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Summary We focussed on evaluating the protective effect of lycopene and resveratrol on post‐thaw bull sperm and oxidative stress parameters. Nine ejaculates for each bull were used in the study. Each ejaculate, splitted into three equal aliquots and diluted at 37 °C with base extenders containing lycopene (1 × 10−3 g ml−1) and resveratrol (1 mm), and no antioxidant (control), was cooled to 5 °C and then frozen. Frozen straws were thawed in a water bath for evaluation. The supplementation of the semen extender with lycopene and resveratrol increased the percentages of post‐thawed computer‐assisted sperm analysis (CASA) motility (55.8 ± 3.8 and 61.9 ± 4.0%) and progressive motility (38 ± 2.4 and 37 ± 8.8), compared with the controls (50.7 ± 2.65 and 33.3 ± 3.74%, respectively, P < 0.05). Resveratrol provided a higher ALH (4.3 ± 0.1), in comparison with the control (3.9 ± 0.3, P < 0.05). The supplementation of the semen extender with lycopene and resveratrol produced a higher mitochondrial activity (24.6 ± 2.9 and 30.1 ± 6.5% respectively), compared with that of the control (11.8 ± 9.5%, P < 0.05). It was determined that both antioxidants resulted in a lower percentage of sperm with damaged DNA than that of the control (P < 0.05). Sperm motion characteristics except for ALH, acrosome integrity, sperm viability and oxidative stress parameters were not affected by the adding of lycopene and resveratrol.

Background:Nailfold videocapillaroscopy (NVC) is a noninvasive diagnostic method to evaluate periungual microcirculation including capillary density and morphology. To date, several studies have demonstrated the diagnostic and prognostic role of NVC, especially in systemic sclerosis and Raynaud phenomenon. Capillary alterations have been described in patients with inflammatory myopathy (IM), but their frequency, typical features, and possible associations with clinical and serological features are not well defined.Objectives:Our study aimed to describe the capillaroscopic features of the patients with IM and their relationship with clinical and serological features.Methods:This cross-sectional study included 63 consecutive IM patients (37 females) during a 24-month period between December 2021 and December 2023. All patients were evaluated with NVC to analyze the capillary number and morphological abnormalities. Stored NVC images were analyzed later by a blinded researcher. Cumulative clinical and serological features were extracted from the medical records.Results:Demographics, clinical and serological characteristics of the patients were summarized in Table 1. Patients with dermatomyositis (DM)(including amyopathic subgroup) had fewer capillaries and more frequent enlarged capillaries, capillary disorganization, and avascular areas than the myositis patients without skin findings. When patients were analyzed by autoantibody profile, severe NVC abnormalities (capillary reduction, dilated and elongated capillary loops, avascular areas and capillary disorganization) were significantly more common in only anti-TIF1γ positive patients (Table 2). Univariate regression analysis revealed that the presence of anti-TIF1γ or DM was associated with capillary number of ≤7 [OR=4.83 (CI95% 1.22-19.13), p=0.025 or OR=9.25 (CI95% 1.10-77.64), p=0.040], avasculaer areas [OR=7.25 (CI95% 1.67-31.52), p=0.008 or OR=9.26 (CI95% 1.10-77.64), p=0.040] and capillary disorganisation [OR=5.20 (CI95% 1.22-22.32), p=0.026 or OR=5.67 (CI95% 1.12-28.87, p=0.037]; after the multivariate analysis these parameters were still significantly associated with anti- Anti-TIF1γ positivity [OR=4.73 (CI95% 1.16- 19.22), p=0.030; OR=7.26 (CI95% 1.61-32.86), p=0.010; OR=5.21 (CI95% 1.15-23.48), p=0.032, respectively]. Presence of giant capillaries (capillary loop diameter >50 µm)(p=0.047) or microhemorrhage (p=0.004) was frequent in patients with uncontrolled disease activity (n=23, 6 of the patients were newly diagnosed).Conclusion:Capillary rarefaction, dilatation, elongation and disorganization were severe NVC abnormalities commonly seen in DM patients and predominatly associated with anti TIF1γ antibodies in patients with IMs. Giant capillaries and hemorrhages were frequent in patients with active disease. The role of microangiopathy in the immunopathogenesis of skin involvement and the pathogenic effects of anti TIF1γ antibodies may be an interesting research topic. NVC may help distinguish some patients among patients with IM in the future with longitudinal studies conducted with larger cohorts and can be used in disease monitoring.REFERENCES:NIL.Acknowledgements:NIL.Disclosure of Interests:None declared.

