Search

Although retinal venous obstruction (RVO) has been primarily related to vascular risk factors and glaucoma, there are a few studies of RVO in patients with solid organ transplants. An analysis was performed on total of 331 patients who presented with RVO (branch RVO in 226 cases and central RVO in 105 cases) over a 10 year period, and the characteristics were compared with the 4 patients with a history of solid organ transplant (liver, renal, or bipulmonary) who presented with RVO. In this series, the onset of RVO in transplant patients occurred at earlier ages than in other patients with RVO (58 vs. 68 years, respectively), affected the central vein of the retina (100% vs. 32%), and was associated with diabetes mellitus (75% vs. 25%), as well as with steroidal (100% vs. 1%) and immunosuppressive (tacrolimus 75% vs. 0%) treatments.

Background and objectives A new coronavirus disease in humans, COVID-19, caused by SARS-CoV-2, emerged in December 2019. It has been associated with the development of thrombotic phenomena. Retinal vein occlusion (RVO) is mainly a consequence of vascular risk factors (VRF). This study aimed to analyze cases of COVID-19 in a cohort of patients with RVO (Valdecilla cohort). Patients and methods Between December 2008 and December 2020, 429 patients with RVO were attended to in our clinic. Ten patients had COVID-19, one of which did not have VRF or thrombophilia. The remaining nine patients had RVO prior to the infection and VRF, six had carotid atherosclerosis, and four had antiphospholipid syndrome. The infection did not cause thrombotic phenomena in any of them. Conclusions RVO is a rare manifestation of COVID-19. In our cohort of patients with RVO, COVID-19 disease did not lead to thrombotic events.

Resumen Antecedentes y objetivos En diciembre de 2019 surgio una nueva enfermedad por coronavirus en humanos causada por el virus SARS-CoV-2, la COVID-19, que se ha asociado con fenomenos tromboticos. La obstruccion venosa retiniana (OVR) es principalmente una consecuencia de los factores de riesgo vascular (FRV). El objetivo de este estudio ha sido analizar los casos de infeccion por SARS-CoV-2 en una cohorte de pacientes con OVR (cohorte Valdecilla). Pacientes y metodos Entre diciembre de 2008 y 2020 hemos atendido 429 pacientes con OVR. Diez han padecido COVID-19, de los que uno no presentaba FRV ni trombofilia. Los otros 9 fueron diagnosticados de OVR antes de la infeccion: todos tenian FRV; 6 ateromatosis carotidea y 4 presentaban un sindrome antifosfolipido. La infeccion no causo en ellos fenomenos tromboticos. Conclusiones La OVR es una manifestacion infrecuente de la COVID-19. En nuestra cohorte de pacientes con OVR la COVID-19 no indujo eventos tromboticos.

Extracellular vesicles (EVs) are a broad, heterogeneous class of membranous lipid-bilayer vesicles that facilitate intercellular communication throughout the body. As important carriers of various types of cargo, including proteins, lipids, DNA fragments, and a variety of small noncoding RNAs, including miRNAs, mRNAs, and siRNAs, EVs may play an important role in the development of addiction and other neurological pathologies, particularly those related to HIV. In this review, we summarize the findings of EV studies in the context of methamphetamine (METH), cocaine, nicotine, opioid, and alcohol use disorders, highlighting important EV cargoes that may contribute to addiction. Additionally, as HIV and substance abuse are often comorbid, we discuss the potential role of EVs in the intersection of substance abuse and HIV. Taken together, the studies presented in this comprehensive review shed light on the potential role of EVs in the exacerbation of substance use and HIV. As a subject of growing interest, EVs may continue to provide information about mechanisms and pathogenesis in substance use disorders and CNS pathologies, perhaps allowing for exploration into potential therapeutic options.

AAC-8 and AAC-24 are two widely used scales to evaluate abdominal aortic calcification (AAC) on X-ray images. Levels of ≥3 (AAC-8) and ≥5 points (AAC-24) are of high relevance since they are associated with greater risk of cardiovascular events. Given that it is unknown, our aim was to determine the reliability of both scales at those levels of atherosclerotic burden.The sample (93 subjects, 67.3 ± 9.7 years, BMI 28.8 ± 3.8, 57.6% smokers, 64.1% with hypertension) was classified according to quartiles of calcification. Six clinicians evaluated AAC independently with both scales on lateral lumbar spine X-ray images. We analyzed inter-rater agreement with the intraclass correlation coefficient (ICC) and the Bland-Altman scatterplots.We assessed 15 pairs of raters. Scores in both scales were significantly correlated with cardiovascular risk (r = 0.31 and r = 0.32; p 0.005). Agreement was very high in the first quartile and moderate in the rest (p 0.05). At cut-off points, ICC = 0.70 (95%CI, 0.54-0.86) and ICC = 0.68 (95%CI, 0.60-0.85) with AAC-8 and AAC-24. With the Bland-Altman method, mean of the differences ranged between 0 and 0.4 (AAC-8), and between 0.2 and 1 (AAC-24), while 95% limits of agreement showed values between 2.9 and 4.4 (AAC-8), and between 6 and 11.2 (AAC-24). Analyzing entire scales, ICC = 0.97 (95%CI, 0.97-0.98) and ICC = 0.98 (95%CI, 0.97-0.98) for AAC-8 and AAC-24, respectively.Both scales presented only moderate reliability at levels of atherosclerotic burden. Analyzing quartiles with ICC and the Bland-Altman plot showed concordant results. High global ICC values traditionally reported with both scales are likely biased.AAC predicts subsequent vascular morbidity and mortality and should implicate evaluation of cardiovascular risk. Optimal visualisation of AAC and its correct assessment are mandatory in order to maximize patient care.

The human retina, as transducer of light energy, is especially exposed to light toxicity. Solar maculopathy has been the only form of photic maculopathy for millennia, often secondary to the observation of an eclipse. During the last century, technological advances have led to the appearance of new forms of photic maculopathy, related to the exposure to new forms of artificial light, such as welding devices and lasers. In recent years, the general use of laser pointers has led to an upturn in interest in this pathology. The aim of this review is to offer an integrated view of the different types of photic maculopathy. Due to the extension of this topic, the review is presented divided into two parts. In this first part solar maculopathy and welding arc maculopathy are presented.

Resumen Objetivos Analizar la importancia de los factores de riesgo vascular, los hallazgos ecograficos de los troncos supraaorticos, y la presencia de fibrilacion auricular no valvular (FANV) anticoagulada en pacientes con obstruccion venosa retiniana (OVR) y en un grupo control. Pacientes y metodos Estudio transversal de todos los pacientes con OVR remitidos consecutivamente a la consulta de Medicina Interna, comparandolos con un grupo control. Se analizaron variables clinicas, electrocardiograficas y ecograficas. Resultados Se estudiaron 212 pacientes (114 varones y 98 mujeres) con OVR y 212 controles (95 varones y 117 mujeres) de edad similar. La hipertension arterial, la dislipidemia y la diabetes mellitus fueron significativamente mas prevalentes en los pacientes con OVR que en los controles (73,6 vs. 50%, 64,6 vs. 48,6%, y 27,8 vs. 12,3%, respectivamente). Se observaron lesiones arterioescleroticas en los troncos supraaorticos en el 55% de las OVR. Los pacientes con OVR y FANV tenian una mayor carga de factores de riesgo vascular que los controles con FANV. No hubo diferencias respecto a la razon internacional normalizada o a la utilizacion de anticoagulantes de accion directa entre casos y controles con FANV. Conclusiones Los factores de riesgo vascular (en especial la hipertension arterial) y la afectacion arterioesclerotica de los troncos supraaorticos son muy prevalentes en la OVR. La anticoagulacion no parece eficaz para prevenir la OVR.

Objective Diurnal birds of prey (raptors) are considered the group of animals with highest visual acuity (VA). The purpose of this work is to review all the information recently published about the visual system of this group of animals. Material and methods A bibliographic search was performed in PubMed. The algorithm used was (raptor OR falcon OR kestrel OR hawk OR eagle) AND (vision OR “visual acuity” OR eye OR macula OR retina OR fovea OR “nictitating membrane” OR “chromatic vision” OR ultraviolet). The search was restricted to the “Title” and “Abstract” fields, and to non-human species, without time restriction. Results The proposed algorithm located 97 articles. Conclusions Birds of prey are endowed with the highest VA of the animal kingdom. However most of the works study one individual or a small group of individuals, and the methodology is heterogeneous. The most studied bird is the Peregrine falcon (Falco peregrinus), with an estimated VA of 140 cycles/degree. Some eagles are endowed with similar VA. The tubular shape of the eye, the large pupil, and a high density of photoreceptors make this extraordinary VA possible. In some species, histology and optic coherence tomography demonstrate the presence of 2 foveas. The nasal fovea (deep fovea) has higher VA. Nevertheless, the exact function of each fovea is unknown. The vitreous contained in the deep fovea could behave as a third lens, adding some magnification to the optic system. pre-print 778 KB

Objectives To analyze the importance of cardiovascular risk factors, ultrasound findings in the supra-aortic trunk and the presence of anticoagulated nonvalvular atrial fibrillation (NVAF) in patients with retinal vein occlusion (RVO) and in a control group. Patients and methods A cross-sectional study was conducted of all patients with RVO consecutively referred to the office of internal medicine, comparing them with a control group. We analyzed clinical, electrocardiographic and ultrasound variables. Results We studied 212 patients (114 men and 98 women) with RVO and 212 controls (95 men and 117 women) of similar ages. Arterial hypertension, dyslipidaemia and diabetes mellitus were significantly more prevalent in the patients with RVO than in the controls (73.6 vs. 50%, 64.6 vs. 48.6% and 27.8 vs. 12.3%, respectively). We observed arteriosclerotic lesions in the supra-aortic trunk in 55% of the patients with RVO. The patients with RVO and NVAF had a greater burden of cardiovascular risk factors than the controls with NVAF. There were no differences in terms of the international normalized ratio or in the use of direct anticoagulants between the cases and controls with NVAF. Conclusions Cardiovascular risk factors (especially arterial hypertension) and arteriosclerotic involvement of the supra-aortic trunk are highly prevalent in RVO. Anticoagulation does not appear to be effective in preventing RVO.

