R. Blanco

Schwannoma intralaberíntico: a propósito de dos casos

Los schwannomas intralaberinticos son tumores benignos muy infrecuentes que surgen a partir de elementos neurales de las ramas terminales del VIII par craneal, que afectan primariamente al vestibulo, los conductos semicirculares y/o la coclea. La sintomatologia mas frecuente consiste en hipoacusia unilateral progresiva con o sin acufenos. Pueden asociarse trastornos del equilibrio. El metodo habitual de diagnostico es la resonancia magnetica con contraste paramagnetico (gadolinio). El tratamiento es conservador enla mayoria de los pacientes, y se reserva el tratamiento quirurgico para indicaciones muy precisas. Presentamos 2 casos de schwannoma intralaberintico diagnosticados en nuestro servicio y discutimos el tratamiento de esta enfermedad.

Oropharyngeal Kaposiform Hemangioendothelioma

The kaposiform hemangioendothelioma is a very infrequent tumor proceeding from the endothelial-derived spindle cells, more often found on the limbs, although peritoneal, retroperitoneal and sacrum locations are also prevailing. Head and neck are exceptional locations. The kaposiform hemangioendothelioma is almost exclusively found in children and early adolescents, and it is highly associated with the Kasabach-Merritt syndrome and lymphangiomatosis. The main treatment is the tumor surgical removal, including wide margins, plus supporting therapy when Kasabach-Merritt syndrome is linked. We report an isolated oropharyngeal kaposiform hemangioendothelioma 9-10-year old male case.

Hemangioendotelioma kaposiforme de orofaringe

El hemangioendotelioma kaposiforme es un tumor raro que procede de celulas fusiformes de derivacion endotelial cuya presentacion mas frecuente es en las extremidades; es frecuente tambien su localizacion peritoneal, retroperitoneal y en el sacro. La presentacion en cabeza y cuello es excepcional. El tumor afecta casi exclusivamente a ninos y adolescentes jovenes y se asocia con frecuencia elevada al sindrome de Kasabach-Merritt y a linfangiomatosis. El tratamiento de eleccion consiste en la extirpacion quirurgica del tumor con amplios margenes y el tratamiento de soporte en los pacientes con sindrome de Kasabach-Merritt. Presentamos un caso unico de hemangioendotelioma kaposiforme localizado en la orofaringe en un varon de 19 anos.

Intra-Labyrinthine Schwannoma: Two-Cases Report

Intralabyrinthine schwannomas are uncommon tumours that arise from neural elements in distal branches of the 8th cranial nerve thus they are confined to or have arisen from the vestibule, the semi-circular canals, or the cochlea. The most common presenting symptom is progressive sensory neural hearing loss with or without tinnitus and vestibular symptoms. The diagnostic procedure of choice is gadolinium-enhanced magnetic resonance imaging. Most of these patients can be managed with observation. The indications for surgery are limited. We present 2 patients with a diagnosis of intralabyrinthine schwannoma. A discussion of the management of the intralabyrinthine schwannoma follows.

