anti-TNF was used more frequently in patients with homozygous M694V (p=0.007; OR=7.2). FMF+SpA patients had higher serum CRP and developed amyloidosis more frequently than those patients with SpA.Table 1.Comparison of clinical and laboratory findings between the patients with FMF+SpA and SpA controlFMF + SpA (n=100)SpA (n=217)P valueAge (years)*43.3 ± 1243.4 ± 110.6Sex (n, %) Male48104 (47.9)0.99 Female52113 (52.1)Duration of SpA (monnths)*181.6 ± 108180.2 ± 1120.8Age onset of SpA (years)*25.1±1128.4±80.008Peripheral arthritis (n, %)35/80 (43.8)79/212 (37.3)0.3HLA-B27 positivity (n, %)6/21 (28.6)105/139 (75.5)CRP (mg/dL)*26.7 ± 25**18.96±290.001ESR (mm/hour)*39.7 ± 2739.4 ± 280.8Hip involvement (n, %)47/96 (49)23/118 (19.5)TJR (n, %)20/93 (21.5)8/205 (3.9)Fulfilling mNY criteria (n, %)52/81 (64.2)164/199 (82.4)0.001 (OR=10.8)Biologic DMARD (n, %)6068/214 (31.8)Anti-TNF (n, %)4668/214 (31.8)0.015 (OR=5.96)Amyloidosis (n, %)165/205 (2.4)* mean±standard deviation, **during the attack-free periodConclusion:In this group of FMF+SpA patients, hip involvement and need for TJR were more frequent and associated with penetrant MEFV variants rather than HLA-B27 positivity. These patients had higher inflammatory response and risk of developing amyloidosis, and they needed biologics more frequently compared to SpA group. More severe disease course in FMF+SpA patients requires further attention and analysis in larger cohorts.Disclosure of Interests:None declared

Background:AA amyloidosis has been associated with uncontrolled chronic inflammatory diseases such as rheumatoid arthritis (RA), ankylosing spondylitis (AS), inflammatory bowel disease (IBD) and hereditary periodic fever syndromes, and the most common cause is familial Mediterranean fever (FMF) in Turkey.Objectives:We herein aimed to evaluate clinical and laboratory characteristics and treatment responses of patients with AA amyloidosis retrospectively in a tertiary referral center.Methods:Study group was consisting of patients with biopsy proven AA amyloidosis, and their data were recorded from their charts. Treatment responses were categorized as follows:complete responsewas defined as no increase in serum creatinine and a proteinuria below 1gr/day;partial responseas 50% decrease in proteinuria; andstable disease as no significant change in serum creatinine and proteinuria. Progressive disease was defined as increase in serum creatinine and/or proteinuria under treatment.Results:173 patients were identified, and 10 patients with no biopsy result and/or missing data were excluded. A total of 163 patients (79 females, 84 males) were included in the study. Median age of patients was 45.4, and median age at diagnosis of amyloidosis was 33.5. Most common cause of amyloidosis was FMF (78.5%), followed by idiopathic cases (7.9%) and patients with AS (4.9%). A quarter (26%) of amyloidosis patients had a family history for AA amyloidosis, and 59% of patients with FMF had a family history of FMF. Amyloidosis was confirmed by renal biopsy in 76.1%, by gastrointestinal (GIS) biopsy in 11.7%, and by other biopsies in the remaining. Renal involvement was documented in 160 (98.2%) patients, while GIS involvement in 20.9%, heart in 13.5%, thyroid in 3.7% and bone marrow in 3.1%. In FMF patients, most common MEFV mutation was M694V (77.7%); and 66.7% of the patients had homozygous, 14.6% had compound heterozygous, and 18.7% heterozygous exon 10 variants. Mean age at diagnosis of amyloidosis was earlier in homozygotes (29.1) and compound heterozygotes (32.3) compared to heterozygotes (43.9) (p = 0.001). There was no difference in treatment responses, organ involvement, progression to end stage renal disease (ESRD) and mortality between monoallelic and biallelic exon 10 mutations (p = 0.42). While 44.3% (n = 70) of patients had chronic renal disease (CRD) at time of diagnosis, ESRD developed in 45.3% (n = 73) of patients. During follow-up, 55 patients underwent renal transplantation and recurrence of renal amyloidosis occurred in 24% of them. Mean creatinine and proteinuria levels at time of diagnosis were higher in patients with ESRD than those without ESRD (p Figure 1: Survival graphic of AA amyloidosis patients who developed ESRD according to their baseline GFR statusConclusion:Increased rate of ESRD and progression of amyloidosis findings in patients who presented with GFRDisclosure of Interests:None declared

