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Background:Gastrointestinal involvement(GI) is a common feature of systemic sclerosis(SSc) and can be highly disabling, representing a major cause of morbidity and reduced quality of life(QoL). The impact of dietary habits on GI symptoms, mood and QoL has not been extensively evaluated.Objectives:To evaluate the adherence to the Mediterranean Diet(MD) in an Italian multicenter cohort of SSc patients, and its impact on GI symptoms and other disease features, depression, anxiety and overall QoL.Methods:Consecutive SSc(ACR/EULAR2013) patients from 4 Italian cohorts were enrolled. Dietary habits and adherence to the MD were assessed using the 14-item MEDAS and QueMD questionnaires. Presence and severity of depressive/anxious symptoms and QoL were evaluated with the Hospital Anxiety and Depression Scale(HADS) and the SSc-HAQ(S-HAQ). GI symptoms were assessed with the Reflux Disease Questionnaire(RDQ) and the UCLA SCTC GI Tract 2.0 questionnaire(USG). Associations with patients’ lifestyle, disease characteristics, and nutritional status were explored.Results:265 patients (94.7% females; age 55.8±13.6years; disease duration 9.1±7.0years; diffuse SSc 31.8%; Scl70 + 35.8%;ulcers 23.4%;ILD 29.4%;BMI 23.7±4.4 Kg/m2; obese 11.3%,overweight 23.4%,underweight 4.9%) were enrolled.Overall MD adherence was moderate(7.5±1.9) according to MEDAS and it correlated with QueMD score(4.53±1.96)(R=.371,pGI symptoms were moderate/severe according to USG in 37(14.0%) patients(USG:0.41±0.40), and heartburn was the most common GI symptom(35.2%). The prevalence of significant anxiety and depression was 24.1% and 17.0%.An inverse correlation was found between MD adherence and mood disturbances at HADS(MEDAS; R=-0.181,p=0.04), work impairment(QueMD;R=-0.247,p=0.005) and reduced QoL, both for GI (constipation at USG: R=-0.133,p=0.032) and general S-HAQ items(bowel:R=-0.181,p=0.04;severity R=-0.202,p=0.01;Raynaud:R=-0.217,pConclusion:Unsatisfactory MD adherence is associated with a low mood, impaired QoL, work impairment, GI and vascular symptoms in Italian SSc patients. The promotion of a healthy lifestyle could positively impact on QoL and disease status of SSc patients.References:[1]Gnagnarella P, et a. NMCD 2018. DOI: 10.1016/j.numecd.2018.06.006[2]Jaeger VK et al. PLoS One, 2016.[3]Dinu M et al. Eur J Clin Nutr 2018. doi: 10.1038/ejcn.2017.58.[4]Khanna D, et al.Arthritis Rheum. 2009. doi: 10.1002/art.24730.Acknowledgments:GILSDisclosure of Interests:Giacomo De Luca Grant/research support from: SOBI, Speakers bureau: SOBI, Novartis, Pfizer, MSD, Celgene, Gerlando Natalello: None declared, Giuseppina Abignano: None declared, Corrado Campochiaro Speakers bureau: Novartis, Pfizer, Roche, GSK, SOBI, Duygu Temiz Karadağ: None declared, Maria De Santis: None declared, Elisa Gremese Speakers bureau: Abbvie, BMS, Celgene, Jannsen, Lilly, MSD, Novartis, Pfizer, Sandoz, UCB, Silvia Laura Bosello Speakers bureau: Abbvie, Pfizer, Boehringer, Lorenzo Dagna Grant/research support from: Abbvie, BMS, Celgene, Janssen, MSD, Mundipharma Pharmaceuticals, Novartis, Pfizer, Roche, SG, SOBI, Consultant of: Abbvie, Amgen, Biogen, BMS, Celltrion, Novartis, Pfizer, Roche, SG, and SOBI

Background:Serum autoantibodies closely reflect patterns of organ involvement and disease progression in systemic sclerosis (SSc). The entire autoantibody profile is less well defined in many cohorts and the data regarding their clinical associations and frequencies is limited.Objectives:To determine the autoantibody profile of patients with SSc, as well as their clinical associations, in well-characterized inception- cohort with disease duration less than 3 years.Methods:Serum samples of 100 patients out of 105 enrolled in the study were analyzed for ANA patterns with indirect immunofluorescence (IIF) assay using HEp-20-10/primate liver mosaic IIFT kit. Sera of 96 patients were subjected to commercial line immunoassay to quantify autoantibodies against 13 different autoantigens.Results:92 (92%) out of 100 patients were positive