Background:Biologic therapy has improved prognosis of Behçet Disease (BD) uveitis (1-5). Although infliximab (IFX) is approved in Japan, most data in Caucasian patients comes from small series. No data on optimization has been publishedObjectives:In a large series of Caucasian patients with refractory uveitis of BD, we assess:a) long-term efficacy and safety;b) IFX optimization when ocular remission was achievedMethods:Multicenter study of IFX-treated patients with BD uveitis refractory to conventional immunosuppressants.103 patients were treated with IFX as 1st biologic as follows: 3-5 mg/kg i.v. at 0, 2, 6 and every 4-8 weeks. The main outcomes were anterior chamber cells, vitritis, retinal vasculitis, macular thickness, visual acuity, and glucocorticoids sparing effect; analysed at baseline, 1st week, 1st and 6th months and 1st and 2nd years. After remission, IFX optimization was performedResults:In whole series (n=103), main outcomes showed a rapid and maintained improvement, reaching remission in 78 patients after a mean IFX duration of 31.5 months. Severe side-effects were observed in 9 patients.Comparative study between optimized and non-optimized groups showed:a) no differences in clinical baseline characteristics;b) similar maintained improvement in most ocular outcomes;c) lower severe adverse events andd) lower IFX cost in optimized group (4826.52 vs 9854.13 euros/patient/year)(Table)Conclusion:IFX seems effective and safe in Caucasian patients with refractory BD uveitis. IFX optimization is effective, safe, and cost-effectiveReferences:[1]Martín-Varillas JL. Ophthalmology 2018;125:1444-1451.[2]Atienza-Mateo B: Arthritis Rheumatol. 2019;71:2081-2089[3]Santos-Gómez M. Clin Exp Rheumatol. 2016;34 (6 Suppl 102): S34-S40[4]Urruticoechea-Arana A. Rheumatol Int. 2019;39:47-58.[5]Atienza-Mateo B. Rheumatology (Oxford). 2018 1;57:856-864Table.OptimizedN=18Non OptimizedN=42PPatients/eyes affected, n/n18/3442/77Age, mean (SD), years39.5 (9.8)38.8 (10.5)0.82Men, %55.659.50.78Duration of uveitis before IFX, median [IQR] months38 [18-119]35 [10-48]0.11Ocular features at time of IFX onset-AC cells count, median [IQR]2 [1-4]2 [1-2]0.29-Vitritis, median [IQR]2 [1.5-3]2 [1-2]0.02-BCVA, mean (SD)0.32 (0.21)0.37 (0.26)0.51-OCT, mean (SD)303.5 (23.3)397.7 (155.7)0.12-Retinal vasculitis, n (%)9 (50)26 (66.7)0.23Uveitis pattern, n (%)-Bilateral/unilateral16/2 (88.9/11.1)35/7 (83.3/16.7)0.71-Anterior0 (0)6 (14.3)0.17-Posterior5 (27.8)8 (19.0)0.50-Panuveitis13 (72.2)28 (66.7)0.67Prednisone dose at IFX onset, mean (SD), mg/d40.3 (20.6)41.4 (15.5)0.81IFX therapyMonotherapy/combined treatment, n (%)15 (83.3)30 (71.4)0.33-AZA5 (27.8)4 (9.5)0.11-CsA9 (33.3)8 (19.0)0.32-MTX4 (22.2)15 (35.7)0.30Follow-up on IFX therapy, median [IQR], months48 [33-60]24 [6-60]0.007-Relapses, median (IQR)0 [0-1]0 [0-2]-Remission, %10075.60.46-Severe side effects, n (per 100 patients/year)0 (0)3 (0.78)0.02-Cost (mean), euros per year4826.529854.130.55–Disclosure of Interests:José Luis Martín-Varillas Grant/research support from: AbbVie, Pfizer, Janssen and Celgene, Speakers bureau: Pfizer and Lilly, Belén Atienza-Mateo: None declared, Vanesa Calvo-Río Grant/research support from: MSD and Roche, Speakers bureau: AbbVie, Lilly, Celgene, Grünenthal, UCB Pharma, Emma Beltrán: None declared, Alfredo Adan: None declared, Elena Aurrecoechea: None declared, Santos Castañeda: None declared, Miguel A González-Gay Grant/research support from: Pfizer, Abbvie, MSD, Speakers bureau: Pfizer, Abbvie, MSD, J. Luis Hernández: None declared, Ricardo Blanco Grant/research support from: AbbVie, MSD, and Roche, Speakers bureau: AbbVie, Pfizer, Roche, Bristol-Myers, Janssen, and MSD

espanolIntroduccion. El diagnostico de la infeccion por SARSCoV-2 presenta limitaciones. La RT-PCR en frotis nasofaringeo es la prueba considerada como el patron oro, aunque puede ofrecer falsos negativos. El objetivo de este trabajo ha sido evaluar la utilidad de repetir el frotis nasofaringeo en pacientes con resultado negativo en funcion de las diferentes probabilidades clinicas. Metodos. Estudio observacional retrospectivo de los primeros pacientes ingresados en el Hospital Universitario Marques de Valdecilla en dos plantas COVID de Medicina Interna durante marzo-abril del 2020. La RT-PCR para la deteccion de al menos dos dianas de los principales genes (E, N, RdRP, ORFab1) y el test de deteccion de anticuerpos para la deteccion de al menos IgG. Resultados. Se analizaron 145 pacientes hospitalizados; 98 (67,5%) con el diagnostico de SARS-CoV-2. Las variables predictivas independientes de SARS-CoV-2 fueron: contacto epidemiologico con otro paciente COVID-19, presentacion clinica como neumonia, ausencia de neumonia en el ano previo, inicio de sintomas >7 dias al ingreso, ≥2 sintomas -tos, disnea y fiebre- y lactato deshidrogenasa >350 U/L (p La repeticion de la prueba permitio detectar un 16% de casos adicionales. El rendimiento global de sucesivas RT-PCR en pacientes con baja probabilidad clinica fue inferior al 5%. Conclusion. Hemos definido un score de probabilidad pre-prueba basado en datos epidemiologicos y clinicos con una buena precision para el diagnostico por infeccion SARSCoV-2. La repeticion del frotis nasofaringeo puede evitar errores de muestreo solo en escenarios de intermedia-alta probabilidad clinica pre-prueba. EnglishBackground. The diagnosis of SARS-CoV-2 infection presents some limitations. RT-PCR in nasopharyngeal swabs is considered the gold standard for the diagnosis, although it can have false negative results. We aimed to analyze the accuracy of repeating nasopharyngeal swabs based on different clinical probabilities. Methods. Retrospective observational study of the first patients admitted to a two COVID Internal Medicine wards at the University Hospital Marques de Valdecilla, Santander, from March to April 2020. RT-PCR targering E, N, RdRP and ORFab1 genes and antibody tests detecting IgG. Results. A total of 145 hospitalized patients with suspected SARS-Cov2 infection were admitted and in 98 (67.5%) diagnosis was confirmed. The independent predictive variables for SARS-CoV-2 infection were: epidemiological contact, clinical presentation as pneumonia, absence of pneumonia in the last year, onset of symptoms > 7 days, two or more of the following symptoms -dyspnea, cough or fever- and serum lactate dehydrogenase levels >350 U/L (p Conclusions. We have defined a pre-test probability score based on epidemiological and clinical data with a high accuracy for diagnosis of SARS-CoV-2. Repeating nasopharyngeal swabs avoids sampling errors, but only in medium of high probability pre-test clinical scenarios.

Objective To communicate relevant and striking aspects about the visual system of some close invertebrates. Material and methods Review of the related literature. Results The capacity of snails to regenerate a complete eye, the benefit of the oval shape of the compound eye of many flying insects as a way of stabilizing the image during flight, the potential advantages related to the extreme refractive error that characterizes the ocelli of many insects, as well as the ability to detect polarized light as a navigation system, are some of the surprising capabilities present in the small invertebrate eyes that are described in this work. Conclusions The invertebrate eyes have capabilities and sensorial modalities that are not present in the human eye. The study of the eyes of these animals can help us to improve our understanding of our visual system, and inspire the development of optical devices.

Background:Anti-TNFα agents are useful in uveitis(1-5).Certolizumab pegol (CZP) differs from other anti-TNFα agents due to its limited placental transfer.Objectives:To assess efficacy and safety of CZP in women with uveitis during pregnancy.Methods:Multicenter study of women with uveitis under CZP during pregnancy and their neonates.Results:14 women (23 eyes); mean age 34.3±5.5 yrs (TABLE 1). Pattern of uveitis: 10 anterior, 2 posterior, 1 intermediate, 1 panuveitis. Uveitis was bilateral in 9 and chronic in 7. CZP was started before conception in 10 patients and after in 4. All patients obtained or maintained ocular remission throughout pregnancy (FIGURE). Prednisone was reduced from a mean dose of 21.7±19.7 mg/day to 4.1±3.8 mg/day at 6 months (p=0.03), leading to complete discontinuation in 4. 15 healthy infants were born. Only 1 woman presented a mild infection. No infections or malformations were found in neonates after a follow-up of 6 months. 6 infants were breastfed and all received scheduled vaccinations without complications (TABLE 2).TABLE 1.AgeUnderlying diseaseImmunosuppressants before CZPCombined treatment134SpAMTX, AZA, ADAAZA237SpAMTX, AZA, IFX, ADA, GOLI339SpAAZA, ADAAZA446SpACyA, ETN, ADA, IFX, GOLI532SpASSZ, ADASSZ636SpAMTX, HCQ, ADA740SpAMTX, LFN, HCQ, IFX, ADA, GOLIHCQ831IdiopathicMTX, MMF, CyA, ADA933IdiopathicMTX, AZA, ADA, ETN1032RAMTXAZA1123Vogt-Koyanagi-HaradaAZA, ADAAZA1236Juvenil Idiopathic ArthritisADA1332Punctate inner choroidopathyADA1429BehcetCyA, IFX, ADAConclusion:CZP seems to be effective and safe in female patients with uveitis during pregnancy and neonates.References:[1]Llorenç V et al. Certolizumab Pegol, a New Anti-TNF-α in the Armamentarium against Ocular Inflammation. Ocul Immunol Inflamm. 2016;24(2):167-72. doi: 10.3109/09273948.2014.967779[2]Urruticoechea-Arana A et al. Efficacy and safety of biological therapy compared to synthetic immunomodulatory drugs or placebo in the treatment of Behçet’s disease associated uveitis: a systematic review. Rheumatol Int. 2019 Jan;39(1):47-58. doi: 10.1007/s00296-018-4193-z[3]Martín-Varillas JL et al. Successful Optimization of Adalimumab Therapy in Refractory Uveitis Due to Behçet’s Disease Ophthalmology. 2018 Sep;125(9):1444-1451. doi: 10.1016/j.ophtha.2018.02.020[4]Santos-Gómez M et al. The effect of biologic therapy different from infliximab or adalimumab in patients with refractory uveitis due to Behçet’s disease: results of a multicentre open-label study. Clin Exp Rheumatol. 2016. Sep-Oct;34(6 Suppl 102): S34-S40[5]Calvo-Río V et al. Golimumab in refractory uveitis related to spondyloarthritis. Multicenter study of 15 patients.Semin Arthritis Rheum. 2016 Aug;46(1):95-101. doi: 10.1016/j.semarthrit.2016.03.002Disclosure of Interests:D. Prieto-Peña: None declared, Monica Calderón-Goercke: None declared, Alfredo Adan: None declared, Lillian Chamorro-López: None declared, Olga Maiz: None declared, JR De Dios-Jiménez Aberásturi: None declared, Raul Veroz Gonzalez: None declared, Soledad Blanco: None declared, José M Santos: None declared, Francisco Navarro: None declared, Adela Gallego: None declared, Senen González-Suárez: None declared, Arantxa Conesa: None declared, Andrea García-Valle: None declared, Miguel Cordero-Coma: None declared, Nieves Pardiñas-Barón: None declared, Rosalía Demetrio-Pablo: None declared, Vanesa Calvo-Río Grant/research support from: MSD and Roche, Speakers bureau: AbbVie, Lilly, Celgene, Grünenthal, UCB Pharma, Victor Manuel Mora-Cuesta: None declared, Santos Castañeda: None declared, J. Luis Hernández: None declared, Miguel A González-Gay Grant/research support from: Pfizer, Abbvie, MSD, Speakers bureau: Pfizer, Abbvie, MSD, Ricardo Blanco Grant/research support from: AbbVie, MSD, Roche, Consultant of: Abbvie, Eli Lilly, Pfizer, Roche, Bristol-Myers, Janssen, UCB Pharma and MSD, Speakers bureau: Abbvie, Eli Lilly, Pfizer, Roche, Bristol-Myers, Janssen, UCB Pharma. MSD