Background:Treatment options for systemic sclerosis (SSc) remain limited especially in severe skin, lung and musculoskeletal involvements. B-cell targeted therapy with anti-CD20 Rituximab (RTX), is widely available, reports from case series are encouraging as a a rescue therapy and might have an improving effect on organ involvement in SSc.Objectives:We aimed to retrospectively analyze the efficacy and safety of rituximab (RTX) courses in patients with severe systemic sclerosis who were refractory to standard immunosuppressive treatment.Methods:Twenty-seven SSc patients fulfilling ACR/EULAR classification criteria (2013) who received RTX treatment due to acive disease despite treatment with immunosuppresives were analyzed. Disease activity was evaluated by using EScSG/EUSTAR activity scores prior to and after RTX treatment. Disease severity was also assessed at baseline by Medsger’s index.Results:The demographics and characteristics of SSc patients were as follows: the median age of 50 (30-70), duration of Raynaud’s 10 (3-26) and non-Raynaud symptom 8.5 (3-18) years and summarised in table 1. RTX was given as a single cycle (2 infusions of 1000 mg) in 12 cases, 2 cyles in 5 cases, ≥3 cyles in 10 cases. DMARDs were prescribed in 19 (73%) patients (14 MMF, 5 MTX) concomitantly with RTX. The main RTX indications were skin and lung involvement (n=9), skin and arthritis (n=6), skin(n=5), lung (n=3), myositis (n=2), cardiac involvement (n=1) and digital vasculopathy (n=1). Medsger severity score was 7.39±3.091(3-13) at baseline.Table 1.Prevelance of Characteristics of SSc Patientsn(%)female/male25 /2diffuse/limited cutaneous SSc22 (81.5) / 5 (19,2)Clinical Characteristicssynovitis / flexion contractures12(44,4) / 10 (37,1)tendon friction rubs / myositis7 (26,9) / 4 (15,4)renal crisis1 (3,8)GI involvement19 (69,2)lung involvement16 (61,5)SerologyANA23 (85,2)Anti-Scl70 / Anti-sentromer16(61,5) / 1(3,8)Anti-Ro6 (22,2)Previous ImmunosuppressivesCYC / MMFAZA / MTX Low dose steroids19 (73,1) / 19 (73,1) 12 (46,2) / 16 (61,5) 27 (100)Disease activity and severity scores prior to and after RTX were summarised table 2. Disease activity scores were improved after RTX in patients who had a median follow-up period of 1 year (0,5-5 years). After RTX treatment, according to EscSG /EUSTAR scores 13 (%46.2) and 10 (%34.6) patients out of 26 were assessed as inactive.Table 2.Disease activity scores prior to and after RTX treatmentPrior to RTX (n=26)After RTX (n=18)median Δ Change (n=18)EscSG activity score4,89±1,82 (2,0-9,0)2,37±1,10 (0,50-4,50)-2,00 (PEUSTAR activity score4,57±2,68(1,0-10,0)2,30±2,15 (0,0-7,25)-2,00 (P=0,002)There were severe infecions in 4 patients (Pneumonia in 2, infected digital ulcers in 2) and an episode of sinusitis in one during treatment period. One patient was deceased because of pneumonia and sepsis after the first cycle of RTX.Conclusion:In our SSc cohort, RTX treatment was used in severe patients, who had predominantly diffuse cutaneous disease with lung and joint involvements, severe vasculopathy and anti-Scl-70 positivity. Concomitant DMARDs were used in three-forth of the patients in addition to RTX cycles. Disease activity scores that assessed retrospectively were shown to be improved after RTX and 37-48% of the cases were assessed as inactive by using activity scores. Serious infections like pneumonia and infected digital ulcers were observed in 14,8% of cases during the follow-up. The addition of RTX treatment can be effective in selected patients with active disease despite immunosuppressive therapy.Disclosure of Interests:None declared

Background:Patients with systemic lupus erythematosus (SLE) can present with acute disease flares/exacerbations during pregnancy and postpartum period.1These flares can cause adverse pregnancy outcomes (APO).Objectives:In this study, our pregnant SLE cohort, which was under medical surveillance of both our Rheumatology and Gynecology and Obstetrics departments was analyzed. We intended to determine the effects of pregnancy on disease activity and the correlation between disease flares and adverse pregnancy outcomes.Methods:168 pregnancy data involving 136 patients with SLE meeting the ACR criteria were examined. Cumulative clinical, laboratory and serological parameters were described and disease activity and flares were calculated using SLEDAI-2K disease activity index during preconceptional six month period, during all trimesters of pregnancy, and during postpartum six month period. Patients with low lupus disease activity scores (LLDAS) during each of these periods were identified. Fetal/neonatal death, premature birth due to preeclampsia, eclampsia or HELLP syndrome, neonates small for gestational age were determined as adverse pregnancy outcomes. Relationship of APO with disease activity was studied and patients with APO were compared to patients without APO.Results:Mean SLEDAI-2K scores was 1.3±2.2 (0-16) during preconceptional six month period, 1.3±2.6 (0-16) during conception period, 1.7±3.2 (0-22) during first trimester, 1.4±2.7 (0-16) during second trimester, 1.5±3.3 (0-20) during third trimester and 3.5±5.4 (0-26) during postpartum six month period. Mean postpartum six month period SLEDAI-2K score was higher compared to the mean pregnancy SLEDAI-2K score (pAPO was observed in 34% of pregnancies (n=57). APO (+) group was characterized by significantly longer disease duration and higher disease activity in all periods compared to APO (-) group (142±70 vs 170±88 months, pConclusion:Postpartum six-month period appears to have the highest risk for disease flares during SLE pregnancies. Disease activity during pregnancy increases the risk of APO. Patients with sustained LLDAS have significantly lower APO rates. In order to achieve a positive pregnancy outcome and lower maternal morbidity, regular follow up of patients during pregnancy and postpartum period by Rheumatology and Gynecology and Obstetrics Departments is necessary.References:[1]Eudy AM, et al. Ann Rheum Dis 2018;0:1–6. doi:10.1136/annrheumdis-2017-212535Disclosure of Interests:None declared