BackgroundBehçet’s disease (BD), a multisystem inflammatory disorder of unknown etiology, is classified as a variable vessel vasculitis affecting all types and sizes of blood vessels; and a tendency for thrombosis in association with inflammatory endothelial activation is an important characteristic of BD vasculitis. Recent studies suggest an association between ABO blood groups and vascular disease in particular in those carrying non-O (A, B, and AB) groups [1].ObjectivesWe aimed to investigate the potential contribution of ABO blood groups to the risk of vascular involvement in patients with BD.MethodsWe retrospectively analyzed the records of BD patients followed between 1978 and 2022. Patients fulfilling the ISG Criteria for diagnosis of BD were screened, and those with information about ABO blood groups were included into the study. Presence or absence of vascular involvement and its features were recorded using a standard form. The chi-square test, t-test, Mann-Whitney U test, and logistic regression tests were used for statistical analyses.ResultsThe study group consisted of 411 patients with available blood group data, and 143 (34.8%) were carrying O [58% men, mean age at diagnosis 31.4±10.1 years, median follow-up period 153 (98-219) months], and 268 (65.2%) were carrying non-O blood groups [60.1% men, mean age at diagnosis 30.7±8.4 years, median follow-up period 148 (92-204) months]. There was no statistical significance between O and non-O groups in regard to the potential confounding factors affecting the risk for vascular disease, including sex, age at diagnosis, family history, HLA-B51 positivity, smoking, comorbidities, and prothrombotic mutations. Vascular involvement was observed in 39 (27.3%) patients with blood group O [venous in 35 (24.5%), and arterial in 11 (7.7%)], whereas 109 patients (40.7%) with non-O blood groups had vascular involvement [venous in 95 (35.4%), and arterial in 38 (14.2%)]. The frequencies of total vascular and venous involvements between the two groups were significantly different (p=0.007, p=0.023, respectively). Unadjusted and adjusted ORs with different models in the multivariate logistic regression analyses are shown in Table 1. After adjustments for age, sex and comorbidities, the risk for arterial disease was also found to be increased in association with non-O blood groups.ConclusionVascular involvement with a tendency for thrombosis is an important feature of BD, and inflammatory characteristics resulting in endothelial dysfunction have been considered as the main underlying pathology. The results of the preliminary study supports the previous reports revealing the potential contribution of ABO blood groups in the development of vascular disease and suggest an approximately two-fold increased risk for vascular involvement in BD patients.Reference[1]Vasan SK, et al. ABO Blood Group and Risk of Thromboembolic and Arterial Disease: A Study of 1.5 Million Blood Donors. Circulation. 2016;133(15):1449-1457.Table 1.Logistic regression analysis to estimate unadjusted and adjusted risk for vascular events comparing O with non-O blood groups. Model 1: ABO blood group, Model 2: Model 1 plus age at diagnosis and sex, Model 3: Model 2 plus comorbidities, Model 4: Model 3 plus malignancy, Model 5: Model 4 plus smoking (missing value for 84 patients).Total vascular involvementVenous involvementArterial involvementp-valueOR (CI %95)p-valueOR (CI %95)p-valueOR (CI %95)Model 10.0071.8 (1.2-2.8)0.0231.7 (1.1-2.7)0.0572.0 (0.9-4.0)Model 20.0071.9 (1.2-3.1)0.0241.7 (1.1-2.8)0.0622.0 (0.9-4.0)Model 30.0052.0 (1.2-3.2)0.0221.8 (1.1-2.9)0.0382.2 (1.0-4.7)Model 40.0062.0 (1.2-3.2)0.0231.8 (1.1-2.9)0.0462.2 (1.0-4.6)Model 50.0291.9 (1.1-3.2)0.0381.8 (1.0-3.2)0.1022.0 (0.9-4.5)Acknowledgements:NIL.Disclosure of InterestsNone Declared.