for ANA by IIF (Table 1). The speckled staining was the most pattern followed by nucleolar in 10 patients, centromere in 4, reticular in 1, nuclear in 2 and homogenous in 1. All patients (n= 96) patients were positive for at least 1 autoantibody by immunoblotting (Table 2). Twenty-two (49%) of patients with antiTopo I, 12 (44%) of the patients with antiCENP and 4 (22%) of the patients with antiRNAPIII were single positive. There was no difference in terms of the clinical findings when the patients with single and coexpression of these antibodies were compared. The distributions of the most frequent autoantibodies are shown in Figure 1. Interstitial lung disease was more frequent in the patients positive for anti-Topo I (78.8%) and anti-RNAPIII (27.3%). One of the two patients with breast cancer was anti-RNAPIII positive and none of the patients have diagnosed scleroderma renal crisis. Anti-Topo I was more common in patients with dcSSc (75%) and anti-CENP in lcSSc (46.4%).Table 1.Demographic, clinical and laboratory characteristics of the SSc patients.Sex Female N, %91 (86.7%) Male N, %14 (13.3) Female-to-male ratioAge, mean±SD years48.6±12.7Disease duration, mean±SD years2±1.4Disease classification N, % Diffuse39 (36.5%) Limited65 (62.5%) Sine scleroderma1 (1%)Interstitial lung disease37 (34.3%)Pulmonary arterial hypertension3 (2.8%)Scleroderma renal crisis0Digital ulcer14 (13.3%)Raynaud phenomenon105(100%)Telengiectasia31 (28.8%)Calcinosis1 (1%)Malignancy3 (2.9%)Antinuclear antibody profile N*, % Positive92 (92%)Staining pattern Speckled65 (65%) Nucleolar13 (13%) Centromere29 (29%) Homogeneous3 (3%) Reticular3 (3%)Table 2.Numbers and combinations of autoantibodies identified in the 96 SSc patients.Topo-ICENPRNAP IIIFibrillarinNOR90Th/ToPm/SclKuPDGFRRo52Topo-I22170159507CENP12202222011RNAPIII40121307Fibrillarin0000000NOR90011002Th/To04202Pm/Scl4306Ku102PDGFR00Ro522Single positive221240004102Total452718058179024Figure 1.Diagram of disease-related antibodies against the four main autoantibodies [anti-centromere (antiCENP) anti-Topoisomerase I (antiTopo I), anti-RNA polymerase III (antiRNAP III) and anti-Ro52).Conclusion:We presented the clinical and serologic features of the Turkish SSc patients from a new inception cohort. Clinical features of the SSc patients with single or multiple antibody positivity were not different.References:NoneDisclosure of Interests:None declared

Background:The modified Rodnan skin score (mRSS) is the current gold standard for skin assessment in systemic sclerosis (SSc) both in clinical trials and practice. High frequency ultrasound (HFUS) has been suggested to offer a quantitative assessment of skin thickness in SSc by several studies, however results are inhomogeneous with regards to the machine used, number of imaged sites, as well as the various stages of skin involvement.Objectives:Aim of this cross-sectional study was to compare performance of HFUS in the assessment of skin involvement in diffuse cutaneous SSc (dcSSc) patients, at different disease stages, as compared with healthy controls (HC).Methods:Dorsal finger, hand, forearm and upper arm skin of consecutive dcSSc patients, at different disease stages, and of matched-HC were scanned bilaterally using HFUS. Two investigators, expert in MSK ultrasound, blinded to the clinical details, measured skin thickness using Esaote MyLab70 equipped with a 22 MHZ probe. Clinical involvement was assessed by a blinded operator using the mRSS and results were compared with imaging data. Statistical analysis was performed using GraphPad Prism software V.7.0.Results:A total of 704 HFUS images were obtained from 22 dcSSc patients [20 Female, mean age 49 (±11) years, 12 with ≤ 5 years disease duration] and 22 HC [20 Female, mean age 50.7 (±6.7) years]. Skin thickness was significantly higher in SSc patients than in HC at fingers (p0.05). HFUS showed a good discriminative ability between SSc and HC skin at fingers and hands (AUC 0.91, 0.81, 0.6 and 0.65 for fingers, hands, forearms and upper arms respectively). When analysing the subgroup