Background Giant cell arteritis (GCA) is a large vessel vasculitis that has a special predilection for extracranial branches of the external carotid artery. Among its most fearsome complications is visual affectation. Tocilizumab (TCZ) is a monoclonal antibody directed against the interleukin 6 receptor that has shown utility in the treatment of GCA. Objectives Our aim was to assess the evolution of visual clinic in patients with GCA treated TCZ. Methods Retrospective multicentre study of 20 GCA patients with visual involvement treated with TCZ. The efficacy of this drug on visual symptoms was evaluated. Results We evaluated 20 patients (14 women and 6 men) with a mean age ±SD of 73.7±10.1 years with GCA and visual symptoms. In total there were 23 affected eyes. The symptoms reported were: unilateral blindness (n=6), unilateral blurred vision (n=6), unilateral amaurosis fugax (n=3), unilateral hemianopsia (n=2), bilateral blindness (n=1), bilateral blurred vision (n=1), bilateral hemianopsia (n=1). Before starting treatment with TCZ all patients had received high doses of prednisone, with a mean ±SD of 54.2±13.8 mg/day (range of doses: 40–80 mg/day). In addition, 9 of them also received intravenous corticosteroid boluses. In addition, 13 patients received traditional immunosuppressants: methotrexate (MTX) (n=12), cyclophosphamide (n=2), leflunomide (n=1) and azathioprine (n=1). Regardless of corticosteroids, TCZ was administered as monotherapy in 14 patients, while in 6 it was administered in combination with MTX. The TABLE shows the evolution of the visual affectation of these patients. Throughout a median follow-up [RIC] of 94–18 months, none of the 7 patients who had a blindness regained vision. The 2 patients who presented unilateral hemianopsia recovered vision. The patient with bilateral hemianopsia and the patient with bilateral blurred vision experienced a partial improvement. The rest of the patients achieved a complete recovery. Conclusions Although TCZ seems to be also useful in the treatment of visual manifestations of ACG, once blindness is established, it does not seem to be effective. Disclosure of Interest None declared

Background Polymyalgia rheumatica (PMR) is a common inflammatory rheumatic disease of the elderly whose diagnosis is usually based on clinical and ultrasound findings. Recently, 18F-FDG PET/CT has been proposed as a promising one-step tool for assessing extent and severity of PMR. However, the pattern of 18F-FDG uptake in PMR is not well established and there is a lack of imaging guidelines. Objectives Our aim was to describe the musculoskeletal pattern of 18F-FDG uptake in PMR patients and assess if there were any differences between classic and atypical PMR. Methods Retrospective study of 75 patients with PMR and their respective PET/CT scans from a referral centre. We considered two groups: a) Classic PMR: patients who fulfilled the 2012 EULAR/ACR criteria; and b) Atypical PMR: patients with symptoms resembling PMR but did not fulfil the 2012 EULAR/ACR criteria. Distributions of categorical variables were compared by Pearson Chi2 or Fisher exact test as appropriate. Results We evaluated 75 patients (27 men and 48 women) with a mean age ±SD of 68.2±10.7 years. A PET/CT was performed in all of them. Forty-two (56%) patients classic PMR and 33 (44%) atypical PMR. FDG-PET uptake was observed in the following musculoskeletal regions: in shoulders (n=45), sternoclavicular joints (n=33), hips (n=32), cervical interspinous bursae (n=8), lumbar interspinous bursae (n=29), pubic symphysis (n=4), subtrochanteric bursae (n=20), ischial tuberosities (n=19) and knees (n=33). The comparative study between both groups is shown in the TABLE, without observing any statistical significance. Conclusions In patients with PMR, 18F-FDG uptake seems to be more frequent in shoulders, sternoclavicular joints, hips and knees. In addition, 18FDG uptake can be also detected in lumbar interspinous bursae and less frequently in subtrochanteric bursae, ischial tuberosities, cervical interspinous bursae and pubic symphysis. No differences between classic and atypical PMR patients were seen. Disclosure of Interest None declared

Background Uveitis is the most common ocular manifestation in Behcet’s Disease (BD), which can cause irreversible blindness.1–2 Objectives To assess efficacy, safety and cost-effectiveness of adalimumab (ADA) therapy optimisation in a series of patients with uveitis due to BD. Methods Multicenter study of 74 ADA-treated patients with BD uveitis refractory to conventional immunosuppressants. Following remission, optimisation was performed by increasing the ADA dosing interval. Comparison between optimised and non-optimised group was performed. Results Ocular remission was achieved in 65 (86.6%) patients after a median ADA duration of 63–12 months. ADA was optimised in 23 cases. In the remaining 42 ADA was maintained at 40 mg/sc/2 weeks. No baseline differences were found at ADA onset between the optimised and non-optimised groups. Ocular outcomes were similar after a mean ±S.D. follow-up of 34.7±13.3 and 26±21.3 months in the both groups (table 1). Adverse effects were seen in non-optimised group (lymphoma, pneumonia, local reaction and bacteremia). Mean ADA treatment costs were lower in the optimised vs non-optimised group (6101.25 €/patient/year vs 12339.48). Conclusions ADA optimisation in BD uveitis refractory to conventional therapy is effective, safe and cost-effective. References [1] Calvo-Rio V, Blanco R, Beltran E, et al. Anti-TNF-alfa therapy in patients with refractory uveitis due to Behcet’s disease: a 1-year- follow-up study of 124 patients. Rheumatol. 2014; 53(12):2223–31 [2] Santos-Gomez M, Calvo-Rio V, Blanco R, et al. The effect of biologic therapy different from infliximab or adalimumab in patients with refractory uveitis due to Behcet’s disease: results of a multicentre open-label study. Clin Exp Rheumatol. 2016; 34 (6Suppl 102):S34-S40 Disclosure of Interest None declared

Background Behcet’s disease (BD) is characterised by recurrent oral and/or genital ulcers accompanied by ocular, cutaneous, articular, gastrointestinal, and/or neurologic manifestations. Oral and/or genital aphthous ulcers are often refractory to conventional treatment. Apremilast is an orally-active small molecule which inhibits phosphodiesterase-4 (PDE-4) that modulates some inflammatory pathways. Objectives Our aim was to assess the efficacy of apremilast in BD patients with oral and/or genital ulcers refractory to conventional treatment. Methods Retrospective national multicenter open-label study on 19 BD patients treated with apremilast at standard dose of 30 mg twice daily. The main outcome was achievement of oral ulcers remission. Results We included 19 patients (14 women and 5 men) with a mean age of 43.6±14.8 years. Before apremilast, all patients had also received several systemic conventional drugs: oral corticosteroids (n=18), colchicine (n=19), NSAIDs (n=10), methotrexate (n=10), azathioprine (n=10), cyclosporine (n=6), infliximab (n=3), adalimumab (n=5), dapsone (n=3), etanercept (n=1), mycophenolate mofetil (n=1), tocilizumab (n=1). The main clinical symptoms for starting apremilast were oral aphthous ulcers (n=19) and genital ulcers (n=14). Other manifestations present at apremilast onset were arthralgia/arthritis (n=6), folliculitis/pseudofolliculitis (n=6), asthenia (n=5), furunculosis (n=1), erythema nodosum (n=1), erythematosus and scaly skin lesions (n=1), psoriasis (n=1), deep venous thrombosis (n=2) and ileitis (n=1). Table 1 shows the evolution of the patients. After a median follow-up of 6 [interquartile range, 5–10] months, most of the patients experienced clinical improvement. In this period of time, 11 patients developed any side-effect: dyspepsia (n=5), nauseas (n=4), diarrhoea (n=4), abdominal pain (n=4), headache (n=3), loss of appetite (n=3), weight loss (n=1) and halitosis (n=1). Three patients had to reduce the dose to 30 mg/day. Apremilast was discontinued in 4 patients: because of not obtaining the expected improvement (n=2), due to desire of pregnancy (n=1) and due to development of neurological involvement (n=1). Conclusions Apremilast leads to a rapid and maintained improvement in many patients with refractory mucocutaneous ulcers of BD. Even in patients refractory to several systemic drugs including biologic therapy. However, the development of adverse digestive effects is frequent. Disclosure of Interest None declared

Background Isolated non-infectious aortitis is a potentially serious condition whose diagnosis is often delayed due to the non-specific nature of its symptoms. Most of the patients initially present with polymyalgic syndrome associated with atypical symptoms. Nowadays 18 F-FDG PET/CT scan is the best tool for early detection of aortitis. However, it is a complex and expensive technique. Thus, we should make an effort to select those patients who will benefit the most from this procedure. Unfortunately, we have no data about which factors may improve the probability of a positive diagnosis of aortitis using this imaging technique. Objectives Our aim was to evaluate, in patients with polymyalgia rheumatica (PMR), the predictive factors for a positive 18F-FDG PET/CT scan, in order to make an early diagnosis of aortitis and optimise the use of this technique. Methods Retrospective study on 97 patients with PMR who had undergone an 18F-FDG PET/CT scan between January 2010 and August 2017 with a high clinical suspicion of aortitis. Only patients with polymyalgic syndrome without any other underlying disease were included. We considered two groups: a) Classic PMR: patients who fulfilled the 2012 EULAR/ACR criteria; and b) Atypical PMR: patients with symptoms resembling PMR but did not fulfil the 2012 EULAR/ACR criteria. Distribution of categorical variables was compared by the Pearson Chi-squared test or Fisher exact test. Quantitative variables were analysed using the Student t test or Mann Whitney U test as appropriate. An adjusted logistic regression model was built to assess the best set of predictive factors for a positive PET scan in both groups of patients. Results 18F-FDG PET/CT scans were performed in 97 patients due to clinical suspicion of aortitis, being positive in 60 (61.9%). Patients (60 women/37 men) had a mean age of 68.4±10.7 years. Fifty-one (52.6%) had classic PMR and 46 (47.4%) atypical PMR. In patients with classic PMR, the best set of predictors for a positive PET/CT scan were lower limb pain (OR=8.4, 95% CI 2.0–35.1; p=0.004) and low back pain (OR=7.6, 95% CI 1.3–45.5, p=0.027), once adjusted for age, sex and current tobacco use. In atypical PMR patients, only the pelvic girdle affection (OR: 5.0, 95% CI 1.3–19.5; p=0.002) was significantly associated with a positive PET/CT scan, in the logistic adjusted model. Conclusions We have found that, in patients with classic PMR, the presence of diffuse lower limb pain and inflammatory low back may have clinical relevance when requesting a PET/CT scan due to aortitis suspicion. On the other hand, in patients with atypical PMR, only the presence of pain in the pelvic girdle seems to be a predictive factor for a positive result in PET/CT scan. Disclosure of Interest None declared