Background:The prevalence of giant cell arteritis (GCA) in Turkey has been reported lower than other European countries and the information on clinical patterns, diagnostic modalities, treatment and prognosis of GCA are limited (1).Objectives:We aimed to analyse our GCA cohort from a large outpatient clinic for the last 20 years.Methods:Data of the GCA patients followed up at least for 6 months in our vasculitis clinic between 1998 and 2018 evaluated retrospectively according to EULAR 2018 GCA clinical research recommendations (2). Chi-square, students t-test, logistic regression analysis and Kaplan-Meier test were used for statistical analysis.Results:Eighty-nine patients with adequate follow-up data (64% female, mean age 67.9 ± 9.1) were analysed. Median follow up duration was 46 months (3-256) and mean time to diagnosis after presenting symptom (TTD) was 5,9±1,2 months (0-60). Polymyalgia rheumatica was found in 36 (40.4%) patients. The clinical findings of the patients are shown in Table-1. Mean TTD was longer in patients with acute vision loss (AVL) (11±4 vs. 4,8±1,1 months p=0.002). Mean CRP was 90,7±82 (8-343) mg/L and ESR was 103,7±25 (52-138) mm/h at the time of diagnosis. Mean age was lower (63±2 vs 69±1 p=0.01); mean CRP (141,8±107,3 vs. 76,6±67,9 mg/dL p=0.023) and ESR (120,8±25,1 vs. 99,3±24,3 mm/h p=0.004) was higher in patients without cranial symptoms (extracranial GCA group). PET-CT findings compatible with large vessel vasculitis were present in 64% (34/53). Sixteen of 19 (%84,2) patients in the extracranial GCA group had positive PET-CT. Temporal artery (TA) biopsy positivity was 64% (34/53). Sensitivity of ACR 1990 Criteria was 77,5% and GIACTA study inclusion criteria was 58,4% in this cohort at diagnosis. Fullfilment of GIACTA criteria was still present in 12 (13,5%) patients after six months of follow up. Treatment data was shown in table-2. Total flare rate was 34,8% and flare risk was lower in the extracranial GCA group (3/20 vs. 28/69 p=0.035 OR=0.78 %95 CI 0.64 – 0.96). Reduced survival was observed in cases diagnosed older than 65 years (168,8±23,9 vs 209±17,3 months p=0,015).Conclusion:The analysis of the largest single center cohort from Turkey confirmed that delayed diagnosis is associated with vision loss. A subgroup of patients without apparent cranial symptoms but positive PET-CT findings is delineated. These patients are younger, present with higher inflammatory response and fewer relapses. The sensitivity of ACR criteria in our cohort is less than 80%. High flare rate especially in GCA patients with cranial symptoms and GIACTA criteria fullfilment after 6 months of treatment in more than 10% of the patients show a need for for new treatment options.References:[1]Pamuk, O.N., et al.,Giant cell arteritis and polymyalgia rheumatica in northwestern Turkey: Clinical features and epidemiological data.Clin Exp Rheumatol, 2009.27(5): p. 830-3.[2]Ehlers, L., et al.,2018 EULAR recommendations for a core data set to support observational research and clinical care in giant cell arteritis.Ann Rheum Dis, 2019.78(9): p. 1160-1166.Table 1.Clinical characteristicsSystemic / Extracranial Findings78 (%87,6)Cranial Findings69 (%77,5)Opthalmologic Findings20 (%22,5)Fatigue72 (%80,9)Headache60 (%67,4)AVL16 (%18,8)Weight loss47 (%52,8)Jaw claudication33 (%37,1)Unilateral12 (%13,5)Arthralgia43 (%48,3)Scalp / TA Tenderness27 (%30,3)Bilateral4 (%4,5)Fever35 (%39,3)Decreased pulsation of TA11 (%12,4)Diplopia4 (%4,5)Arthritis13 (%14,6)CVE6 (%6,7)Vascular murmur3 (%3,4)Swollen TA6 (%6,7)Pericardial effusion2 (%2,2)Vertigo5 (%5,6)Extremity Claudication1 (%1,1)TABLE 2Treatment data of GCA cohortInitial glucocorticoid (GC) dosage (mg)46,7±20,1Pulse GC treatment%12,312nd month cumulative GC dosage (g)4,7±2,5MTX usage%63,3bDMARD usage%6,7Acetyl salicylic acid usage%51,7Disclosure of Interests:None declared