of SSc patients with ≤5 years disease duration, HFUS showed a slightly lower performance in discriminating between SSc without clinical skin involvement (site mRSS=0) and HC (AUC 0.68, 0.57, 0.68 for hands, forearms and upper arms respectively). Mean HFUS skin thickness significantly correlated with mRSS at site of analysis (hand: r=0.78, p=0.05). Interobserver reliability for skin thickness was good to excellent at all sites with intraclass correlation coefficient ranging between 0.79 and 0.94.Conclusion:HFUS of the skin is a reliable measure of skin involvement in SSc. Studies with higher number of patients with different clinical features are needed to explore the potential of HFUS to discriminate between healthy and SSc skin, including sites at a preclinical stage of involvement.Disclosure of Interests:None declared

Background Familial Mediterranean Fever (FMF) is an autosomal recessive genetic disorder that causes recurrent episodes of fever, poliserositis, arthritis, skin eruptions. Objectives In this study, we aim to present clinical and demographic features of FMF patients followed up in our clinic. Methods The clinical, demographic, genetic features and managements of 402 FMF patients (fulfilling Tel-Hashomer Diagnostic Criteria) were analysed. Results The mean age was 36.8±11.2 years, mean diagnosis age was 28±11.9 years, and mean disease duration was 189.2±124.5 months, mean duration between onset of disease and onset of treatment was 93.6±104 months. Consanguineous marriage was detected in 7%29 patients. Fever and abdominal pain both were initial symptoms in 72% of the patients, while 7% of them had chest pain, 4% had only fever, 15% had arthritis, 1% had erysipel-like erythema and 1.5% had inflammatory back pain as the first symptom of FMF (table 1). Eight patients (2%) were suffered from chronic kidney disease and 2 of them were on dialysis programme. Amyloidosis were identified in 14 patients (3.5%) with biopsy. At least one mutation of MEFV gene was detected in 78% patients There was no mutation in 8% patients. In 15% patients, MEFV gene analysis could not be done. The most frequent mutation was M694V mutation and its allel frequency was 54%; the frequency of V726A, M680I, E148Q, R761H and A744S allels mutation were 11%, 7%, 7%, 2%, 1%, respectively. The frequency of compound mutation was 38%, and the most common compound mutation was M694V+R202Q (11%). There was significantly relationship between M694V mutation and arthritis, erysipel-like erythema, proteinuria, sacroiliitis(table 2). Amyloidosis was more frequent in patients who had M694 homozygous mutation. Mean age of disease onset was lower in patient who had M694V homozygous mutation than M694V heterozygous mutation (p Conclusions Tight control and sustained management are important in FMF to protection from amyloidosis. Similar to literature, the most frequent mutation was M694V mutation, and there was significantly relationship between M694V mutation and arthritis, erysipel-like erythema(1), proteinuria, sacroiliitis(2) in our study. References [1] Dewalle M, Domingo C, Rozenbaum M, et al. Phenotype-genotype correlation in Jewish patients suffering from familial Mediterranean fever (FMF). Eur J Hum Genet1998;6:95–7. [2] Akkoc N, Sari I, Akar S, et al. Increased prevalence of M694V in patients with ankylosing spondylitis: additional evidence for a link with Familial Mediterranean fever. Arthritis Rheum2010;62(10):3059–63. Acknowledgements None Disclosure of Interest None declared