Background Cystoid macular oedema (CME) represents the leading cause of blindness in uveitis of different immune-mediated inflammatory diseases (IMIDs). Objectives Our aim was to evaluate the efficacy of Tocilizumab (TCZ) in different IMIDs with refractory CME. Methods Multicentre study of 24 patients with CME due to uveitis of different IMIDs refractory to traditional treatment with systemic corticosteroids and at least one conventional immunosuppressive drug including in most cases biological therapy (n=21). CME was defined by (OCT >300 µm). We studied CME with TCZ in 4 different IMID; juvenile idiopathic arthritis (JiA), Behcet’s disease (BD), Birdshot retinochoroidopathy (BR) and idiopathic. The main outcome was the improvement of macular thickness. Other variables assessed were inflammation of the anterior chamber and vitreous and best corrected visual acuity (BCVA) Results We studied 16 ♀/8 ♂, mean age 35.2±19.3 years. The associated diseases were: JiA (n=9), BD,7 BR4 and idiopathic.4 The ocular patterns were: panuveitis,9 anterior uveitis,6 posterior uveitis5 and intermediate uveitis.4 Most patients had bilateral involvement.22 The biological therapy used before the administration of TCZ were infliximab,8 adalimumab,18 etanercept,2 golimumab,2 rituximab,2 abatacept,3 anakinra1 and daclizumab.1 TCZ administration schedule was 8 mg/kg/4 weeks iv.23 or every 2 weeks.1 TCZ was used in monotherapy12 or combined with conventional immunosuppressive drugs.12 OCT values improved considerably in 12 months: in JiA from 340.6±134.1 µm to 252.5±30 µm, in BD from 375.1±117 µm to 235±7.1 µm, in BR from 550.7±214.4 µm to 295.5±43.2 µm and in idiopathic from 515±219.6 µm to 208.3±46.7 µm (figure 1). Inflammation in anterior chamber and vitritis and BCVA also improved in the 4 subtypes. No minor side effects were observed, so no patient had to stop treatment. Conclusions TCZ seems a rapid effective treatment in severe and refractory uveitic CME, regardless of the underlying IMIDs. References [1] Calvo-Rio V, de la Hera D. et al. Tocilizumab in uveitis refractory to other biologic drugs: a study of 3 cases and a literature review. Clin Exp Rheumatol2014;32(Suppl 84):S54–S57. [2] Calvo-Rio V, Santos-Gomez M, et al. Anti-IL6-R Tocilizumab for Severe Juvenile Idiopathic Arthritis-Associated Uveitis Refractory to anti-TNF therapy. A multicenter study of 25 patients. Arthritis Rheumatol 2016;69:668–75. [3] Calvo-Rio V, Blanco R, Santos-Gomez M, et al. Efficacy of Anti-IL6-Receptor Tocilizumab in Refractory Cystoid Macular Edema of Birdshot Retinochoroidopathy Report of Two Cases and Literature Review. Ocul Immunol Inflamm2017Oct;25(5):604–609. Disclosure of Interest None declared

Background Rheumatoid arthritis (RA) is associated with accelerated atherosclerosis, which results in high cardiovascular morbidity and mortality. The European Atherosclerosis Society recommends a strict control of cardiovascular (CV) risk factors, focusing in a healthy lifestyle, for the prevention of future CV events. Objectives Our aim was to assess the characteristics of healthy lifestyle in patients with RA and very high risk of CV events. Methods Prospective study that included a group of 113 patients, of whom 65 presented carotid plaques on carotid ultrasound study and, due to this, were categorised as having very CV high risk at the baseline visit. At this time, patients were informed about the risks associated to their disease and the high risk of having CV events. They were even warned of the need of healthy lifestyle habits. Data on lifestyle, smoking, obesity and diabetes mellitus were collected at baseline and last visit (with an interval of more than 5 years) Results The results of the 65 patients at the last visit are shown in table 1. The average age was of 67 years, with a disease duration of 17 years and high prevalence of dyslipidemia (72%), HTA (62%) and obesity (40%). Data obtained at the basal and last visit (with an average difference of 5.5 years) were compared in table 2. At last follow-up none of the smokers at baseline had discontinued smoking. There were no significant differences in the body mass index (BMI) average. Nevertheless, there was increase in the number of patients with a BMI 3 times/week) and did not have a sedentary lifestyle (50%), despite having a mean age of 67 years and long disease duration at baseline. Poor control of diabetes mellitus (DM), was found in more than half of the patients. Conclusions Despite having been informed on the high risk of CV events, patients with RA included in the category of very high CV risk performed poor long-term control of factors that include a healthy lifestyle. Disclosure of Interest None declared

Background Polymyalgia rheumatica (PMR) is an inflammatory disease characterised by pain and stiffness of the neck, shoulder and pelvic girdles. It can also be accompanied by other non-specific symptoms such as inflammatory low back pain, diffuse lower limb pain and constitutional syndrome. The role of 18F-FDG PET/CT is increasing in the diagnosis of PMR but it remains unknown if there is a correlation between clinical symptoms and 18F-FDG uptake. Objectives Our aim was to asses if the localization of pain of patients with PMR correlates with 18F-FDG uptake in the corresponding region of interest. Methods Retrospective study of 75 patients with PMR and their respective PET/CT scans from a referral centre. PMR diagnosis was based on 2012 EULAR/ACR criteria. Results We evaluated 75 patients (27 men and 48 women) with a mean age ±SD of 68.2±10.7 years. A PET/CT was performed in all of them. Pattern of F-FDG uptake in patients with different clinical manifestations were summarised in the TABLE. Twenty-two out of thirty-three patients (66.7%) with 18F-FDG uptake in sternoclavicular joints had shoulder girdle pain. In all patients with morning stiffness (n=11) an increase of 18F-FDG uptake in shoulders was observed. Twenty-three patients of thirty-two patients (71.8%) with18F-FDG uptake in hips had pelvic girdle pain. The remaining localizations of 18F-FDG uptake in PET/CT scans did not show significant correlations with clinical symptoms. Conclusions In patients with PMR, the presence of shoulder girdle pain seems to correlate with F-18FDG uptake in sternoclavicular joints, morning stiffness with 18F-FDG uptake in shoulders and pelvic girdle pain with 18FDG uptake in hips. No other significant correlations were found between any other symptom and 18F-FDG uptake. Disclosure of Interest None declared

Background In uveitis, remission-inducing therapy with even more vigor than does rheumatology is mandatory. Since the eye is so much less forgiving of chronic inflammation than is the joint, with profound life-altering consequences. Cystoid macular edema (CME) is the leading cause of blindness in uveitis. Objectives To evaluate the rapid efficacy of Tocilizumab (TCZ) in refractory CME. Methods Multicentre study of 25 patients with CME due to non-infectious uveitis who had inadequate response to traditional treatment with corticosteroids and at least one conventional immunosuppressive drug including in most cases biological therapy (n=22). CME was defined as OCT >300 μm. The outcome variables were the degree of inflammation, visual acuity, macular thickness. The results are expressed as mean±SD for normally distributed variables, or as median [IQR] when are not. Comparison of continuous variables was performed using the Wilcoxon test. Results 25 patients (17 women/8 men), mean age 33.6±18.9 years were studied. The associated diseases were: juvenile idiopathic arthritis (9), Behcet9s (7), Birdshot (4), idiopathic (4), sarcoidosis (1). The ocular pattern was: panuveitis (9), anterior uveitis (7), posterior uveitis (5) and intermediate uveitis (4). Most patients had bilateral involvement (24). Prior to TCZ they received: intraocular corticosteroids (22), iv. methylprednisolone (7), methotrexate (MTX) (19), cyclosporine A (CSA) (17), mycophenolate (4), azathioprine (2), leflunomide (2), cyclophosphamide (1), sulfasalazine (1), acetazolamide (1) and thalidomide (1). The biological used before TCZ were infliximab (8), adalimumab (19), etanercept (2), golimumab (2), rituximab (2), abatacept (3), anakinra (1) and daclizumab (1). TCZ administration schedule was 8 mg/kg/4 weeks iv. (n=23), every 2 weeks (1) and subcutaneously 162 mg/2 weeks (1). TCZ was used in monotherapy (13) or combined with conventional immunosuppressive drugs (12). Most of intraocular inflammation parameters showed a rapid improvement after TCZ onset (Table), with corticosteroid-sparing effect (15.9±13.6 to 8.5±5.17 mg; p=0.001). Remission was achieved in 8 patients and improvement in 17. After one month of therapy, no side effects were observed. Conclusions TCZ seems a rapid effective treatment in refractory uveitic CME. Disclosure of Interest None declared

Background Uveitis is a severe manifestation of Juvenil Idiopathic Arthritis (JIA). Anti-TNFa are recommended in refractory cases, mainly infliximab (IFX) or adalimumab (ADA) (Levy-Clarke et al. Ophthalmology 2014; 121: 785–796). However, sometimes they are ineffective, contraindicated or not tolerated. The next therapeutic step is not defined. Objectives To compare the efficacy of Golimumab (GLM) and Tocilizumab (TCZ) in related AIJ uveitis refractory to conventional immunosuppressive drugs and anti-TNFα. Methods Multicenter study of 33 patients with uveitis associated-JIA. They were refractory to conventional treatment with high dose of corticosteroids and at least a) one conventional immunosuppressive drug and b) one anti-TNFa. For this reason it was decided to iniciate TCZ or GLM. TCZ was used in 25 patients: 8 mg/kg/4 w iv (n=21), 8 mg/kg/2 w (n=2); 8 mg/kg/8 w (n=1) and 2.9 mg/kg sc/w (n=1). GLM was used in 8 patients (50 mg/sc/month). We assessed visual acuity (VA), degree of intraocular inflammation, vitreous inflammation and macular thickening (with OCT). Quantitative variables were expressed with mean±SD or median [IQR], according to its distribution. They were compared with the Student t or the Mann-Whitney U test, respectively. Dichotomous variables were expressed as percentages and compared by the chi-square test. Results We studied 33 patients/61 affected eyes. There were no significant differences between TCZ and GLM at baseline in sex (♂/♀;4/21 vs 3/5; p=0.19), mean age (18.5±8.3 vs 19.9±8.7; p=0.55), positive ANA (95% vs 100%; p=0.7), uveitis duration before TCZ or GLM onset (116.4±93.6 vs 142.3±74.7 p=0.46), number of previous biological treatments (1.9±1.1 vs 2±1.4; p=0.84), VA (0.57±0.35 vs 0.5±0.37; p=0.42), combined immunosuppressive therapy (88% vs 75%; p=0.37), presence of cells in the anterior chamber (median, 1 [0–1] vs 1 [0.25–1.5]; p=0.6), vitritis (0 [0–0] vs 0 [0–1]; p=0.7), macular thickening (358.7±92.2 vs 313.6±77.1; p=0.32). There were no significant difference in the efficacy between TCZ and GLM (TABLE). After a mean follow-up of 20.48±11.7 months with TCZ and 24.25±17 months with GLM the following side effects were observed: TCZ: viral conjunctivitis plus bullous impetigo (n=1), severe thrombocytopenia and pneumonia. This last patient showed hemolytic anemia, thrombocytopenia and splenomegaly, for this reason treatment with TCZ was discontinued. With GLM cutaneous reaction was observed in 2 patients. Conclusions TCZ and GLM seem to be equally effective and safe for refractory uveitis associated-JIA. The superiority of one or the other should be established with prospective randomized studies “Head to Head” Disclosure of Interest None declared