AB0584 Subclinical Atheromatosis in Patients with Systemic Lupus Erythematosus

Background Patients with systemic lupus erythematosus (SLE) have an increased cardiovascular (CV) risk, probably due to accelerated atherosclerosis. Objectives To analyze the prevalence of subclinical CV disease in SLE using carotid ultrasonography. Methods We studied 36 SLE patients and 127 age and sex matched controls. Traditional CV risk factors were recorded according to a standardized protocol. Carotid ultrasonography was performed by a MyLab 70 scanner (Esaote; Genoa, Italy), equipped with 7–12 MHz linear transducer and the automated software guided technique radiofrequency – Quality Intima Media Thickness in real-time (QIMT, Esaote, Maastricht, Holland) to determine carotid intima-media thickness (cIMT) and plaques, according to the Mannheim Carotid Intima-Media Thickness Consensus. Results No statistically significant differences in the frequency of smoking, hypertension, diabetes mellitus or personal and family history of CV events between patients and controls were found. However, the prevalence of dyslipidemia was increased in SLE (31% vs 16% in controls; p=0.056) and SLE patients had a higher body mass index (BMI) (27.1±5.4 vs 25.2±4.8, p=0.051). The cIMT was also significantly higher in SLE patients when compared to controls (0.644±0.122 vs 0.573±0.113 mm; p=0.001). This difference persisted after adjustment for BMI and dyslipidemia (p=0.009). Moreover, the prevalence of carotid plaques in SLE patients was also increased (42% vs 23% in controls; p=0.033).Table 1 Variable SLE (n=36) Controls (n=127) p Men/Women, n 3/33 13/114 1 Age (years), mean ± SD 49.0±16.1 45.1±12.7 0.132 Traditional CV risk factors, n (%)  Non-smokers 21 (58) 75 (59) –  Smokers 7 (19) 26 (21) 0.971  Former smokers 8 (22) 26 (21) –  Obesity 9 (27) 19 (15) 0.122  Dyslipidemia 11 (31) 20 (16) 0.056  Hypertension 9 (25) 15 (13) 0.111  Diabetes mellitus 0 (0) 3 (2.5) 1 Previous history of CV events, n (%) 2 (6) 2 (2) 0.212 Family history of premature CV disease, n (%) 9 (25) 18 (15) 0.208 Mean blood pressure, mean ± SD (mm Hg) 92.3±10.2 91.6±11.1 0.728  Systolic blood pressure 121.8±15.6 121.4±17.0 0.914  Diastolic blood pressure 77.6±8.4 76.6±9.3 0.599 Body mass index, mean ± SD (kg/m2) 27.09±5.36 25.19±4.82 0.051 Abdominal perimeter (cm), mean ± SD 91.5±13.7 86.1±16.6 0.096 Carotid Intima-media thickness (mm), mean ± SD 0.644±0.122 0.573±0.113 0.001 Carotid plaques, n (%) 15 (42) 28 (23) 0.033 Conclusions Both cIMT and carotid plaque frequency are increased in SLE patients. The presence of carotid plaques identifies individuals at very high cardiovascular risk. Therefore, tight controls of dyslipidemia and other cardiovascular risk factors should be conducted in SLE patients. Disclosure of Interest None declared

FRI0323 Apremilast therapy in refractory skin lupus lesions

Background Skin lesions of lupus may be refractory to standard therapy. Apremilast is an orally small molecule which inhibits phosphodiesterase-4 (PDE-4) that modulates some inflammatory pathways. Objectives Our aim was to assess the efficacy of apremilast in lupus rashes refractory to conventional treatment. Methods Retrospective study on 5 lupus patients treated with apremilast at standard dose of 30 mg twice daily. The outcome was improvement of lupus rashes. Results We described 5 patients (4 women and 1 male) with a mean age of 44.2±8.5 years with extensive skin lesions due to lupus. Three patients had a discoid lupus and 2 patients had systemic lupus erythematosus (SLE) (one with panniculitis and the other with polycyclic ring lupus). The cutaneous lupus was confirmed in all patients by skin biopsy. Prior to apremilast all patients had received conventional treatment: topical corticosteroids (n=5), antimalarials (n=5), topical tacrolimus (n=2), oral corticosteroids (n=2), thalidomide (n=1), belimumab (n=1) and rituximab (n=1). After a mean follow-up of 6.2±2.9 months, all the patients experienced improvement of the skin lesions (in two patients was complete). In one patient it was necessary to reduce the dose of apremilast to 30 mg/day because of digestive symptoms. Conclusions Apremilast can be useful in the treatment of refractory skin lesions of lupus. Disclosure of Interest None declared

SAT0535 Carotid Ultrasound in The Cardiovascular Risk Stratification of Patients with Ankylosing Spondylitis: Results of A Population-Based Study

Objectives To determine if the use of carotid ultrasonography (US) may improve the cardiovascular (CV) risk stratification in patients with ankylosing spondylitis (AS). Methods A set of 127 consecutive patients without history of CV events, diabetes mellitus or chronic kidney disease that fulfilled definitions for AS according to the 1984 modified New York criteria were recruited to assess carotid intima-media thickness and presence of plaques. CV risk was calculated according to the systematic coronary risk evaluation (SCORE), the Framingham Risk Score (FRS) and the Reynolds Risk Score (RRS). Results Men outnumbered women (61.4%). The mean±SD age at the time of the study was 44.5±11.6 years. The median (interquartile range) delay to the diagnosis was 5 (1–12) years. HLA-B-27 was positive in 77.2%, and syndesmophytes were present in 38.9%. Carotid plaques were found in 43 (33.9%). Regardless of the algorithm used for CV risk stratification, more than 50% of the patients classified as having moderate CV risk had carotid plaques. Moreover, 20.8%, 24.6% and 53.3% of AS that fulfilled the category of low CV risk according to the total cholesterol (TC)-SCORE, FRS and RRS, respectively had carotid plaques. A model that included patients with a chart TC-SCORE ≥5% or SCORE-TC ≥1% <5% plus carotid plaques or TC-SCORE <1% and CRP >3 mg/L at diagnosis plus syndesmophites and carotid plaques or TC-SCORE <1% and CRP >3 mg/L at diagnosis plus extraarticular manifestations plus carotid plaques yielded the highest sensitivity (93.0%) for high/very high CV risk in these patients. The presence of syndesmophytes was associated with increased risk of carotid plaques in AS that fulfilled definitions for low CV risk according to the TC-SCORE (OR 8.75 [95% CI 2.11 - 36.40]; p=0.002). Conclusions Our results support the use of carotid US in the assessment of CV risk in patients with AS. Disclosure of Interest None declared