Background:There is limited information on how the combination of antibodies other than anti-centromere (ACA) and limited cutaneous systemic sclerosis (lcSSc) will affect the distribution of future organ involvement and mortality.Objectives:We aimed to evaluate the prevalence and progression of the interstitial lung disease(ILD) and pulmonary hypertension(PH) in patients with lcSSc according to the presence of antibodies (ANA only vs anti-scl70 vs ACA).Methods:The medical records of 210 SSc patients who were regularly followed-up between 1988-2021 were screened, the data of 160 patients with limited cutaneous SSc (lcSSc) were extracted and 155 lcSSc patients (145 females, 93.5%) with existing autoantibodies were included into this retrospective analysis.Results:Mean age, duration of Raynaud’s and non-Raynaud’s were 54.2(±12.5), 15.7(±11.2) and 12.8(±7.5) years, in 155 lcSSc patients with a mean follow-up of 96.2(±68.8) months. ANA was detected in 49 (31.6%) (patterns of speckled in 42.9%, nucleolar in 10.2%, homogeneous in 16.3%, nucleolar + speckled or +homogenous in 18.4%, undefined in 12.3%), anti-Scl70 in 62(40%), and ACA in 44(28.4%). Twenty-five patients (16.1%) progressed to dcSSc during the follow-up in 33.4(±49.5) of months. LcSSc patients with anti-Scl70 more frequently progressed to dcSSc (22 vs 14.3 and 9.1% for ANA and ACA groups). ACA(+)’s less frequently received immune-suppressives (43.2 vs 89.8 and 85.5%, p=0.002). ACA(+) patients who progressed to dcSSc had more frequent initial and cumulative ILD when compared to those did not progressed (p= 0.036 and p=0.032). The frequency of initial and cumulative ILD or worsening ILD was highest in anti-Scl70(+)’s followed by ANA(+)’s and then ACA(+)’s (p=0.003 and p=0.005 or p=0.02, respectively) in lcSSc patients who were not progressed to dcSSc. Although not significant the progression of ILD took longer to occur in ACA(+)’s (med 118 vs 47 and 69 months). Cumulative PH groups were as follows; PAH in 4 patients with ACA(lcSSc), group 3-PH in 2 patients with ANA only (lcSSc), 5 patients with anti-scl70 (n=3, dcSSc) and 3 with ACA (lcSSc)(Table 1). ANA(+) 4 patients (at 36., 39.,48., and 120. months), anti-Scl70(+) one patient (at 235. month) and ACA(+) one patient (at 132.month) deceased during the follow-up period.Conclusion:LcSSc patients with anti-Scl70, tended to progress to dcSSc more frequently. Progression to dcSSc was associated with higher frequency of ILD in ACA(+) patients. Anti-Scl70 or ANA positivity was associated with a higher frequency ILD and progression compared to ACA positivity. Grup 1 PH was diagnosed only in ACA(+)’s, group3-PH was seen in all autoantibody groups. In lcSSc, mostly autoantibodies determine the course of the disease; the potential for progression to dcSSc and major organ involvement should be monitored in high risk patients.REFERENCES: NIL.Acknowledgements:NIL.Disclosure of Interests:Yasemin Yalçinkaya Boehringer Ingelheim, Melodi Gizem Can: None declared, Büşra Demir: None declared, Bahar Artim-Esen: None declared, Ahmet Gül: None declared, Murat Inanc Boehringer Ingelheim.