Background:Proximal scleroderma is the major criterion in both 1980 and 2013 classification criteria for sytemic scleroris (SSc). ACR(1980) criteria included digital lesions and bibasiler fibrozis, nonetheless ACR/EULAR(2013) criteria based on a scoring system including digital lesions, telangiectasia, abnormal nailfold video-capillaroscopy(NVC), PAH, Raynaud’s and specific autoantibodies.Objectives:We aimed to implement both criteria in a Turkish SSc kohort to evaluate the contribution of non-skin manifestations, NVC and autoantibodies.Methods:A consecutive hundred and thirty-nine (125 females) SSc patients diagnosed and evaluated by the same experts (YY, MI) with relevant NVC records and at least 6 months follow-up were included into the study. Classificaiton criteria were used retrospectively using a preformed database.Results:Characteristics of the SSc patients were summarized in table-1. The mean age, duration of Raynaud’s and non-Raynaud symptoms were 47.1±11.9, 8.9±7.9 and 5.7±5.8 years, respectively. Diffuse and limited cutaneous disease were diagnosed in 62(44.6%) and 60(43.2%) patients respectively. Asclerodermic disease was present in 17(12.2%) patients. ANA, anti-centromere and anti-Scl70(+) positivity was 80.5%, 18.0% and 37.4%, respectively.Twelve patients (8.6%) could not be classified as SSc by both criteria; 5 with Raynaud’s+specific antibodies (2 anti-centromere+, 2 anti-Scl70+), 4 with Raynaud’s+puffy hands+NC abnormalities, 2 with Raynaud’s+telangiectasia and a patient with Raynaud’s+sclerodactyly. Nineteen (13.7%) patients could not be classified as SSc according to ACR (1980) can be classsified according to ACR/EULAR (2013) (table-1 and -2).The sensitivity for ACR/EULAR (2013) and ACR (1980) criteria were found to be 91,4% vs 75,5%; 98.4% vs 96.8% in diffuse cutaneous SSc, 98.3% vs 68.3% in limited cutaneous SSc and 47.1 vs 23.5% in asclerodermic SSc, respectively.Table 1.The sensitivity for ACR (1980) and ACR/EULAR (2013) classification criteriain SScACR/EULAR (2013) +ACR/EULAR (2013)-totalACR (1980)+1080108ACR (1980)-191231total12712139Table 2.SSc patients fulfilling ACR (1980) and/or ACR/EULAR (2013) criteriaACR/EULAR (2013)ACR (1980)Classified n=127Not classified n=12+Prox scleroderma+87+Puffy hands294+Sclerodactyly+1121+Digital ulcers + +Pitting scars62 72+Telangiectasia862+Nailfold capillaroscopy974 Normal9 Early173 Active40 Late501+PAH12+Interstitial lung Disease +60+Raynaud’s12012+SSc- specific antibodies795Conclusion:The sensitivity of ACR/EULAR (2013) criteria was shown to be higher than ACR (1980) criteria in our Turkish SSc cohort with established cases. Although in diffuse cutaneous subgroup, the sensitivity was >%96 for both criteria, in limited cutaneous subgroup, the sensitivity was preserved for ACR/EULAR(2013) while apparently decreased for ACR(1980) criteria (Disclosure of Interests:None declared

Background:Evaluating disease activity and severity in systemic sclerosis (SSc) is crucial to define the patients who are candidate for treatment options.Objectives:We aimed to investigate the relationship between disease activity and severity in SSc in a large cohort.Methods:This is a cross-sectional prospective analysis of 278 (253 females) patients fulfilling ACR/EULAR (2013) classification criteria for SSc. Disease activity and severity were calculated seperately for cutaneous subsets (EscSG and Medsger). The patients were grouped as inactive if EscSG score=0, mildly active if EscSG score>0Results:The mean age, duration of Raynaud’s and non-Raynaud features were 48.5±13.1, 12.1±9.8 and 8.3±7.5 years respectively. Characteristics of the SSc patients were summarized in table-1.Ninety-three (34%) and 151(54%) patients were evaluated as having active and mildly active disease. Only 34(12%) patients had inactive disease. The patients with diffuse cutaneous involvement (dcSSc) who were active had higher modified Rodnan Skin score(mRSS) and severity scores of general, skin and joint-tendon involvements; the patients who had mildly active disease also had higher scores of mRSS and severity scores of skin compared to those with inactive disease (table-2).The patients with limited cutaneous involvement (lcSSc) who were assessed as having active disease had higher mRSS and higher severity scores of general, skin, peripheral vascular, lung, joint-tendon and gastrointestinal involvements; the patients who had mildly active disease also had higher scores of mRSS and severity scores of skin, lung, joint-tendon and gastrointestinal involvements compared to those with inactive disease (table-2).Conclusion:One third of our cohort was found to have active disease despite treatment and only 12% had inactive disease. Skin involvement and severity of different organs were shown to be higher in patients with active disease in both cutaneous subsets, together with severity of lung, peripheral vascular and gastrointestinal involvements in active lcSSc. LcSSc and dcSSc patients who had mildly active disease also had severe disease similar to those with active patients. Disease activity and severity should be assessed as separate measurements to highlight the course of the disease and may guide to the management of patients with SSc.Disclosure of Interests:None declared