Purpose Anti-C1q has been shown to be associated with systemic lupus erythematosus (SLE) and disease activity of lupus nephritis in previous studies. We studied anti-C1q specificity for SLE versus rheumatic disease controls and healthy controls and the association with SLE manifestations in a single centre cross-sectional study. Methods Demographics, disease information and blood samples were obtained during routine follow-up visits of patients attending Kocaeli University rheumatology outpatient clinic. There were 150 SLE patients (92% female, mean: 46 years). Control group had 85 rheumatoid arthritis patients, 16 patients with other diseases (Sjogrens’s syndrome, systemic sclerosis, adult onset Still disease, psoriatic arthritis) and 49 healthy persons (88% female, mean: 45 years). Anti-C1q was measured by ELISA according to manufacturer’s instructions. Results In SLE group 72 patients had renal, 10 patients had neurologic disease, 25 patients had antiphospholipid syndrome (APS). Anti-dsDNA was positive in 92 patients, anti-Sm was positive in 18 patients, 83 patients had low complement levels. Prevalence of anti-C1q was 5% (8/150) in patients with SLE and 1% (2/150) in controls (p=0.88). There was no correlation between clinics (renal, neurologic, hematologic, mucocutanous disease, arthritis, serositis, APS) or laboratory findings (Anti-dsDNA, anti-Sm, low complement levels, direct Cooms’ test). Within anti-C1q positive group 6 patients had renal disease, 4 had SLEDAI scores 4 or more. None of them had nervous system disease. Six patients had leukopenia, 2 had thrombocytopenia, 5 had mucocutanous disease. Laboratory findings were as follows; 6 patients had anti-dsDNA, 1 had anti-Sm antibody, 6 had low complement levels. Conclusions Even though there were studies showing the relationship between anti-C1q antibodies and renal disease, it was shown that most of the patients with antibody positivity were Asian ethnicity and younger than 30 years of age. Furthermore anti-C1q antibody was related to disease activation and eliminated within 3 months of treatment. There were also different commercial products available with different cut-off levels. Meta-analysis have shown although anti-C1q antibodies are associated with lupus nephritis, the post-test probabilities are not sufficient to provide certainty of the presence or absence of history of disease.

Background The Health Assessment Questionnaire (HAQ) is one of the main instruments for assessing disability in rheumatic diseases.The Scleroderma HAQ (S-HAQ) combines the HAQ with five systemic sclerosis (SSc) related visual analogue scales for Raynaud9s phenomenon (RP), digital ulcers, digestive symptoms, pulmonary symptoms, and overall disease severity. Objectives To perform cross-cultural adaptation and validation of the S-HAQ in patients with SSc. Methods Sixty patients who fulfilled the 2013 ACR/EULAR Classification Criteria for Systemic Sclerosis ACR criteria for SSc were were recruited.Fifty three percent of the patients fulfilled the criteria for limited systemic sclerosis (lcSSc).We evaluated test–retest reliability using the intraclass correlation coefficient (ICC); known-groups construct validity by stratifying patients according to severe organ involvement; and convergent validity using Spearman9s correlation with mental and physical components of Short Form 36 version 2 (SF36v2). Results Eighty percent of the patients were female and the median age was 56 years old.Finger tip ulcers were observed in 16 patients (26.7%) and severe organ involvements were detected as intersititial lung disease in 32 (53%) and pulmonary hypertension in 5 (8%) of the patients.HAQ Disability Index (HAQ-DI), digestive VAS, pulmonary VAS, digital ulcer VAS, Raynaud9s phenomenon VAS and overall disease severity VAS demonstrated high reliability (ICCs=0.819–0.908).The HAQ-DI showed higher correlation coefficients with physical function and role physical components of physical-related scores (r=-0.537 and – 0.453, respectively); social function and role emotional components of mental-related dimensions (r= -0.470 and -0.328, respectively) in the SF-36.Two among five SSc related VAS (VAS digital ulcer and VAS overall disease severity) were highly correlated with HAQ-DI (r=0.527 and 0.481 respectively).The instruments could not discriminate between severe organ involvements and both subsets of SSc. Conclusions The Turkish version of the S-HAQ meet the requirements of reproducibility and validity.More studies are needed to examine the capacity of these instruments to discriminate between severe involvements and disease subsets.This study forms the basis for future studies to evaluate the properties of S-HAQ in Turkish speaking SSc patients more extensively. References R Hesselstrand et al, Psychometric properties of the Swedish version of the Scleroderma Health Assessment Questionnaire and the Cochin Hand Function Scale in patients with systemic sclerosis, Scandjrheumatol Vol. 42, Iss. 4,2013. Disclosure of Interest None declared