PurposeTo evaluate the topographic, tomographic, and densitometric properties of patients with pellucid marginal degeneration (PMD) and inferior keratoconus.Patients and methodsRetrospective, comparative case series. Forty-seven eyes of 32 patients with crab claw patterns were identified from 2751 patients with corneal ectasia. They were divided into two groups, inferior keratoconus and PMD, based on clinical findings. The topographic, tomographic, and densitometric measurements were analyzed.ResultsPMD was detected in 11 eyes of eight patients (mean age 50.2±11.1 years), and inferior keratoconus was detected in 36 eyes of 24 patients (mean age 34.7±10.1 years). The control group consisted of 40 patients (33.1±4.6 years). The thinnest corneal point and maximum anterior and posterior elevation points were located lower in the PMD than in the inferior keratoconus (P<0.01). In the PMD, all deviation indices were higher than the controls (P<0.01), whereas the deviation indices, except Dt (P=0.960), were lower than the inferior keratoconus (P<0.01). The densitometry values of PMD were significantly higher than those of the controls in all zones and layers (P<0.01) and significantly higher than the densitometry values of inferior keratoconus in the 6-10 and 10-12 mm zones (P<0.05).ConclusionThere is a higher probability of a patient with crab claw pattern on the topography of having inferior keratoconus than having PMD. Therefore, analyzing only the anterior corneal surface is not sufficient in differential diagnosis. Tomographic and densitometric evaluations may facilitate the differential diagnosis.

BackgroundIn systemic sclerosis (SSc), studies showed that permenant organ damage may exist very early in the disease course.ObjectivesTo reveal the differences between diffuse cutaneous and limited cutaneous SSc (dcSSc and lcSSc) patients regarding the progress of disease related organ damage over time.MethodsMedical records of consecutive 210 patients(91.9% women) who fulfilled SSc classification criteria and had a follow-up period of at least 12 months were retrospectively evaluated. ‘Scleroderma Clinical Trials Consortium Damage Index‘ score* was calculated initially and after a follow-up period of 101±70 months in dcSSc and lcSSc patients.ResultsIn dcSSc; joint contractures, low BMI/weight loss, and dependence on oxygen, ILD with>20% extent on HRCT and FVC<70% were more frequent at follow-up, total damage scores were higher at baseline and follow-up (3.1±2.8 vs 1.9±2.5,p=0.004 and 9.7±5.9 vs 5.8±4.6,p<0.000) (Table 1, Figure1). In dcSSc; presence of puffy fingers was associated with initial respiratory damage (OR:3.88,p=0.008) and ILD with musculoskeletal-skin damage at follow-up (OR=3.547, p=0.02). In lcSSc; follow-up period of >5 years was associated with musculoskeletal-skin damage (OR=10.17,p=0.002), presence of skin thickening proximal to the MCP with peripheral vascular damage (OR=8.48,p=0.000 and OR=2.87,p=0.047), presence of anti-Scl70 with respiratory damage (OR=4.84, p=0.008), presence of ILD, DU and follow-up period of >5 years with GIS damage(OR=2.48,p=0.001, OR=1.79,p=0.029 and OR=5.77,p=0.000) at follow-up. In lcSSc, the presence of DU and ILD were associated factors with moderate damage (6-12 points)(OR=2.41,p=0.000 and OR=1.94,p=0.006), telangiectasias and PAH with high damage (≥13 points) (OR=2.42,p=0.041 and OR=2.87,p=0.004) at follow-up. Of the patients 4.3%(n=9) died during follow-up; cardiovascular damage (baseline and follow-up) was associated with mortality (5.5% vs.55.6%, p<0.000, and 15.4% vs.55.6%, p=0.009).ConclusionIn SSc patients, the frequency and severity of organ damage were increased in both cutaneous subtypes during 8 years of follow-up. Presence of ILD, DU, telangiectasia and PAH and long duration of follow-up period were prominent factors associated with damage. Cardiovascular damage was found to be associated with mortality. The