Background:Development of organ damage is a major concern in patients with systemic vasculitis. Treatment may also contribute to this important outcome. Scoring systems has been developed to evaluate organ damage in systemic vasculitis and specifically for large vessel vasculitis (1).Objectives:We aimed to investigate permanent organ damage and determining factors in our giant cell arteritis GCA cohort.Methods:Organ damage detected at the time of diagnosis and / or follow-up and irreversible for at least 3 months in GCA patients followed up between 1998-2018 were recorded by using Vasculitis Damage Index (VDI) and Vascular Vasculitis Damage Index (LVVID) fom patient records of our vasculitis clinic. In the statistical evaluation, chi-square, students t-test and logistic regression analysis were used.Results:Eighty-nine patients (64% women, mean age 67.9 ± 9.1) included in the study, the mean follow-up duration was 61.6 ± 58.6 months. All organ damage findings according to both VDI and LVVID are shown in table-1. In this cohort, cardiovascular damage items and diabetes mellitus were prevalent at baseline. At least one damage item was present in 53 (59,5%) according to VDI; 54 (%60,7) according to LVVID and agreement was high between two damage indices (kappa=0.97). Forty-seven of patients (52%) had a damage item presumably with contribution of GC treatment e.g. locomotor system findings, hypertension, diabetes and cataract; 12 (13,5%) had damage items related to disease (total or partial vision loss, ischemic optical neuropathy). Mean time to diagnosis after initial symptoms was longer in patients with permanent vision loss (10,2±4,3 vs. 5,2±1,2 months p=0.006). The presence of damage was associated with flares in univariate and multivariate analysis (29/54 vs. 2/35 pConclusion:In our study, permanent organ damage was analysed by using diffrerent indices. In this patient population baseline cardiovascular damage and diabetes mellitus were frequent as expected but information for osteoporosis was lacking. More than half of the patients had damage and significant part of the present items was considered due to corticosteroid treatment. The most common damage item developed was osteoporosis. There was a very good agreement between the two indices, despite few specific items in LVVID. The striking relationship of disease flare with damage and frequency of visual problems despite treatment indicate the necessity of new treatment strategies.References:[1]Kermani, T.A., et al.,Evaluation of damage in giant cell arteritis.Rheumatology (Oxford), 2018.57(2): p. 322-328.Disclosure of Interests: :None declared

Background:Serious infections (SI) are one of the main complications in patients with ANCA associated vasculitis (AAV).Objectives:We planned to investigate the prevalence, features and risk factors of SI in our AAV cohort during follow-up.Methods:Outpatient and hospital data of patients diagnosed with granulomatous polyangiitis (GPA), microscopic polyangiitis (MPA) and eosinophilic granulomatous polyangiitis (eGPA) between 1999 and 2019 according to Chapel Hill Consensus Criteria and followed up at least 6 months in our vasculitis clinic were evaluated. Development of sepsis, requirement for intravenous (IV) antibiotherapy and / or hospitalization during infection episodes were considered as SI. Chi-square, student’s t-test and logistic regression analysis were used for statistical analysis.Results:Study was conducted with 186 (53.6% female) patients with adequate follow-up data. Mean age of diagnosis was 54.3±14,5 (23-79), mean follow-up duration was 86,4 ± 54,3 (6-251) months. Number of GPA, MPA and eGPA patients were 132 (71%), 42 (22,5%) and 12 (6,5%), respectively. IV cyclophosphamide (CYC) was used in 148 (79,6%), azathioprin in 105 (56,5%), rituximab (RTX) in 69 (%37,1), methotrexate in 29 (15,6%) and mycophenolate mofetil in 14 (7,5%) patients. Number of patients developed SI was 66 (34.7%), total SI episode was 86, patients who had multiple episodes was 15. All SI is shown in Table-1. Bacterial pneumonia was the most common diagnosis and 26 of SI (30.2%) were considered as opportunistic (systemic viral, parasite, fungus) infections. Thirty-one of patients developed SI (40,7%) in the first year after diagnosis. SI were observed more frequently in the presence of major organ involvement (kidney, lung, neurological) (65/173 vs. 1/13 p = 0.02 OR = 8.7 95% CI 1.06-64.4). Diffuse alveolar hemorrhage (DAH) was associated with SI in multivariate analysis (12/52 vs. 0/34 p=0.007 OR=1.6 95% CI 1.3-1.96). Cumulative CYC dose was significantly higher in patients with SI (14,2±21 vs. 8.2±13.9 p=0.045). During maintenance, patients treated with RTX had significantly more SI (18/53 vs. 17/99 p=0.19 OR=3,3 95% CI 1,55- 7,07). Hypogammaglobulinemia (HIgG) (IgGConclusion:Long-term follow-up results of a single center cohort of AAV patients revealed that approximately one third of patients developed