Background Rheumatoid arthritis (RA) patients have higher levels of resistance to the action of insulin (IR) compared to healthy subjects.The “Incretin effect” consists in a greater release of insulin by the pancreas when there is a gastrointestinal glucose stimulation, compared to an intravenous stimulation. It is known that this effect is altered in patients with IR. Objectives To determine if the incretins-insulin axis is altered in patients with RA and if this correlates to IR in patients with RA, as well as if it is explainable by certain features of the disease. Methods Cross-sectional study that includes 361 non-diabetic individuals, 151 patients with RA and 210 controls. Serum levels of insulin, C-peptide, Amylin, glucagon-like peptide 1 (GLP-1), gastric inhibitory polypeptide (GIP) and dipeptidyl peptidase 4 (DPP-4) were analyzed in patients and controls. Indexes of resistance and sensitivity to insulin activity were determined by HOMA2. A “meal test” consisting on the intake of 500 kcal was performed to a subset of 10 patients and 10 controls, in order to determine the postprandial curves of glucose, insulin, C-peptide, GLP-1 and GIP. Differences between patients and controls as well as the relationship of selected characteristics of the disease with the baseline and postprandial levels of incretins were analyzed using multivariate regression. During the meal test, areas under the curve (AUC), maximum concentrations and the minutes of response were compared between patients and controls. This study was approved by the Clinical Trial Committee of the University Hospital of the Canary Islands. Results Patients with RA showed, at baseline and after multivariate adjustment, higher levels of insulin (9.8±6.5 vs 13.0±13.4 U/ml, p=0.05), C-peptide (1.53±0.77 vs. 3.37±2.94 ng/ml, p=0.00), GLP-1 (0.49±1.28 vs. 0.71±0.22, p=0.00) and GIP (0.37±0.40 vs. 1.78±0.51, p=0.00). Similarly, IR indexes such as HOMA2-IR and HOMA2%B, built with insulin or Cpeptide, were higher in patients with RA compared to controls. Patients with RA, showed significantly lower levels of DPP-4 (811±459 vs. 696±301 ng/ml,p=0.02) after multivariate analysis. Amylin levels did not differ between patients and controls. Both C-reactive protein (beta coefficient 0.54,95% CI 0.16–0.96, p=0.013) and Erythrocyte Sedimentation Rate (beta coef. 0.01,0.00–0.01, p=0.033) showed a positive relationship with HOMA2%B and GIP levels, respectively. The presence of anti-cyclic citrullinated peptide antibody (beta coef. 157 CI 58–256, p=0.002) and the levels of disease activity measured by DAS28 (beta coef. 46,CI 6–87, p=0.026) and CDAI (beta coef 1.27 (0.28–2.26), p=0.012) showed a positive relationship with the levels of DPP-4. Patients with RA showed Pearson correlation coefficient of incretines, DPP-4 with insulin, C peptide and IR lower compared to control group. After the meal test patients with RA exhibited an AUC of glucose and GIP greater than controls and a slower C-Peptide response time (75 vs. 30 minutes, p=0.029). Conclusions Incretins-insulin axis is altered in patients with RA compared to controls. Disclosure of Interest None declared

Background Lipid profiles appear to be altered in rheumatoid arthritis (RA) patients due to disease activity and inflammation. Cholesterol efflux capacity (CEC) has not only been linked to cardiovascular events in the general population, but also to be impaired in RA patients. Objectives To analyze whether CEC is related to subclinical atherosclerosis, as determined by the presence of carotid plaque or increased levels of carotid intima-media thickness (cIMT) in RA patients. Secondarily, we aimed to describe the disease-contributing factors that are related to CEC as an expression of the abnormalities in the lipid profile associated with the disease. Methods Cross-sectional study that encompassed 401 individuals; 178 patients with RA and 223 sex-matched controls. CEC was measured using an in vitro assay, lipoproteins serum concentrations, and standard lipid profile were assessed in patients and controls. Carotid intima-media thickness and carotid plaques were assessed in RA patients. A multivariable analysis was performed to evaluate the relation of CEC with RA-related data, lipid profile and subclinical carotid atherosclerosis. Results Mean CEC was not significantly different between RA patients (18.9 ± SD 9.0%) and controls (16.9±10.4%), p=0.11. Demographic variables were not associated with CEC except for a correlation with male gender that was only found in RA patients, but not in controls. Systolic blood pressure inversely correlated with CEC in controls (beta coefficient -0.1 [-0.2–0.0] %, p=0.025). In RA patients, a similar trend was found although a statistically significant difference was not reached. Neither the traditional cardiovascular risk factors nor the cardiovascular co-morbidity-related data were associated with CEC. Similarly, lipid profile did not show any relationship with CEC in patients or controls. ESR tended to be associated with a lower CEC although it did not reach statistical significance. RA patients with low (beta coef. -5.2 [-10.0–0.3] %, p=0.039) and moderate disease activity (beta coef. -4.6 [-8.5–0.7] %, p=0.020) were associated with inferior levels of CEC when compared to patients in remission. CEC was not found to be associated with cIMT in RA patients. However, higher CEC was associated with a protective effect for the presence of carotid plaque in RA patients. This relationship was maintained even after multivariate analysis (OR 0.94 [0.89–0.98], p=0.015). Conclusions Our study, which includes the largest series of RA patients ever assessed for CEC, reveals for the first time that CEC is related to subclinical atherosclerosis in RA patients. The fact that CEC is also associated with disease activity reinforces the idea that CEC may be a mediator between disease activity and subclinical atherosclerosis. Disclosure of Interest None declared

Objectives To assess the efficacy of tocilizumab (TCZ) at short and long term follow-up for severe juvenile idiopathic arthritis-associated uveitis. Methods Multicentre study of 25 patients who had inadequate response to traditional treatment with corticosteroids and at least one conventional immunosuppressive drug including biological therapy. The outcome variables were the degree of inflammation, visual acuity and macular thickness. The results are expressed as mean±SD for normally distributed variables, or median [IQR] when are not. Comparison of continuous variables was performed using the Wilcoxon test. Results We studied 25 patients (21 women/4 men); mean age 18.6±8.3. Uveitis was bilateral in 22. JIA subsets were oligoarthritis (n=17), polyarthritis (5), psoriatic (2) and enthesitis-related arthritis (1). Ocular sequelae at TCZ onset were cataracts (13), glaucoma (7), synechiae (10), band keratopathy (12), maculopathy (9), and amblyopia (5). Pattern of uveitis was: anterior (17), panuveitis (4), intermediate (2) and posterior (2). Before TCZ, they had received corticosteroids, conventional immunosuppressive drugs and biologics, including adalimumab (24), etanercept (8), infliximab (7), abatacept (6), rituximab (2), anakinra (1), and golimumab (1). TCZ dosage regimen was 8 mg/kg IV/4 weeks (21), every 2 weeks (2), every 8 weeks (1), or 2.9 mg/kg sc every week (1). All outcome variables showed a rapid and maintained improvement (Table 1) after a follow up of one year (n=21), 2 years (n=11), and 3 years (n=5). A reduction in the daily median dose of prednisone from 10 mg [0–15 mg] to 0 [0–0 mg] in 3 years, (p Conclusions TCZ is useful at short and long term follow-up for severe Juvenile Idiopathic Arthritis-associated uveitis. It is possible to optimize the TCZ dose. Disclosure of Interest None declared

Background Non-infectious aortitis is an inflammation of aortic wall which may be isolated or associated with a cluster of diseases. Objectives Our aim was to compare the clinical and laboratory findings of patients with isolated aortitis and patients with aortitis secondary to other underlying conditions. Methods Retrospective study of 93 patients with non-infectious aortitis diagnosed by PET/CT scan from a referral center from January 2010 to December 2016. We have considered two groups: group a) isolated aortitis; and group b) secondary aortitis. Distributions of categorical variables were compared by the Pearson Chi2 or Fisher exact test. Quantitative variables were analyzing using the Student t test or Mann-Whitney U test as appropriate. Results Ninety-three patients were diagnosed with non-infectious aortitis. One patient was excluded due to missing data. Group a) was composed by 54 patients (34 women/ 20 men) with a mean age of 67±11 years; group b) comprised 38 patients (28 women/ 10 men) with a mean age of 68±11 years. In this group, the underlying conditions we found were: giant cell arteritis (n=24), Takayasu arteritis (n=3), spondiloarthropathy (n=3), Sjogren9s syndrome (n=3), ulcerative colitis (n=2), sarcoidosis (n=1), rheumatoid arthritis (n=1), polyarteritis nodosa (n=1). The comparative study between both groups is shown in the TABLE. Only inflammatory low back pain and polymyalgic syndrome yielded statistical signification. Conclusions In this study, we observed that both the presence of inflammatory low back pain and polymyalgic syndrome might have clinical relevance in the clinical suspicion of primary aortitis. However, larger studies are needed to corroborate these findings. Disclosure of Interest None declared