SAT0102 Influence of Vaccination Program To Prevent Acute Respiratory Infection in Rheumatoid Arthritis

Background Patients with autoimmune rheumatic diseases such as rheumatoid arthritis (RA) are at increased risk for infections, especially respiratory type. Immunization is a recommended measure to reduce these complications. Objectives Assess the incidence of acute respiratory infections (ARI), defined as one that causes hospitalization, before and after inclusion in a systematic vaccination program. Methods Prospective study in a cohort of 294 patients diagnosed with RA, were included in the vaccination program. This program includes: seasonal flu vaccine (october to april), pneumococcal (combined regimen 13-valent and 23-valent) and Haemophilus influenzae B. Only 7 patients (2.94%) refused vaccination. The follow-up time was from October 1st, 2011 (starting date) to June 30th, 2015. Information on ARI episodes before and after immunization was collected from the Hospital Information System, reviewing hospital records. Results 287 RA patients (225 women/62 men) were vaccinated, with an average age of 58.1 ± 12.7 years. RA was characterized at the time of vaccination by the time of evolution of RA 93 ± 95.9 months; 154 (53.65%) positive RF, 97 (33.8%) erosive and 40 (13.9%) with extra-articular manifestations (7 with pulmonary fibrosis). Only 42 (14.6%) were not taking any immunosuppressive or immunomodulatory therapy at the time of immunization. 147 patients were treated with Disease Modifying Antirheumatic Drugs (DMARDs), mainly methotrexate, and 98 patients (34.1%) were with a TNFa inhibitor. Regarding the ARI, before inclusion in the vaccination program, 20 (7%) patients in the cohort studied had been admitted into the hospital for this reason. During follow-up, and subsequent immunization, only 6 of the 287 patients (2.1%) had ARI (Table 1). One of them had an ARI prior to vaccination program. This reduction from 7% to 2.1% achieved statistically significant difference (p=0.0017). Conclusions Incorporating a program of systematic vaccination in RA patients appears to be an effective prophylactic measure to prevent the ARI. Disclosure of Interest None declared

AB0277 Comparison between Intima-Media Thickness and Coronary Artery Tomography in Subclinical Atherosclerosis Detection in Rheumatoid Arthritis