SI, most frequently in the first year of treatment. During the maintenance period, the risk of SI continues. Cumulative CYC dosage and maintanence with RTX is associated with SI, especially in patients who developed hIgG. Major organ involvement, disease flares and organ damage are significant risk factors for SI. In this regard, protection measures (vaccination, prophylaxis) should be reviewed and the quality of follow-up should be improved.Table 1.Serious infections in AAV patients.BACTERIALNFUNGALNVIRALNPROTOZOANNPneumonia37PJP7Zoster Zona3Intramuscular abscess (Nocardiosis)1Urinary Tract Infection (UTI)8Aspergilloma1CMV Pneumonia1Gr (-) sepsis2Invasive Fungal Infection3CMV Colitis1Perianal abscess2Candida Eosephagitis4CMV Gastritis1Intraabdominal abscess2Candidemia2HSV Eosephagitis1Catheter infection2UTI1Sellulitis1Fungal otitis1Orbital sellulitis1Maxillary sinüs abscess1Mastoiditis1Prosthesis infection1Septic artrhitis1Lung tuberculosis1Disclosure of Interests:None declared

Background:Hydroxychloroquine (HCQ) is an immunomodulatory drug that has been shown to improve disease activity in systemic lupus erythematosus (SLE). However, retinal toxicity is an important concern.Objectives:In this study we sought to evaluate the effect of HCQ on disease activity and damage in patients with SLE in whom HCQ was discontinued due to retinal toxicity and whether it could be restarted by a detailed ophthalmologic examination.Methods:Patients who met SLE SLICC classification criteria and were on HCQ for at least 3 years after reaching Lupus Low Disease Activity State (LLDAS) following remission induction and were followed up for at least 3 years after termination of HCQ treatment due to retinal toxicity diagnosed with visual field test were analyzed. Disease activity (LLDAS and SLEDAI-2K) and both the number and severity of flares were recorded for each patient whilst on HCQ and after cessation of treatment. All patients were examined by two experienced ophthalmologists and were assessed by computerized visual field, optical coherence tomography (OCT), fundus autofluorescence (FAF) and fundus florescein angiography (FFA) to further analyze toxicity.Results:Out of 88 patients with recorded HCQ retinal toxicity in a cohort of 1500 patients with SLE, 64 patients (mean age at diagnosis 33.4 ± 10.5 (10-57); 88.5% female) with complete data and opthalmologic re-examination results were included in the analyses. The average duration on HCQ was 122 ±85 (39-336) months, and the mean follow-up time was 74.6 ± 48.3 (36-239) months after the drug was discontinued. Comparison of mean disease activity in the 3-year period when patients were on HCQ to 3 years post-cessation revealed a significantly lower mean SLEDAI-2K score in the former (0.89±1.28 vs.1.3±1.6, p=0.02). The % of visits maintaining LLDAS was higher during HCQ treatment (89.7 ± 17.6 vs. 80.1 ± 23.5, p=0.001). There was significantly a higher frequency of flares with a dominance of mild-moderate types after HCQ was ceased (47.5 vs. 26.2%, p=0.03; 39.3 vs. 22.9%, p=0.024 respectively). There were more patients with serious flares in the post-discontinuation period but without statistical significance (13.1% vs. 4.9% p=0.08). Thirty-seven (60%) of patients restarted the treatment after ophthalmological examination. Although 38 (62,3%) patients had visual field defects in the latest examination, multimodal imaging with OCT, FAF and FFA revealed that only 19 (31%) patient had typical retinal toxicity. Five patients were found to have macular atrophy due to other causes (4 age related macular degeneration and 1 vitromacular adhesion). Since the discrimination of macular pathology would not be possible with imaging in these patients, HCQ was not prescribed. Comparison of patients with and without retinal toxicity showed that duration of HCQ use and HCQ-free time was not significantly different between patients.Conclusion:HCQ is effective in controlling disease activity in patients with SLE and an opportunity for re-medication with HCQ is valuable. More than half of the patients being able to restart HCQ after ophthalmologic examination in this study shows that it is important to perform multimodal imaging techniques in patients with retinal toxicity diagnosis. Since macular pathology can have a different etiologic background, an initial opthalmologic examination is also necessary. Lack of difference in the duration of HCQ exposure and drug-free time between patients who restarted treatment and who could not may be a sign of personal sensitivity to HCQ toxicity.Disclosure of Interests: :None declared