Background Disease modifying antirheumatic drugs (DMARDs) such as methotrexate (MTX), leflunomide (LFN) or antiTNFα have been implicated in development/exacerbation of Interstitial lung disease (ILD)of rheumatoid arthritis (RA). Several radiological patterns of ILD have been described: i) usual interstitial pneumonia (UIP), ii) nonspecific interstitial pneumonia (NSIP), iii) obliterating bronchitis (OB), and iv) Organized pneumonia (OP) Objectives To assess the response to Abatacept (ABA) in these patterns of ILD Methods Multicenter study of RA-ILD treated with ABA. ILD was diagnosed by high-resolution CT scan (HRCT) and classified in radiological patterns (Travis et al). We consider 3 subgroups: a) UIP, b) NSIP and c) “other” (OB, OP or mixed). ABA was used at iv or sc standard dose. We assessed: a) Dyspnea (Medical Research Council-modified scale; significant variations≥1); B) Respiratory function tests; significant changes≥10% in forced vital capacity (FVC) and DLCO≤10%, c) HRCT, d) DAS28. A comparative study was performed for the quantitative (U-Mann-Whitney) and qualitative variables (Fisher test) between the baseline and 3, 6 and 12 months. Results We included 63 patients (27 women/36 men), mean age; 63.1±9.6 years. At ABA onset the RA had a median evolution of 6.8 [2–13.6] years and the ILD of 1 [0.3–3.03]. RA was seropositive in 85.7%. The diagnosis of ILD was confirmed by biopsy (n=18). The ILD was related to DMARDs: MTX (4), etanercept (3), adalimumab (3), certolizumab (2), Infliximab (1). ABA was used in monotherapy (26) or combined with other DMARDs (37); LFN (15), Cyclosporin (1), sulfasalazine (4), MTX (6), hydroxychloroquine (10), azathioprine (4), chloroquine (1). Table 1 shows the evolution in the available cases. A significant improvement in dyspnea and HRCT was observed in the NIU type. DLCO remained stable in most patients regardless of the radiological pattern. The activity of RA (DAS28) also improved. Conclusions ABA appears to be effective in ILD associated-RA, including the pattern of poor prognosis (UIP). References Travis WD et al. J Respir Crit Care Med 2013 188:733–748. Disclosure of Interest None declared

Background Baseline characteristics of patients from GiACTA trial have been recently reported at the ACR-2016 conference (1). GiACTA trial is a randomized, phase III controlled clinical trial evaluating the efficacy of tocilizumab (TCZ) in giant cell arteritis (GCA) (2). We had previously published a multicenter study on the use of TCZ in refractory GCA in a clinical practice setting (3). Objectives Our aim was to compare both studies, emphasizing on the baseline characteristics of the patients. Methods Comparative study between the GiACTA trial and our multicenter clinical practice study. In the latter, the diagnosis of GCA was established by the ACR-1990 criteria and in the GiACTA trial by the ACR modified criteria. In the clinical practice study, TCZ was used at standard IV dose (8 mg/kg/month), while in the GiACTA trial it was given subcutaneously (162 mg every 1 or 2 weeks, depending on the therapeutic arm). Quantitative variables were expressed as mean ± SD and were compared with the Student9s t-test. Dichotomous variables were expressed as percentages and compared using the chi-square test. Results In the GiACTA trial, 47.4% were newly diagnosed GCAs, while in the clinical practice study were all refractory to conventional treatment. The TABLE summarizes the main baseline characteristics of both studies. Compared with the GiACTA trial, in the clinical practice study were significantly greater: a) the mean time between the diagnosis of GCA and the onset of TCZ, b) the proportion of patients with polymyalgia rheumatica and ischemic optic neuritis, c) the proportion of positive PET/CT scans, d) the mean value of ESR, and e) the proportion of patients who had received conventional immunosuppressant agents (mainly MTX) before starting TCZ. There was also a significant lower proportion of sustained remission in the clinical practice study. When only GiACTA patients with relapsing-GCA were analyzed, these differences were maintained, except for the mean time from GCA diagnosis and the prevalence of ischemic optic neuropathy. Data on remissions were not available in this subgroup of GiACTA patients. Conclusions Patients receiving TCZ in the clinical practice study have several baseline clinical and laboratory differences with regard to those included in the GiACTA trial and, therefore, data of this trial should be taken cautiously when applied in a real-world scenario. References Stone J et al. Arthritis Rheumatol.2016; 68 (suppl 10). http://acrabstracts.org/abstract/efficacy-and-safety-of-tocilizumab-in-patients-with-giant-cell-arteritis-primary-and-secondary-outcomes-from-a-phase-3-randomized-double-blind-placebo-controlled-trial/. Tuckwell K et al. Semin Arthritis Rheum. 2016 Nov 15. pii: S0049–0172(16)30275-X. Loricera J et al. Semin Arthritis Rheum. 2015; 44: 717–23. Disclosure of Interest None declared

Background Aortitis is the inflammation of the aortic wall. This entity is often under-recognised due to its frequent presentation with non-specific symptoms. PET/CT scan represent a major breakthrough to establish an early diagnosis, but this is an expensive technique. Objectives Our aim was to compare the baseline characteristics of patients with a suspicion of aortitis and positive results on PET/CT scan, and those with a negative result, in order to search for predictive factors, that improve the clinical probability of diagnosis aortitis by this imaging technique. Methods Retrospective study on 170 patients and PET/CT scans ordered by suspicion of aortitis from a referral center from January 2010 to December 2016. According to a pre-specified protocol, the baseline epidemiological and clinical variables of patients with positive and negative PET/CT scans results for aortitis were reviewed. Distributions of categorical variables were compared by the Pearson Chi 2 or Fisher exact test. Quantitative variables were analyzing using the Student t test or Mann-Whitney U test as appropriate. Results In 170 patients, PET/CT scans were performed due to clinical suspicion of aortitis, and were positive in 93 (54.7%) cases. Patients (113 women/57 men) had a mean age of 67.7±13.1 years (range, 20–90 years). One patient was excluded because missing clinical or laboratory data. The underlying diseases at the moment of ordering the PET/CT scan were: giant cell arteritis (GCA) (n=28), spondiloarthropaties (n=7), conectivopaties (n=6), Takayasu arteritis (n=3), ulcerative colitis (n=3), other condition (n=11). The remaining 111 patients did not have any underlying condition suggestive of aortitis. Two out of 170 patients suspected an infectious aortitis ( Brucella and Salmonella ); however, PET/CT was negative in both cases. Characteristics of patients with positive and negative PET/CT scans were summarized in the Table. Patients with GCA had a higher percentage of positive PET/CT scans, whereas they were negative more frequently in patients who did not have any condition suggestive of underlying aortitis. Only inflammatory low back pain and polymyalgic syndrome were significantly more frequent in patients with positive PET/CT scans. The remaining clinical and laboratory variables did not show differences between both groups. Conclusions In this study, we have found that the presence of inflammatory low back pain and polymyalgic syndrome, especially in GCA patients, may have clinical relevance in ordering a PET/CT scan when aortitis was suspected. Disclosure of Interest None declared

Introduction: Tuberous sclerosis complex (TSC) is one of the most frequent neurocutaneous disorders. Cortical tubers are the most common pathological changes in TSC and they are directly related to the disease's main clinical manifestations: seizures, mental retardation, and autistic behaviour. Objective: The aim of this study is to establish a correlation between tuber size and the severity of clinical features in TSC. Material and methods: We performed a retrospective study of the clinical and imaging findings from 45 TSC patients (22 females and 23 males) and compared the clinical features with the location, size, and number of the cortical tubers in each patient. Results: Four patients had voluminous tubers located in 1 or both cerebral hemispheres. All of these patients had intractable seizures and severe mental retardation; 3 of these cases also presented with autistic behaviour, despite tubers having been resected in all 4 patients. Thirteen patients had tubers of large-to-average size, and all patients in this group showed intractable seizures and mental retardation. Nine patients who had experienced infantile spasms during the first year of life presented autistic behaviour. Multiple tubers of small to average size were found in 28 patients. In general, this group had seizures that responded well to antiepileptic drugs and a low prevalence of autism. In 3 patients who all presented good seizure control and normal intelligence, single cortical/subcortical tubers were located in the frontal or occipital lobes. Of the total of 45 patients, 13 had cerebellar as well as cerebral tubers; these were generally present in cases with more severe clinical features. Conclusions: Although large tubers are less common than small to medium-sized ones, they are much more likely to be accompanied by severe clinical symptoms (seizures, mental retardation and autistic behaviour), even when the smaller tubers are quite numerous. Resumen: Introducción: El complejo esclerosis tuberosa (CET) es uno de los trastornos neurocutáneos más frecuentes. Las tuberosidades corticales son las alteraciones patológicas más frecuentes y están relacionadas directamente con las principales expresiones clínicas, crisis epilépticas, retraso mental y comportamiento autista. El motivo de este trabajo es mostrar la importancia de los diferentes tipos de tuberosidades en la expresión clínica de los pacientes. Objetivo: La finalidad de este trabajo es relacionar el tamaño de las tuberosidades con la severidad de las alteraciones clínicas. Material y métodos: Se estudiaron retrospectivamente los hallazgos clínicos y neurorradioló-gicos de 45 pacientes infantiles (22 mujeres y 23 varones) con CET y comparamos los hallazgos clínicos con la localización, el tamaño y el número de las tuberosidades corticales en cada paciente. Resultados: Cuatro pacientes tenían tuberosidades muy voluminosas en los hemisferios cere-brales. Todas mostraban crisis epilépticas muy rebeldes y retraso mental profundo con comportamiento autista en 3 de ellos, pese a que se extirparon las tuberosidades en los 4 casos. Trece pacientes tenían tuberosidades de tamaño promedio-grande. Todos tenían crisis epilépticas muy rebeldes y retraso mental. Nueve pacientes habían tenido espasmos infantiles durante el primer año de vida y presentaban comportamiento autista. Veintiocho pacientes mostraban muchas tuberosidades de tamaño promedio-pequeño. La mayoría de ellos tenían crisis con buena respuesta al tratamiento farmacológico y poca prevalencia del autismo. Tres pacientes mostraban tuberosidad córtico-subcortical única en un polo frontal u occipital, todos ellos con crisis controladas con medicación y cociente intelectual normal. Trece pacientes de los 45 tenían tuberosidades cerebelosas, siempre asociadas a algún tipo de tuberosidad hemisférica y generalmente presentes en casos con mayor expresividad clínica. Conclusiones: Las tuberosidades de gran tamaño, aunque sean poco numerosas, tienen mucha mayor relación con la presencia de sintomatología clínica severa—crisis epilépticas, retraso mental y comportamiento autista—que las tuberosidades de pequeño-mediano tamaño, aunque sean muy numerosas. Keywords: Tuberous sclerosis complex, Tubers, Seizures in tuberous sclerosis complex, Autism in tuberous sclerosis complex, Mental retardation in tuberous sclerosis complex, Magnetic resonance imaging in tuberous sclerosis complex, Palabras clave: Complejo esclerosis tuberosa, Tuberosidades, Crisis epilépticas en el complejo esclerosis tuberosa, Autismo en el complejo esclerosis tuberosa, Retraso mental en el complejo esclerosis tuberosa, Resonancia magnética en el complejo esclerosis tuberosa

Resumen Fundamentos No esta establecida de forma definitiva la importancia de los factores de riesgo vascular (FRV) y del estado de hipercoagulabilidad en los pacientes con obstruccion venosa retiniana (OVR). Objetivos Analizar la importancia de los FRV, la prevalencia de trombofilia y los hallazgos ecograficos en los troncos supraaorticos en los pacientes con OVR. Pacientes y metodo Estudio prospectivo de todos los pacientes con OVR remitidos a una consulta de Medicina Interna. Se analizaron las variables clinicas, de laboratorio (incluido un estudio de trombofilia) y ecograficas. Resultados Se estudiaron 98 pacientes (56 varones y 42 mujeres) con una media de edad de 66 anos. La hipertension y la dislipemia fueron los FRV mas prevalentes. Tenian hiperhomocistinemia un 24% y presentaban placas de ateroma en los troncos supraaorticos el 48%. La OVR fue periferica en 69 y central en 29. En un 13% se detecto una trombofilia genetica y en un 9% adquirida. En los sujetos menores de 50 anos la trombofilia se observo en el 50% y en los que no tenian FRV en el 67% (p = 0,004). El 77% recibieron antiagregantes. Siete estaban anticoagulados por fibrilacion auricular y ello no evito la aparicion de la OVR. En 4 casos se inicio anticoagulacion que se mantuvo de forma indefinida en los 3 pacientes con sindrome antifosfolipido. Conclusiones En los pacientes con OVR parece recomendable el control de los FRV clasicos y la determinacion de los niveles de homocisteina. Nuestros datos sugieren ademas, descartar la presencia de un sindrome antifosfolipido, y solamente en los sujetos menores de 50 anos o sin FRV se podria considerar un estudio de trombofilia. La antiagregacion con aspirina es el tratamiento recomendado para reducir el riesgo vascular global, y solo se consideraria la anticoagulacion en aquellos con trombofilias de mayor potencial trombotico.