Background Intima media thickness (IMT) and coronary artery calcification (CAC) quantification using multidetector computed tomography (MDCT) scanner are useful in detecting subclinical atherosclerosis and are good surrogate markers of cardiovascular morbidity and mortality in general population and in rheumatoid arthritis (RA) A good correlation between these methods has been reported in RA, being CAC a slightly more sensitive technique (1) Objectives Our aim is to determine the value of IMT that better predicts the presence of coronary atherosclerosis, using CAC as reference and assuming CAC value of 100 as the cut-off point indicating high cardiovascular risk. Methods We evaluated 127 RA patients without previous cardiovascular events. Carotid ultrasonography was performed by a MyLab 70 scanner (Esaote; Genoa, Italy), equipped with 7–12 MHz linear transducer and the automated software guided technique radiofrequency – Quality Intima Media Thickness in real-time (QIMT, Esaote, Maastricht, Holland). According to data from non-rheumatic patients and also from RA patients, an IMT≥0.90 mm is a good predictor of high cardiovascular risk. To determine CAC score, a CT Imaging of coronary arteries using a 32-slice MDCT scanner (Lightspeed, Pro 32, GE Healthcare, USA) was performed. Results Patients with IMT below 0.90 mm had CAC values of 88±210 whereas patients with IMT≥0.90 mm had mean CAC values of 190± 272 (p=0.066). We found a positive correlation between IMT and CAC (correlation coefficient 0,303; p=0.001). The IMT cut-off value ≥0.90 mm had a sensitivity of 32% for detecting CAC ≥100. ROC curve analysis showed and area under the curve of 0.664. The IMT cut-off value of 0.80 mm had a sensitivity of 40% and a specificity of 71.3%. Lowering the IMT cut-off point to 0.70, we reached a sensitivity of 76% and a specificity of 51.5% for CAC ≥100 detection. The positive predictive value for the IMT cut-off point of 0.70 mm was 76% in our population, being the negative predictive value 49.5% Variable Variable Age (mean ± SD) 58. 57±9.7 IMT ≥0.7 mm, n (%) 71 (56) Female sex, n (%) 92 (72.,4) CAC score (mean ± SD) 103.4±222.6 IMT ≥0,9 mm, n (%) 19 (15) CAC score ≥100, n (%) 26 (20.5) Conclusions IMT values ≥0.70 mm predict coronary artery calcification score above 100 with a sensitivity of 76%. According to that, the IMT value considered as predictor of high cardiovascular risk would be 0.70 mm instead of 0.90 mm. References Corrales A et al. “Cardiovascular risk stratification in rheumatic diseases: carotid ultrasound is more sensitive than coronary artery calcification score to detect subclinical atherosclerosis in patients with rheumatoid arthritis” Ann Rheum Dis. 2013,72 1764–1770. Disclosure of Interest None declared

THU0566 Adalimumab Optimization in Behcet's Syndrome Refractory Uveitis Once Obtained Remission

Background In non-infectious no anterior uveitis, adalimumab (ADA) is the only biologic that has shown efficacy in phase III randomized, double blind studies, such as VISUAL I and VISUAL II. (Brezin AP et al Arthritis Rheumatol 2015;67 (suppl 10): 2038 & Nguyen QD et al Arthritis Rheumatol 2015; 67 (suppl 10): 1388). After a 80 mg loading dose, maintenance dose is the standard for other indications, 40 mg/sc/2 weeks. Objectives Our objective is to test a series of Behcets síndrome uveítis, and proving if once remission was obtained, it was posible to “optimize” the maintenance dose. Methods Multicenter study of 174 patients with Behcets síndrome refractory uveitis. All of them had insufficient response or intolerance to conventional treatment with corticosteroids and at least 1 systemic immunosuppressive drug. 71 patients started treatment with ADA and once remission was achieved, it was optimized in 23 of them. The degree of ocular inflammation was assessed by “the Standardization of Uveitis Nomenclature (SUN) Working Group” (Am J Ophthalmol 2005; 140: 509–516), and macular thickness by optical coherence tomography (OCT). A comparison was made between the first visit before initializing ADA at standard dose, the start of the optimization of the drug, and the final visit. The results are expressed as mean ± 1 SD for variables with a normal distribution, and as median [IQR 25–75] (range) when it is not normal. Comparison of continuous variables was performed using the Wilcoxon test. Results 23 patients/42 affected eyes (15 men/8 women) who had a dose optimization were studied, the median age was 37.2 ± 13.4 years (range 10–62). HLA-B51 was positive in 60.8%. Prior to ADA, and as systemic treatment besides oral steroids, they had received intravenous methylprednisolone bolus (n=7), cyclosporin A (CyA) (n=20), methotrexate (MTX) (n=11) and azathioprine (AZA) (n=11). ADA monotherapy was used in 5 cases and also in combination with immunosuppressants: CyA (n=12), MTX (n=4) and AZA (n=2). The average remission before optimization of the dose was 15.3 ± 9 months. In 23 patients the interval dose of adalimumab was progressively increased to 3 weeks (n=6), 4 weeks (n=13), 5 weeks (n=1), 6 weeks (n=1) and 8 weeks (n=2). Only 2 patients had to return to the standard dose of ADA due to serious outbreak after optimizing the drug, reaching again remission of uveitis. It was also posible to suspend ADA in 4 patients after 35.2 ± 9.3 months in remission, not presenting a new outbreak after a mean of 20 ± 6.9 months following the suspension. There were not serious side effects during a mean follow-up of 34.7 ± 13.3 months after starting ADA. Conclusions Optimizing ADA therapy, once remission is achieved, seems feasible in Behcets síndrome uveítis refractory to systemic treatment. Disclosure of Interest None declared