Background:Adjusted global antiphosholipid syndrome score (aGAPSS) is the simplified version GAPSS that was recently developed to assess thrombotic risk by the consideration of antiphospholipid antibody (aPL) profile and conventional cardiovascular risk factors.Objectives:The aim of this study was to evaluate the validity of the aGAPSS in predicting thrombosis and extra-criteria manifestations in our antiphospholipid syndrome (APS) cohort.Methods:Ninety-eight patients with APS were classified according to clinical manifestations as vascular thrombosis (VT), pregnancy morbidity (PM) or both (VT+PM). The aGAPSS was calculated as defined before. Arterial hypertension and hyperlipidemia definitions were made according to the ESC/ESH ve NCEP/ATP III guidelines, respectively.Results:Demographic, laboratory and clinical characteristics of patients are summarized in table-1. Mean aGAPSS was calculated as 10.2 ± 3.8. Significantly higher aGAPSS values were seen in VT (n=58) and VT+PM (n=29) compared to PM (n=11) (mean aGAPSS 10.6 ± 3.7 vs 7.3 ± 2.9, P=0.005; 10.5 ± 4 vs 7.3 ± 2.9, P=0.01, respectively). AUC demonstrated that aGAPSS values ≥ 10 had the best diagnostic accuracy for thrombosis (figure-1). Higher aGAPPS values were also associated with recurrent thrombosis (mean aGAPSS 11.5 ± 3.7 vs 9.9 ± 3.6, P=0.04). Regarding extra-criteria manifestations, patients with livedo reticularis (n=11) and APS nephropathy (n=9) had significantly higher aGAPSS values (mean aGAPSS 12.9 ± 3.4 vs 9.9 ± 3.7, P=0.02; 12.4 ± 2.9 vs 10 ± 3.8, P=0.04, respectively).Conclusion:Our results suggest that patients with higher aGAPSS values are at higher risk for developing vascular thrombosis (either single or recurrent) and extra-criteria manifestations, especially livedo reticularis and APS nephropathy.Table-1.Demographic, laboratory and clinical characteristics of patients.PAPS (n=42)n(%)SLE/APS (n=56)n(%)PFemale36 (85.7)47 (83.9)0.52Age, years (mean±SD)44.6 (11.6)40.8 (10.1)0.42Disease duration, years (mean±SD)10 (8.8)9.7 (7.1)0.16Thrombosis35 (83.3)52 (92.9)0.12•Arterial24 (68.6)34 (65.4)0.47•Venous19 (54.3)26 (50)0.43•Recurrent15 (42.9)22 (42.3)0.56Pregnancy morbidity20 (47.6)20 (35.7)0.16•5 (25)4 (20)0.5•≥ 10 weeks, ≥ 1 abortion14 (70)15 (75)0.5•Pre-eclampsia/eclampsia3 (15)5 (25)0.34•1 (5)5 (25)0.09Convensional risk factors•Arterial hypertension17 (40.5)35 (62.5)0.02•Hyperlipidemia21 (50)26 (46.4)0.41•Diabetes mellitus3 (7.1)3 (5.4)0.51•Obesity19 (45.2)16 (28.6)0.07•Smoking12 (28.6)18 (32.1)0.43aPL profile•LA29 (69)48 (85.7)0.04•aCL IgG/IgM31 (73.8)28 (52.8)0.03•aβ2GPI IgG/IgM22 (56.4)27 (52.9)0.45•Triple positive14 (33.3)17 (30.4)0.46Figure 1.ROC curve according to cut-off aGAPSS value: 10 (AUC: 0.71, sensitivity: 0.52, specificity: 0.91, positive predictive value: 0.98, negative predictive value: 0.19, p-value: 0.01).Disclosure of Interests:None declared

Background:ANCA-associated vasculitis (AAV) is a multisystemic autoimmune disease with high mortality and morbidity.Objectives:We aimed to present the long-term follow-up results of our cohort.Methods:Data of patients who fulfilled Chapell Hill Consensus Criteria and followed up at least 6 months between 1999-2019 were analyzed. A standard form including vasculitis damage index (VDI) was used. Multivariable analysis was performed by using logistic regression.Results:Long-term data was available for 197 patients (%53.8 female) from 208 patient records. Mean age at diagnosis was 49.4 years and mean follow-up was 80.7 months. Granulomatosis with polyangiitis (GPA); microscopic polyangiitis (MPA), eosinophilic GPA (EGPA) were 117 (64.5%), 52 (26.4%), 17 (8.6%), respectively. Relapses are observed in 31.6% of patients. Disease relapses were higher in GPA compared to MPA and EGPA (p = 0.014). Relapse rate was higher in patients withs.aureuscarriage (p = 0.037). Cyclophosphamide (CYC) (76.6%) was most commonly used drug for induction, whereas azathioprine (57.3%) was used mostly in maintenance. In multivariate analysis relapse was found to be associated with maintenance treatment with rituximab (p Conclusion:In our AAV cohort, GPA was most frequent. Although survival was improved, permanent organ damage was detected in the majority of patients. Relapse and organ damage were found to be increased in patients with GPA. Relapses are frequent and maintenance with rituximab could not prevent relapses. Also relapses were associated with venous thrombosis and severe infections. Patients should be screened for malignancies especially of the genitourinary tract.Table 2.Damage findings of AAV patients according to VDIOrgan/systemNumber(%)Steroid myopathy23 (%11.7)Osteoporosis31 (%15.9)AVN30 (%15.4)Cataract30 (%15.4)Partial loss of vision6 (%3.1)Blindness (one eye)2 (%1)Subglottic stenosis9 (%4.5)Hearing loss18 (%9.1)Nasal septum perforation21 (%10,7)Chronic nasal crusting9 (%4,6)Chronic ashtma28 (%14,2)Chronic dispnea1 (%0,5)Hypertension60 (%30,5)Coronary artery disease / Angioplasty10 (%5,1)Cardiomyopathy6 (%3)Valvular heart disease5 (%2,5)Myocardial infarction7 (%3,6)Deep vein thrombosis12 (%6)Chronic renal failure (GFR 51 (%26)End stage renal disease22 (%10.8)Cerebrovascular accident9 (%4,4)Peripheric neuropathy39 (%19.8)Malignancy9 (%4.5)Diabetes mellitus24 (%12.2)Gonadal failure2 (%1)Figure 3.Cumulative Relapse Rate: Hazard ratio of patients treated with Rituximab versus Azathioprine (Log Rank: pDisclosure of Interests:None declared

