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Background:Cutaneous manifestations are observed in 59–85% of patients with SLE but less than 5% developed BSLE. In the GLADEL cohort, the prevalence is 0.41%. BSLE literature in children is scarceObjectives:to describe the clinical characteristics of the patients with BSLEMethods:series of cases between 2010-2019 of two reference centers. The cases met Camisa and Grimwood criteria for BSLEResults:5 cases had bullous lesions that resolved with residual hypopigmentation. One case had focal seizure and other patient had arthritis with leukopenia and thrombocytopenia. 2 patients had proteinuria <500 mg/24 hours. There were no cases of lupus nephritis. The median SLEDAI-2K score was 12 (IR: 8-17). All had ANAs in titers greater than 1:160 and four had anti-DNA (+). 5 patients had anti-RNP and 4 had anti-Sm. One case had anti-Ro/anti-La. All presented low C3 and 80% had low C4. 80% had ESR ≥20 mm/hour and CRP greater than 0.5 mg/dl in 60%. All presented clinical response with glucocorticoids and dapsone; one patient had methemoglobinemia that improved. At 3 months, the blisters did not recur in 4 patients except one case that presented relapse due to inadherence.Histologically, the most common finding was subepidermal blisters with neutrophils in the papilar dermis. DIF showed linear deposits of Igs and complement in 4 cases and granular deposits in one case; IgG/IgM were in 5 of the samples. IgA was positive in 60% and C3 in 80%Conclusion:In this series, BSLE was associated with neuropsychiatric, joint and haematological involvement in 40% of patients, without lupus nephritis. Such abnormalities had a parallel course to skin involvement, without recurrences. BSLE tends to have a single-phase behavior and in children unlike adults, severe renal involvement is uncommonReferences:[1]Pons-Estel GJ, et al. Lupus 2018; 27(10): 1753-1754Disclosure of Interests:None declared

Background:The NIH criteria are the main measure to determine activity in TAK. NLR and PLR appear promising to predict disease activity(1). TAK is one of the most frequent vasculitis in Colombia with a prevalence of 13.3%; however, in more recent years the information is sparse about TAK clinical behavior in our region, which leads to a late diagnosis. Although survival has improved with immunosuppressive treatment, relapses remain high especially in the first year of diagnosisObjectives:Compare NLR and PLR with ESR/CRP to predict TAK activityShow survival and relapse in patients with TAK followed to 7 yMethods:Retrospective cohort of 43 patients with TAK between 2011-2018 with prospective follow-up of relapses and mortality. 88% fulfilled the ACR 1990 criteria. The disease activity was determined according to NIH criteria: active disease (n=34) and inactive disease (n=9). Through bivariate analysis, we compared the clinical and radiologic characteristics between age groups (table 1) using the Pearson test and Wilcoxon range test. Value of p <0.05 was statistically significant. Survival and relapse analysis were performed using Kaplan-Meier plots. Sensitivity (Sn), specificity (Sp) and area under the curve (AUC) were determined for NLR, PLR, ESR and CRP by receiver-operating curves (ROC) compared to NIH criteria.Table 1.Comparison of Clinical Features in Patients with TAKNo (%) of PatientsAge at diagnosis≤40 y (n=32)Age at diagnosis≥40 y(n=11)P valueFemale30 (94)11 (100)0.39Age at symptom onset (years), median: (Interquartile range –IQR-)20 (17-25)52 (47-57)0.110Time from symptom onset to diagnosis (months) median (IQR)9.5 (2-15)36 (1.5-24)0.774Smoking2 (6)6 (54)0.002Dyslipidemia7 (22)6 (54)0.042ACR Criteria 1990Claudication of extremities14 (43)5 (45)0.922Upper extremities blood pressure discrepancy > 10 mmHg19 (59)6 (54)0.779Decreased brachial artery pulse24 (75)9 (82)0.644Subclavian artery or aorta bruit19 (59)6 (54)0.774Arteriographic abnormality27/27 (100)11/11 (100)1.0ESR (mm/h), median (IQR)56 (23-115)38 (33-76)0.011CRP (mg/dL), median (IQR)1.35 (0.4-3.4)3 (1.49-18.5)0.020Creatinine (mg/dL), median (IQR)0.72 (0.6-0.83)0.84 (0.75-1)0.007Hata-Numano classification I15%18% IIa6%9% IIb6% III6% IV3%9% V59%64%Results:41 patients were women (96%) with a median age at diagnosis of 22 y and an interval from the onset of disease to diagnosis of 12 months (IR:1-168 m). The population over 40 years had a greater comorbidities burden (54% had history of smoking and dyslipidemia) and a major interval between the onset of disease and the diagnosis (36 months vs 9.5 months). Most frequent vascular phenotypes were types V (62%) and I (16%). NLR and PLR showed poor performance to predict activity compared with CRP; NLR level of 1.74 showed to be the predictive cut-off