identification of risk groups in different cutaneous subtypes of SSc and early efficacious treatment strategies could improve survival.Reference[1]Ferdowsi N, et al. Ann Rheum Dis. 2019;78:807-816.Table 1.Frequency of Some Disease Related Organ Damage Parameters in SSc PatientsBaselineLastDAMAGE PARAMETERSlcSSc (n=160)dcSSc (n=50)lcSSc (n=160)dcSSc (n=50)Small joint contractures12 (7.5)7 (14)29 (18)20 (40) p=0.004Large joint contractures1 (0.6)2 (4)7 (4.4)7 (14) p=0.025Digital ulcer19 (12)11 (22)60 (38)28 (56)Esophageal dysmotility13 (8)3 (6)44 (28)17 (34)Low BMI/ weight loss7 (4.4)2 (4)15 (9)12 (24) p=0.015Refractory GERD30 (19)9 (18)105 (66)37 (74)HRCT>20%28(18)19 (38) p=0.00566 (40)41(82) p=0.000HRCT>20% and FVC<70%6 (4)7 (14) p=0.01327 (17)24(48) p=0.000Dependence on home oxygen0 (0)0 (0)2 (1.3)4 (8) p=0.030PAH5 (3.1)3 (6)14 (8.8)5 (10)Right ventricular dysfunction0 (0)0 (0)7 (4.4)4 (8)Myocardial disease0 (0)1 (2)4 (2.5)4 (8)Figure 1: Progress of total damage scores in SSc patients with different cutaneous subtypesAcknowledgements:NIL.Disclosure of InterestsNone Declared.

BackgroundSome specific autoantibodies were known to be associated with cutaneous subtype, type of organ involvement and prognosis in systemic sclerosis (SSc).ObjectivesThe aim of our study was to evaluate the impact of autoantibody profile on the course of SSc regarding new organ involvement over time.MethodsThe data of 206 consequitive patients (189 women) with SSc who were followed up at least 12 months were retrospectively analyzed. Associations between the autoantibodies (anti-nuclear antibody (ANA) (27.7%, n=57), anti-Scl70 (47.1%, n=97) and anti-centromere (ACA) (22.3%, n=46)) and disease-free survival (without new organ involvement) were evaluated in SSc patients with a mean follow-up time of 101.26±71.02 months.ResultsAnti-Scl70 was associated with diffuse cutaneous disease involvement, digital ulcers, arrhythmia, and interstitial lung disease (ILD) (OR:3.43, p<0.001, OR:1.835, p=0.033, OR:4.06, p=0.038 and OR:2.393, p=0.006, respectively). ACA was associated with PAH (OR:3.319, p=0.040) and protective against diffuse skin involvement, flexion contractures and ILD (OR:0.203, p=0.001, OR:0.251, p=0.012 and OR:0.288, p=0.008, respectively).In ACA (+) group, new organ involvement was observed less frequently (HR: 0.523 (95% CI (0.29-0.94), p=0.030) and after a longer time period when compred to ACA (-) group (82.65±14.5 vs 53.98±5.49 months, p=0.026). At the 5th and 8th years of the follow-up, half of the ACA(+) patients did not develop new organ involvement. In the ACA(-)[(ANA(+) or antiScl70(+)] group, new organ involvement was detected in >67% within 5 years and in >80% within 8 years (figure-1, table-1).Figure 1.Cumulative risk of the new organ involvement for different systemic sclerosis patient groups according to autoantibody profile over time.Table-1Time to New Organ Involvement and Disease-Free Survival according to Autoantibody Profile in SSc PatientsTime to New Organ InvolvementDisease-Free Survival without New Organ InvolvementMean (months)CI 95%2.year5.year8.yearACA(+) (n=17)82,65±14,554,25±111,1%76.5(±10.3)%52.9(±12.1)%47.1(±12.1)ANA(+)-ENA(-)(n=18)*52,06±9,3133,82±70,29% 61.1(±11.5)%33.3(±11.1)%16.7(±8.8)Anti-scl70(+) (n=36)54,95±6,8841,45±68,44%72.2(±7.5)%38.9(±8.1)%16.7(±6.2) ACA(-)(n=54)^53,98±5,4943,22±64,75%68.5 (±6.3)%37.0 (±6.6)%11.1 (±4.3)* p=0,026, ^p=0,026 compared to ACA(+) patients, Log Rank (Mantel-Cox)testConclusionAlthough ACA(+) patients are considered to have a better prognosis, some patients may develop new or worsening manifestations after a longer time period. The prognosis of ANA or anti-Scl70(+) patients was shown to be more severe and most patients had new organ involvement within the first 5 years of the disease, warranting a careful evaluation and efficacious treatment at this period.REFERENCES:NIL.Acknowledgements:NIL.Disclosure of InterestsNone Declared.