Background:Pregnancies of patients with systemic lupus erythematosus (SLE) can be risky both for the mother and the fetus because of disease activity and pregnancy complications.1Objectives:In this study, we evaluated the risk factors related to adverse pregnancy outcomes (APO) in our pregnant SLE cohort who were followed up by both Rheumatology and Obstetrics and Gynecology departments at our university.Methods:168 pregnancy data were analyzed from 136 patients who fulfilled ACR classification criteria for SLE. The course of pregnancies were monitored and fetal/neonatal outcomes were recorded. Unexplained fetal death after 12 weeks of gestation, neonatal death, preterm birth due to preeclampsia, eclampsia or HELLP and birth of small for gestational age (SGA) infant were defined as APO. Cumulative clinical, laboratory and serological findings, disease activity (SLEDAI-2K) and damage (SLICC/ACR), and conventional risk factors were compared between APO(+) and APO(-) groups.Results:The comparison of demographics, conventional risk factors and disease characteristics in APO(+) and APO(-) groups are summarized in Table-1. In APO(+) pregnancies, the duration of disease was longer (p Conclusion:Although an important proportion of SLE pregnancies result in live birth, active disease, especially renal and NP involvement, and presence of damage at the beginning of pregnancy increase the risk of maternal and fetal complications. Furthermore, the presence of APS or antiphospholipid antibody positivity are important risk factors for obstetric complications. In conclusion, pregnancy should be allowed after controlling the disease activity and patients should be closely monitored in coordination with Obstetrics and Gynecology clinics. In case of presence of damage, both the patient and the physician should be aware of a possible adverse pregnancy outcome.References:[1]Ann Intern Med. 2015 August 4; 163(3): 153–163. doi:10.7326/M14-2235.Table 1.Demographic data of APO (+) and APO (-) groups, comparison of conventional risk factors, cumulative clinical, serological and laboratory featuresAPO(-)(n=111)APO (+)(n=57)pAge35.1±6.734.9±5.9NSAge at conception30.6±5.628.9±4.2NSDisease duration (months)141.6±70166.9±87.9Chronic hypertension, n (%)6 (7)11 (19.6)Photosensitivity, n (%)86 (77.5)43 (75.4)NSMalar rash, n (%)66 (59.5)38 (66.7)NSOral ulcer, n (%)11 (9,9)6 (10.5)NSArthritis, n (%)77 (59.4)42 (73.7)NSSerositis, n (%)17 (15.3)13 (22.8)NSRenal, n (%)39 (35.1)30 (52.6)Hematologic, n (%)78 (70.3)40 (70.2)NSThrombocytopenia, n (%)37 (33.3)30 (52.6)AIHA, n (%)16 (14.4)14 (24.6)NSNeurologic, n (%)7 (6.3)9 (15.8)Anti-cardiolipin IgG, n(%)28 (25.2)18 (32.1)NSAnti-cardiolipin IgM, n (%)18 (16.2)18 (32.1)Lupus anticoagulant, n (%)26 (23.4)28 (49.1)Antiphospholipid syndrome, n (%)28 (25.2)30 (52.6)(NS=not significant, APO=adverse pregnancy outcome, AIHA=autoimmune hemolytic anemia)Disclosure of Interests: :None declared

Objectives To determine the association of nailfold videocapillaroscopy(NVC)findings and telangiectasia score with DU history and severity of peripheral vascular involvement(PVI) in Systemic Sclerosis(SSc). Methods Fifty-nine SSc patients fulfilling Leroy and Medsger criteria were evaluated including Telangiectasia Score(TS)(Shah AA et al. J Rheumatol 2010), Modifiye Rodnan Skin Score(MRSS), Valentine Activity Scale(VAS) and Medsger Severity Scale(SS). Qualitative(early, active and late patterns) and semiquantitative assessments [capillary number(CN), irregularly enlarged capillaries(IEC), giant capillaries(GC), capillary ramifications(CR), microhaemorrhages(H), capillary array disorganisation(CAD) and microangiopathy evolution score(MES)]. was performed by NVC(Sulli et al. Ann Rheum Dis 2008) Results The mean age of patients was 45.6 and 91,5% were females. The mean duration of Raynaud’s(RS), non-Raynaud symptoms(NRS), skin involvement(SI)(year) were 6.1±6.5, 3.1±2.0, 3.0±2.0 respectively. Of the patients 20(34%) were diffuse cutaneous,35(59%) were limited and 4(7%) were sine-scleroderma;13(22%) were anti-centromere(+) and 29(49%) were anti-Scl70(+). DU history (DU+) was present in 27(46%) and telangiectases were present in 34(58%). When we compare DU- and DU + groups, the mean score of CN was 1.4±0.7 vs 2.0±0.5*(p Conclusions DU history and severe PVI in SSc was associated with capillary loss and microangiopathy. ‘Early’ NVC pattern was very rare in patients with DU history and was not found in severe PVI. Severe PVI in males was more frequent than females. Telangiectasia scores were not found to be related to PVI. NVC may be a helpful method in the assessment of SSc patients for PVI prognosis, warranting prospective studies. Disclosure of Interest None Declared