Background Systemic lupus erythematosus (SLE) is an autoimmune disease with diverse clinical manifestations. Objectives Here, we present 300 patients with SLE attending our clinic between 2001 and 2017. Methods Demographics, clinics, laboratory findings, Systemic Lupus International Clinics (SLICC)/American College of Rheumatology (ACR) damage index scores and treatments were analyzed. Diagnosis was confirmed with 1997 ACR (1) or 2012 SLICC (2) classifications. Descriptive statistical tests were used for analysis. Results Demographics and clinical characteristics are presented in Table 1. Anti-dsDNA was positive in 185 (61.6%), anti-Sm was positive in 35 (11.6%), anti-phospholipid antibodies was positive in 67 (22.3%), direct Coombs test was positive in 69 (23%) patients. Complement levels were low in 171 (57%) patients. In patients with renal disease, class IV lupus nephritis was the most common form (37 patients [12%]) followed by class II nephritis (34 patients [11%]). Forty-six patients had antiphospholipid syndrome. Treatments patients ever received are presented in Table 2. Cyclophosphamide treatment was given mostly for renal disease. Within 22 patients receiving rituximab, 2 had thrombocytopenia and 1 had hemolytic anemia unresponsive to other treatments, 1 had protein-losing enteropathy and 1 had lupus enteritis; the rest had lupus nephritis. One patient received intravenous immune globulin (IVIG) for severe neutropenia and 2 patients for severe thrombocytopenia. One patient received plasmapheresis for vasculitis. One patient received IVIG, plasmapheresis, rituximab for severe renal failure, alveolar hemorrhage requiring intensive care unit admission and 1 patient received same treatment for severe unresponsive hemolytic anemia. Conclusions In long term, SLICC/ACR damage index was highest in patients received pulse steroid for renal disease (min:0, max:5). Four (1.3%) patients had pulmonary hypertension, 31 (10.3%) had avascular necrosis, 24 (8%) had viral infections requiring treatment or bacterial infections requiring admission due to immunosuppression, 12 (4%) had malignancy. SLE is an autoimmune disease requiring multi-faceted approach. References ) Hochberg MC: Updating the American College of Rheumatology revised criteria for the classification of systemic lupus erythematosus. Arthritis Rheum. 40:1725 1997. ) Petri M, Orbai AM, Alarcon GS, et al.: Derivation and validation of the Systemic Lupus International Collaborating Clinics classification criteria for systemic lupus erythematosus. Arthritis Rheum. 64:2677–2686 2012. Disclosure of Interest None declared

Background and aims Systemic lupus erythematosus (SLE) is an autoimmune disease with diverse clinical manifestations. Here, we present 150 patients with SLE attending our clinic between January and November 2016. Methods Demographics, clinics, laboratory findings, Systemic Lupus Erythematous Disease Activity Index (SLEDAI), Systemic Lupus International Clinics (SLICC)/American College of Rheumatology (ACR) damage index scores and treatments were analysed. Diagnosis was confirmed with 1997 ACR or 2012 SLICC classifications. Chi-squared or Fisher’s exact tests were used for statistical analysis. Results General characteristics are presented in Table 1. Clinics are presented in Table 2. Treatments patients ever received are presented in Table 3. Conclusions SLICC damage was positive in patients receiving pulse steroids (57%), cyclophosphamide (51%), rituximab (73%). In long term, 3 (2%) patients had pulmonary hypertension, 21 (14%) had avascular necrosis, 6 (4%) had malignancy. SLE is an autoimmune disease requiring multi-faceted approach.