Axial spondyloarthritis (axSpA) is a chronic inflammatory disease involving the axial skeleton and commonly include some coexisting extra-axial manifestations (EAMs) [1] which may have an influence on prognosis and treatment decisions [2]. We aim to show the characteristics of EAMs in patients with axSpA and to assess the progress under different therapeutic agents. In this retrospective cohort study; medical records of all patients with axSpA who fulfilled ASAS classification criteria and were followed up between 1980 to 2022 at our tertiary referral outpatient clinic were screened. The data of 201 (59.7% males) patients who had sacroiliitis (by using X-Ray or MRI) and coexisted EAMs was recorded into a pre-defined protocol. Demographics and progress of EAMs under different therapeutic agents were analysed. The mean age and duration of follow-up period were 34 years (IQR 25-43) and 144 months (IQR 84-213). Prevalence of family history and HLAB27 were 30.6% (38/124) and 77.4% (65/84). Non-radiographic sacroiliitis was observed in 21.9%. Peripheral arthritis (60%) was the most common EAM and followed by uveitis (46%), IBD (17%), psoriasis (14%), enthesitis (13%) and dactylitis (5%). Demographics and treatment details are shown in Table 1 and Figure 1. Approximately, half of the patients were treatment naive at onset of each EAMs. Anterior uveitis and psoriasis were frequently occured despite using bDMARDs (31% and 39%). Fifty nine percentage of the patients with psoriasis and one third of patients with uveitis and dactylitis required a switch to bDMARDs. In patients with axSpA, some of EAMs required treatment switches and relapses occured despite treatment optimization. Determining appropriate and efficaceous treatment algorithms according to presence of EAMs should be one of the main goals of axSpA management strategies. [1]Navarro-Compán V, Sepriano A, El-Zorkany B, van der Heijde D. Axial spondyloarthritis. Ann Rheum Dis. 2021 Dec; 80: 1511-21. [2]Ward MM, Deodhar A, Gensler LS, et al. 2019 Update of the American College of Rheumatology/Spondylitis Association of America/ Spondyloarthritis Research and Treatment Network Recommendations for the Treatment of Ankylosing Spondylitis and Nonradiographic Axial Spondyloarthritis. Arthritis Care Res (Hoboken) 2019;71:1285-99. NIL. None Declared. [Display omitted] Table 1Demographics and treatment details in patients with axSpA and coexisted axtra-articular manifestationsArthritis(n=122)Uveitis(n=94)IBD(n=35)Psoriasis(n=29)Enthesitis(n=27)Dactylitis(n=10)Prevalence up the diagnosis of axSpA, %40.621.913.31335.750Duration of onset of EAMs' to the diagnosis of AxSpA, months, med (min-max)0(-324-324)0(-192-528)-12(-276-144)-12(-312-180)0(-12-24)12(0-276)Simultaneous axial activity, %63.743.818.230.87566.7Treatment at onset of EAMsS, %Naive57.647.257.953.846.757.1NSAID5.97.55.3013.30cDMARD22.415.121.17.733.328.6bDMARD14.130.215.838.56.714.3Treatment change for EAMs, %Unchanged3.411.552500Switch to NSAİD3.49.6006.70Switch to cDMARD64.840.45516.773.366.7Switch to bDMARD28.438.54058.32033.3Response to first treatment agent without relapses, %NSAİD6740--0-cDMARD543055506775bDMARD56635757100100(AxSpA: axial spondyloarthritis, EAM: extra-axial manifestation, IBD: inflammatory bowel disease, NSAID: nonsteroid anti inflammatory drugs, cDMARD: conventional disease modifying anti-rheumatic drugs, bDMARD: biologic DMARD)