Resumen La posible relacion entre la osteoporosis y la artrosis continua debatiendose en la actualidad. Durantedecadas se considero que ambas enfermedades eran mutuamente excluyentes debido a las caracteristi-cas antropometricas y la diferente masa osea que con frecuencia presentan los pacientes con osteoporo-sis y artrosis. Sin embargo, en los ultimos anos se ha senalado la posibilidad de que ambos procesos coe-xistan e incluso que guarden una relacion directa. En el presente articulo revisamos algunos aspectos dela relacion entre estas dos enfermedades siguiendo una perspectiva temporal. Palabras clave: enfermedades musculoesqueleticas, artrosis, osteoporosis. Osteoporosis and osteoarthritis: two mutually exclusive diseasesor two related entities? Summary The possible association between osteoporosis and osteoarthritis represents an ongoing matter of deba-te. It was considered, for decades, that both diseases were mutually exclusive due to the anthropometriccharacteristics and the difference in bone mass that patients with osteoporosis and osteoarthritis oftenpresent. However, in recent years, it was pointed out that both processes can coexist, and even that theymay have a direct relationship. In this paper we review some aspects of the association between bothdiseases from a temporal perspective.Key words

CANDIPOP Project: B. Padilla, P. Muñoz, J. Guinea, J. R. Paño Pardo, J. García-Rodríguez, C. G. Cerrada, J. Fortún, P. Martín, E. Gómez, P. Ryan, C. Campelo, I. de los Santos, Gil, V. Buendía, B. P. Gorricho, M. Alonso, F. S. Sanz, J. M. Aguado, P. Merino, F. González Romo, M. Gorgolas, I. Gadea, J. E. Losa, A. Delgado-Iribarren, A. Ramos, Y. Romero, I. S. Romero, O. Zaragoza, M. Cuenca-Estrella, J. Rodríguez-Baño, A. I. Suarez, A. Loza, A. I. Aller García, E. Martín-Mazuelos, M. R. Pérez de Pipaón, J. Garnacho, C. Ortiz, M. Chávez, F. L. Maroto, M. Salavert, J. Pemán, J. Blanquer, D. Navarro, J. J. Camarena, R. Zaragoza, V. Abril, C. Gimeno, S. Hernández, G. Ezpeleta, E. Bereciartua, J. L. Hernández Almaraz, M. Montejo, R. A. Rivas, R. Ayarza, A. M. Planes, I. R. Camps, B. Almirante, J. Mensa, M. Almela, M. Gurgui, F. Sánchez-Reus, J. Martínez-Montauti, M. Sierra, J. P. Horcajada, L.Sorli, J. Gómez, A. Gené, M. Urrea, A. Mularoni, M.Valerio, A. Díaz-Martín, F. Puchades., [Objectives] The clinical correlation of fluconazole antifungal susceptibility testing (AST) for Candida isolates and its integration with pharmacokinetics/pharmacodynamics (PK/PD) parameters is unclear. We analysed the impact of fluconazole minimum inhibitory concentration (MIC) values, 24-hour area under the concentration–time curve (AUC24) and AUC24/MIC ratio on the outcome of candidemic patients., [Methods] We included 257 episodes of candidaemia treated with fluconazole monotherapy for ≥72 hours from a population-based surveillance conducted in 29 hospitals (CANDIPOP Project). AST was centrally performed by European Committee on Antimicrobial Susceptibility Testing (EUCAST) and Clinical and Laboratory Standards Institute (CLSI) microdilution methods. Primary outcome was clinical failure (30-day mortality and/or persistent candidaemia for ≥72 hours from initiation of therapy). Secondary outcomes included early (3–7 days) and late (3–30 days) mortality., [Results] Rates of clinical failure, early and late mortality among evaluable episodes were 32.3% (80/248), 3.1% (8/257) and 23.4% (59/248). There was no relationship between fluconazole MIC values or PK/PD parameters and clinical failure. Although MIC values ≥2 mg/L by EUCAST (positive predictive value 32.1%, negative predictive value 68.7%) and ≥0.5 mg/L by CLSI (positive predictive value 34.8%, negative predictive value 74.4%) appeared to be optimal for predicting clinical failure, no significant associations remained after multivariate adjustment (odds ratio 1.67; 95% confidence interval 0.48–5.79; p 0.423). Lack of association was consistent for alternative thresholds (including proposed clinical breakpoints). The only association found for secondary outcomes was between an AUC24/MIC ratio >400 h by CLSI and early mortality (odds ratio 0.18; 95% confidence interval 0.04–0.98; p 0.026)., [Conclusions] High fluconazole MIC values did not negatively impact outcome of patients with candidaemia treated with fluconazole. No effect of PK/PD targets on the risk of clinical failure was found., Supported in part by nonrestrictive research grants from Gilead, MSD, Astellas and Pfizer. This study was cofunded by Fundación SEIMC-GESIDA; the Spanish Ministry of Economy and Competitiveness, Instituto de Salud Carlos III (cofinanced by the European Development Regional Fund (ERDF) ‘A Way to Achieve Europe’); and the Spanish Network for the Research in Infectious Diseases (REIPI RD12/0015). MFR holds a ‘Juan Rodés’ clinical research contract (JR14/00036) and JG a ‘Miguel Servet’ contract (CPII15/00006), both from the Spanish Ministry of Economy and Competitiveness, Instituto de Salud Carlos III. MFR has received honoraria for talks on behalf of Pfizer and Gilead Sciences. JG has received grant support from Astellas Pharma, MICOLAB, the Mutua Madrileña Foundation and the Spanish Health Research Fund (FIS). He has been paid for talks on behalf of Gilead Sciences, Merck Sharp & Dohme, Pfizer, Astellas Pharma, Hickma Pharmaceutica and United Medical. BA has received grant support from Gilead Sciences, Pfizer and the Instituto de Salud Carlos III. He has received honoraria for talks on behalf of Gilead Sciences, Merck Sharp & Dohme, Pfizer, Astellas Pharma and Novartis. BP has received honoraria for talks on behalf of Gilead Sciences, Merck Sharp & Dohme, Pfizer, Astellas Pharma and Novartis. MCE has received grant support from Astellas Pharma, bioMérieux, Gilead Sciences, Merck Sharp & Dohme, Pfizer, Schering Plough, Soria Melguizo SA, Ferrer International, the Europea Union, the ALBAN program, the Spanish Agency for International Cooperation, the Spanish Ministry of Culture and Education, the Spanish Health Research Fund, the Instituto de Salud Carlos III (Spanish Ministry of Economy and Competitiveness), the Ramon Areces Foundation and the Mutua Madrileña Foundation.

Introduction: The term focal cortical dysplasia (FCD) describes a particular migration disorder with a symptomatology mainly characterised by drug-resistant epileptic seizures, typical neuroradiological images, and histological characteristics, as well as a very positive response to surgical treatment in the majority of cases. Material and methods: A total of 7 patients were studied, comprising 6 children with a mean age of 34.3 months and one 25-year-old male with very persistent focal seizures and MRI images that showed FCD. Results: Three of the patients (all girls) were operated on while very young, with extirpation of the FCD and the surrounding area; with the histopathology study showed agreement between the MRI images and the macroscopic study of the slices. The histology study showed findings typical of a Taylor-type FCD (poor differentiation between the cortical grey matter and the subcortical white matter, and balloon cells). Three years after the FCD extirpation, the same 3 patients remained seizure-free with no anti-epilepsy medication. Two others have seizure control with medication, another (the adult) is on the surgical waiting list, and the remaining patient refused the operation. Conclusion: Taylor-type FCD is associated with a high percentage of all drug-resistant focal seizures, and it needs to be identified and extirpated as soon as possible. Well planned and well-performed surgery that leaves no remains of dysplasia can cure the disease it in many cases. Resumen: Introducción: El término displasia cortical focal (DCF) expresa una patología muy particular de trastorno de la migración que conlleva una sintomatología caracterizada principalmente por crisis epilépticas fármaco-resistentes, unas imágenes neurorradiológicas y unas características histológicas peculiares, así como una respuesta al tratamiento quirúrgico muy positiva en la mayoría de los casos. Material y métodos: Se estudia a 7 pacientes, 6 niños con edad promedio de 34,3 meses y un varón de 25 años con crisis focales muy rebeldes e imágenes de RM que mostraban DCF. Resultados: Tres de los pacientes (todas niñas) fueron operadas en edades muy tempranas, con extirpación de la DCF y la zona circundante, demostrando el estudio anatómico la concordancia de las imágenes de RM con las macroscópicas de los cortes anatómicos. El estudio histológico mostró los típicos hallazgos de la DCF tipo Taylor (mala delimitación entre sustancia gris cortical y la sustancia blanca subcortical, y «células balonadas»). Tres años después de la resección de la DCF los 3 pacientes estaban curados de las crisis y sin medicación antiepiléptica. Dos de los pacientes están controlados de las crisis con medicación, otro (el adulto) está en espera de decisión quirúrgica y el restante desechó la operación. Conclusión: La DCF tipo Taylor es una patología asociada a una buena parte de las crisis focales fármaco-resistentes, que debe tratarse de identificar y de extirpar lo antes posible ya que la cirugía, bien proyectada y realizada, sin dejar residuos displásicos, puede curarla en un alto porcentaje de casos. Keywords: Balloon cells, Focal cortical dysplasia, Taylor-type focal cortical dysplasia, Epilepsy, Drug-resistant epilepsy, Surgical treatment of epilepsy, Palabras clave: Células balonadas, Displasia cortical focal, Displasia cortical focal tipo Taylor, Epilepsia, Epilepsia fármaco-resistente, Tratamiento quirúrgico de la epilepsia