value for active TAK (Sn: 85.3%, Sp: 37.5%, AUC = 0.563). PLR level of 112.5 was found to be the predictive cut-off value for active TAK (Sn: 76.5%, Sp: 50%, AUC = 0.517). The CRP was the most accurate biomarker (Sn: 79.4%, Sp: 75%, AUC = 0.761) while the ESR was lower to predict activity (Sn: 63.6%, Sp: 75%, AUC = 0.598) (figure 1). At 5 years, survival was 83% and 50% of patients had presented at least one relapse (figure 2)Conclusion:Our data does not support the use of NLR or PLR to differentiate relapse and remission in TAK. CRP had better diagnostic performance than ESR in the prediction of activity compared to NIH criteria. The 5-year survival in this cohort is below that reported after 1985 (reported survival: 90-96%)(2)References :[1]Pan L, et al. Platelet-to-lymphocyte ratio and neutrophil-to-lymphocyte ratio associated with disease activity in patients with Takayasu’s arteritis: a case-control study. BMJ Open 2017; 7: e014451[2]Schmidt J, et al. Diagnostic features, treatment, and outcomes of Takayasu arteritis in a US cohort of 126 patients, Mayo Clin Proc. 2013; 88(8): 822-30Disclosure of Interests:None declared

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Background:Takayasu’s arteritis (AT) is a systemic vasculitis that affects large vessels, mainly the aorta, its branches and the pulmonary arteries. There are few data on their behavior in our region.Objectives:to describe the clinical and angiographic characteristics and outcomes of a group of patients with AT.Methods:a cross-sectional, retrospective study based on a secondary source taken from two high complexity hospitals in Medellin - Colombia between August 2011 and December 2018.Results:medical records of 41 patients were evaluated, 87.5% met the 1990 ACR criteria of AT. The majority were women (95.1%), with a median age at the time of diagnosis of 22 years (IR 52), 11 patients (26.8%) were over 40 years old at debut. The median delay in diagnosis was 12 months (IR: 167). Systemic symptoms included general malaise (29.3%), weight loss (29.3%) and fever (19.5%); the main vascular manifestations were hypertension and blood pressure difference (61%), headache (39%), upper limb claudication (31.7%), dyspnea (29.3%), angina (26.8%) and carotidinia (22%). Most frequent findings on physical examination were pulse absence or decrease (78%) and subclavian murmur (53.7%). ESR and CRP were found high in 85.3% and 22%, respectively. Angiographic studies revealed stenosis or occlusion in multiple arterial territories, being the stenosis of the left subclavian artery the most frequent lesion (68.3%), followed by the involvement of the abdominal aorta (58.5%) and thoracic (51%). The distribution according to Hata-Numano classification was: type V (58.5%), type I (17.1%) and type IIa (7.3%), indicating complex vascular lesions and associated serious complications. Abnormalities of the renal arteries, pulmonary arteries involvement, and aortic insufficiency were present in 39%, 14.6%, and 7.3% respectively. Seventy-five percent of patients had active disease, glucocorticoids were the main treatment in 90.2% of the cases, used isolated (2.4%) or in combination with other immunosuppressants (87.8%), including methotrexate (75.6%), azathioprine (26.8%) and cyclophosphamide (14.6%); vascular interventions were performed in 11 patients (26.8%). Most relevant vascular complications were renovascular hypertension (26.8%), stroke (19.5%), chronic kidney disease (19.5%) and chronic heart failure (17.1%). From the clinical point of view, our findings were similar to those shown in series from Brazil, Japan, and Turkey. Contrary to what was reported in other series, carotidinia was a finding that occurred in almost a quarter of our patients (23%) and maybe one of the initial symptoms of the disease. The findings of the vascular physical examination are similar to those reported by the IRAVAS group, where the asymmetry of the pulses was most frequent, followed by the presence of murmurs and the difference in blood pressure, claudication being less frequent in the upper and lower limbs.Conclusion:patients in this series are characterized by having an extensive disease partly due to a late diagnosis, with a high percentage of complications associated with vascular stenotic compromise, which generates morbidity and impact on the quality of life.References:[1]Sato EI, et al. Takayasu arteritis. Treatment and prognosis in a university center in Brazil, Int J Cardiol. 2000; 75 Suppl 1: S163-6.[2]Arnaud L, et al. Takayasu arteritis in France: a single-center retrospective study of 82 cases comparing white, North African, and black patients. Medicine (Baltimore). 2010; 89(1): 1-17.Disclosure of Interests: :None declared