Background:Cardiovascular events have a high impact on morbidity and mortality in lupus disease. Eary-onset subclinical vascular lesions show faster progression in systemic lupus erythematosus (SLE) patients. Besides traditional and SLE-spesific cardiovascular risk factors, development of dysfunctional HDL plays a pivotal role in pathogenesis of accelerated atherosclerosis and correlates with indicators of atherosclerosis like plaque and intima-media thickness [1]. SLE patients with nephritis have increased plaque prevalence. However, studies on markers of dysfunctional HDL and its relation to plaque in different SLE subgroups are limited.Objectives:We aimed to investigate the relationship between markers of dysfunctional HDL (Pon-1, Apo-A1, Anti-Apo A1) and subclinical atherosclerosis using carotid and femoral Doppler ultrasonography in a subgroup of SLE patients with nephritis.Methods:68 patients with SLE nephritis (class 4 and 5), 45 non-nephritis SLE patients and 36 healthy volunteers were included in the study. Total 139 participants underwent carotid and femoral Doppler ultrasonography and echocardiography. Serum levels of Pon-1, Apo-A1, Anti-ApoA1 were tested using ELISA.Results:The average age was 43,08±9.6 in renal SLE patients, 41,5±11.05 in nonrenal SLE patients and 38,7±10.7 in healthy controls. Both renal SLE and non-renal SLE patients had similar median disease duration (167,4±85,3 vs 141,06±98,9 months). The female proportion across patient groups and healthy control had no difference. SLE patients with renal and non-renal involvement had both decreased Pon-1 and Apo-A1 and increased Anti-ApoA1 levels compared to healthy controls (p<0.05 for all). Biomarkers showed no statistical significance between renal and nonrenal SLE. Patients with nephritis and without had both increased intima-media thickness (IMT) compared to healthy controls and IMT correlated positively with total SLICC score. Biomarker levels showed no diagnostic value for differentiation of active and inactive disease assessed by SLEDAI. Both SLE groups had statistically significantly more either carotid and/or femoral plaque than healthy controls. Plaque prevalence showed no statistical difference among renal and nonrenal SLE patients. Renal SLE patients had higher both carotid and femoral plaque than non-renal SLE patients (p=0,02). Comparing renal and non-renal SLE patients with plaque, Pon-1 activity and Apo-A1 levels were lower and Anti-ApoA1 titer was higher in renal SLE patients without statistical significance. Biomarkers demonstrated no diagnostic value in the presence of plaque. Age and disease duration were significant variables predicting plaque presence in renal and nonrenal SLE patients (p<0.001, p<0.05).Conclusion:SLE patients with renal and non-renal involvement have both significantly increased plaque prevalence and higher intima-media thickness. Older age, longer disease duration and cumulative damage are important risk factors for plaque formation. Although a higher proportion of plaque presence was detected in patients with nephritis than in those without, the difference did not reach statistical significance. The increase in the number of patients can lead to different results. Significantly lower Pon-1 and Apo-A1 levels and elevated Anti-ApoA1 titers were demonstrated in SLE patients; however, data about their association with the presence of plaque revealed no statistical significance. Considering the contribution of proinflammatory HDL to the pathogenesis of early-onset atherosclerosis, markers of dysfunctional HDL can provide important data when used simultaneously with imaging modalities in longitudinal follow-up.REFERENCES:[1] McMahon, Maureen et al. Arthritis and rheumatism vol. 60,8 (2009): 2428-37Acknowledgements:NIL.Disclosure of Interests:None declared.