Objective: Presentation of 8 patients with subependymal giant-cell astrocytomas (SGCA) associated with tuberous sclerosis complex (TSC). Material and methods: There are 8 patients, 6 males and 2 females with TSC, who presented with the tumour between the neonatal period and 24 years. Results: All patients showed bilateral hypersignalised areas in zones close to the foramen of Monro. Three of the patients were admitted urgently due to blindness and increased intracranial pressure. Incomplete removal of the tumour has always been bad solution as it resulted in the death of the patient (in one case) or further surgery operation in the short term. Only one patient developed the tumour suddenly from pre-existing subependymal nodules from the childhood and they had to be removed at 24 years of age. By contrast, 32 patients with TSC and images of subependymal nodules whose CT or MR progress was followed up for between 10 and 30 years did not develop a tumour. One patient had to be operated four times over 20 years. Conclusions: SGCA associated with TSC is a severe complication which as likely to develop and careful monitoring is required from neonatal age with periodic-clinical and imaging studies in order to avoid its irreversible complications. Hydrocephaly, blindness and even the death can be the main consequences. Reintervention of the recurrent tumour is often necessary. Resumen: Objetivo: Presentar 8 pacientes con astrocitomas subependimarios de células gigantes (ASGC) en relación con el complejo de esclerosis tuberosa (CET). Material y métodos: Ocho pacientes, 6 varones y 2 mujeres, con CET, que desarrollaron el tumor entre la etapa neonatal y los 24 años. Resultados: Todos mostraban áreas localizadas bilaterales de hiperseñal, en zonas próximas a los foramina de Monro. Tres ingresaron urgentemente con ceguera e hipertensión intracraneal. La extirpación parcial del tumor fue siempre una mala solución ya que acabó en reintervenciones a corto, medio o largo plazo o en la muerte de un paciente. Sólo en un caso vimos desarrollarse el tumor desde las zonas de hiperseñal subependimaria a partir de la preadolescencia para acabar en extirpación a los 24 años, mientras que 32 pacientes a los que se siguió la evolución de estas zonas de hiperseñal entre 10 y 30 años no desarrollaron tumor. Un paciente tuvo que ser operado cuatro veces a lo largo de 20 años por recidiva del tumor; se extirpó otro ASGC en el lado contralateral al mismo tiempo de la cuarta intervención en el lado del tumor primitivo. Otros 2 pacientes también mostraron recidiva y tuvieron que ser reintervenidos del tumor. Conclusiones: El ASGC en relación con CET es una complicación grave cuya posibilidad de desarrollo hay que controlar cuidadosamente desde la época neonatal, con estudios periódicos clínicos y de imagen, para evitar sus complicaciones irreversibles. La hidrocefalia, la ceguera e incluso la muerte pueden ser sus consecuencias. La reintervención de tumores recidivados a menudo es necesaria. Keywords: Subependymal giant-cell astrocytoma, Tuberous sclerosis complex, Blindness, Headache, Hydrocephalus, Palabras clave: Astrocitoma subependimario de células gigantes, Complejo de esclerosis tuberosa, Ceguera, Cefalea, Hidrocefalia

Resumen Los tumores metastasicos suponen aproximadamente el 15% de los tumores malignos de ovario. Los tumores primarios mas frecuentes derivan del tubo digestivo, la mama y el endometrio. Revisamos los tumores de ovario metastasicos diagnosticados en nuestro servicio de ginecologia en los ultimos 5 anos. La edad media del diagnostico fue de 56,96 anos. Dos terceras partes fueron de origen no ginecologico, y el resto derivaron del aparato genital y la mama. La mitad de las pacientes presentaron un tumor primario gastrointestinal. La mayoria de las metastasis se diagnosticaron en segundo lugar, con un tamano no superior a 5 cm y afectaron a un solo ovario. El componente predominante de las tumoraciones fue solido. La clinica de las pacientes fue desde nula a dolor y crecimiento del perimetro abdominal. El tratamiento en todos los casos fue quirurgico mas quimioterapia y/o radioterapia. En la actualidad una tercera parte de las mujeres ha fallecido.

Summary Carcinoma of the fallopian tube is the least frequent of the gynaecological carcinomas, representing between 0.8 and 1.5% of all of them. Diagnosis is not usually made pre-operatively, but casually during examination of surgical specimens. The average age of the patients is around 60 years. We review the cases of carcinoma of the fallopian tube diagnosed in our Gynaecology Department in the years 1993-2002. The mean age of the patients was 65.5 years. The most frequent symptoms were vaginal bleeding, followed by abdominal mass and pelvic pain. None of the cases was diagnosed pre-operatively, 60% were Stage I, 20% were Stage II, and 20% were Stage III, according to FIGO clssification. Surgery was used in 30% of the cases and 60% received adjuvant chemotherapy with Cisplatin. We collect the clinical symptoms, diagnosis, treatment, and evolution of the patients.

Resumen El diagnostico de mola hidatiforme en pacientes mayores de 50 anos es dificil si no se sospecha clinicamente. Presentamos el caso de una paciente de 54 anos con metrorragias a la que diagnosticamos de mola hidatiforme completa, destacando la importancia de la sospecha clinica que nos llevara a un diagnostico facil y rapido con la ecografia y la determinacion de hCG serica. El diagnostico correcto y tratamiento precoz son fundamentales en estas pacientes para evitar complicaciones.

Objetivo Desarrollar un modelo que facilite la clasificacion de los pacientes con demencia vascular en dos de sus subtipos mas relevantes: demencia multiinfarto (afectacion cortical) y enfermedad de Binswanger (afectacion subcortical). Pacientes y metodos Analisis retrospectivo de 50 pacientes consecutivos con enfermedad de Binswanger y 50 enfermos con demencia multiinfarto estudiados durante un periodo de doce anos (1986–1998) en un hospital universitario de tercer nivel. El modelo estadistico se obtuvo mediante analisis multivariable (modelo logit) y se estimo su exactitud mediante analisis de curvas ROC. Resultados Las variables que integraron el modelo de regresion fueron: hipertension arterial (p Conclusiones A pesar de existir unos criterios diagnosticos de consenso de demencia vascular, su utilizacion muchas veces esta circunscrita al area de investigacion. Hemos desarrollado un modelo de clasificacion de los pacientes con demencia vascular en dos subgrupos relevantes: demencia multiinfarto y enfermedad de Binswanger. Este modelo puede contribuir a facilitar la identificacion de estos pacientes en la practica clinica diaria.

Objetivo Conocer el espectro de enfermedades responsables del sindrome general solitario en nuestro medio. Hemos definido este sindrome como «el cuadro clinico caracterizado por la presencia de astenia, anorexia y adelgazamiento de al menos el 5% del peso corporal en los ultimos 6 meses, no acompanado de ningun sintoma o signo que oriente al diagnostico del padecimiento de una enfermedad propia de un determinado organo o sistema». Pacientes y metodo Todos los pacientes diagnosticados de sindrome general solitario (328) en un hospital universitario de tercer nivel, durante el periodo comprendido entre enero de 1991 a diciembre de 1996. Resultados El 51,8% (170) de los pacientes con sindrome general solitario eran varones y el 48,2% (158) mujeres. La media de la edad fue de 65,4 anos, con unos limites de 15 y 97 anos. La media de la perdida estimada de peso mensual se situo entre los 3 y 4 kg/mes. Se diagnosticaron 115 neoplasias malignas (35%) y 5 tumores benignos (1,5%). Los tumores malignos mas frecuentes fueron los del aparato digestivo (el 51,3% del total de neoplasias malignas). La segunda causa en frecuencia del sindrome general solitario fueron las enfermedades psiquiatricas, con un total de 80 pacientes (24,3%). Se detectaron 116 enfermedades organicas no neoplasicas, representando las enfermedades del aparato digestivo, principalmente la enfermedad peptica, la causa mas frecuente de este grupo. Tras el periodo de seguimiento solamente en 20 casos no se logro poner de manifiesto un cuadro clinico que justificase su causa. De ellos, en 9 el sindrome general solitario fue autolimitado. El 43,9% de los pacientes presentaban al menos otra enfermedad acompanante a la entidad responsable de este sindrome y en el 23,6% de ellos se encontro mas de un proceso asociado. Conclusion Las enfermedades mas frecuentemente responsables del sindrome general solitario fueron, por este orden, las neoplasias malignas, los procesos psiquiatricos y las enfermedades organicas no malignas del aparato digestivo. Quiza un mejor conocimiento del espectro etiologico de dicho sindrome en nuestro medio pueda ayudar a un manejo mas eficiente de estos pacientes.

La metastasis vaginales son un fenomeno raro, pero constituyen el grupo mayoritario de tumores de vagina. La afeccion vaginal de los linfomas no hodgkinianos es muy infrecuente, y tanto en estadios avanzados como en las formas lozalizadas (presumiblemente primarias) la forma clinica de presentacion es la aparicion de un sangrado vaginal, y el hallazgo mas comun es una masa en la vagina. Las formas secundarias de afeccion vaginal se encuentran en edades mas avanzadas y tienen una peor superviviencia que las primarias.

Acute fatty liver of pregnancy is an uncommon entity, which affects women during the last trimester of pregnancy. It is characterized by a prodromic period of symptoms such as uneasiness, nausea, epigastric pain, and jaundice. Analysis shows high levels of bilirubin, transaminase, hypoglycaemia and prolonged clotting time. Treatment is by ending the pregnancy immediately. Hepatic biopsy is useful for atypical cases. The maternal and fetal mortality rate is 20%. We analyze the clinical presentation, analytical parameters, treatment and evolution of this disease with the report of a clinical case in our hospital, as a contribution to the scarcity of available publications.

Resumen La rotura uterina es una de las mayores catastrofesque podemos encontrar en la practica obstetrica,ya que se asocia con una alta mortalidad materna y perinatal. En el utero sin cicatriz es un acontecimientoque ocurre habitualmente intraparto,y con frecuenciatiene consecuencias fatales para la madre y el feto.

Resumen Las metastasis vaginales son un fenomeno raro,pero constituyen el grupo mayoritario de tumores dela vagina. Las metastasis vaginales del adenocarcinoma renal usualmente se presentan como una masa polipoidea unica en el tercio inferior y la pared anteriorde la vagina. El mecanismo que mejor explica estetipo de metastasis se basa en el flujo venoso retrogrado de celulas tumorales desde la vena renal izquierdaa la vena ovarica izquierda,el plexo ovarico y el plexo ureterovaginal. El diagnostico diferencial debe hacerse con el adenocarcinoma primario de celulas claras y el melanoma de vagina,y para ello la inmunohistoquimica es una herramienta muy util.