Background:Systemic autoinflammatory disorders (SAIDs) include a group of diseases, which are associated with genetic variations resulting in dysregulation of innate immunity and hyperinflammatory response. Pathogenic and likely pathogenic variants in certain genes have been linked to an already defined phenotype, but screening of more patients with some autoinflammatory features, but no diagnosis, has usually revealed several variants of uncertain significance (VUS) and/or variants in more than one autoinflammatory gene.Objectives:We herein investigated the relationship between phenotypes and genotypes of the patients who were screened for autoinflammatory genes and identified as carriers of variants in ≥2 genes in a tertiary referral center.Methods:Charts of the patients who had been referred to our center with a potential diagnosis of an autoinflammatory disorder between April 2019 and January 2023, and the results of their genetic analyses were evaluated. All patients had the results of genetic screening of at least 22 genes, which were associated with different autoinflammatory disorders, and analyses covered the exons and 10 base pair intron borders of the selected genes and were carried out by using the Ion Torrent platform or Illumina platform.Results:We evaluated 146 referred patients (60 male, 85 female, mean age 41.0±12.6, range 19-82), and 44 of them (30.1%) were identified as having ≥2 gene variants. By using the recent classification criteria for autoinflammatory recurrent fevers, fifteen out of 44 (34.0%) patients were classified as FMF. Among the patients with the pathogenic MEFV mutations, variants of the additional genes may have had an effect on the phenotype such as sensorineural hearing loss in one with NLRP3 VUS. One patient with pathogenic MEFV and NLRC4 mutations was classified as FMF and NLRC4-AID. Since the Gattorno et al. criteria were aimed at only 4 common SAIDs, 5 (11.3%) additional patients could be diagnosed with other disorders such as VEXAS syndrome (n=1), deficiency of ADA2 (DADA2, n=2), A20 haploinsufficiency (HA20, n=1), DADA2 and HA20 combination (n=1) (Table 1). The remaining patients were grouped as having NLRP1-AID (n=2), NLRP1-AID and NLRC4-AID (n=1) combination, PFAPA-like (n=2) and Behçet disease-mimic (n=7) according to their dominant phenotype (Table 2). Twelve (27.2%) patients could not be classified into any groups (Table 3). Two of these cases had pathogenic, likely pathogenic variants, or VUS, and the remaining had between 2-7 different VUS combinations. NLRP1 variants were identified in 5 (11.3%) cases and the most common clinical finding was recurrent urticaria (n=4). All of the NLRP1 gene variants were classified as VUS; one of them (p.Val939Met) was a relatively common variant (allele frequency, 0.01), one of them (p.Val1235Ile) was not reported before, and the other one (p.Cys904Ter) was expected to have a pathogenic role due to early termination of the protein. Case 9 had an atypical FMF phenotype with a pathogenic variant in the MEFV and VUS in others, including the NLRP1, NLRP3, and NLRP12.Conclusion:The criteria set developed by Gattorno et al. have some limitations in practice since they cover only CAPS, FMF, TRAPS, and MKD, and are not validated in adults. Although those patients with pathogenic variants could be classified easily by using the criteria sets, the contribution of additional variants to their phenotypes needs further investigation. Four patients with recurrent urticaria as the most common clinical feature in association with 3 different VUS in the NLRP1 gene may suggest adding this gene to the list of autoinflammatory variants associated with urticarial manifestations, which may expand the spectrum of NLRP1-AID. An important group of adult patients may have an unclassified systemic autoinflammatory disorder, mainly associated with a combination of VUS in different genes, and this “mixed autoinflammatory disorder (MAID)” phenotype needs to be followed carefully for the evaluation of long-term prognosis.REFERENCES:NIL.Acknowledgements:NIL.Disclosure of Interests:None declared.