Background: Giant cell arteritis (GCA) is a heterogeneous disease with diverse clinical presentations and varying degrees of severity. This study aimed to assess the incidence of 3 clinical subsets in GCA and analyze associated severe complications and survival rates. By identifying distinct clinical patterns, the goal is to customize treatment approaches and minimize severe complications during follow-up. Methods: This retrospective study classified clinical manifestations of GCA into 3 major phenotypes based on the reason for consultation: i) cranial, ii) extracranial, and iii) occult GCA. These groups were analyzed and compared for acute complications, including severe ischemic complications, “true” occlusive disease, and late complications such as aortic aneurysm. Survival data were also collected during follow-up. Results: Visual disturbances were more common in the cranial GCA group compared to other subsets (P < .001). Blindness and stroke showed a clinically relevant trend, although statistical differences were not significant between the cranial GCA groups. Limb claudication was significantly more prevalent in the extracranial subset compared to the cranial or occult GCA subsets (12% vs. 2.6% vs. 0% respectively). Severe ischemic complications and true occlusive disease were more frequent in the cranial GCA groups (60%, P=.005 and 40%, P=1.64 respectively). Regarding mortality, there were no statistically significant differences in survival among the different clinical subsets. However, the occult GCA subset showed a trend towards a higher prevalence of deaths, both overall and specifically due to GCA. Conclusion: Clinical subsets in GCA present distinct complications and survival outcomes, with the cranial subset showing a higher incidence of severe ischemic events and the occult subset associated with delayed diagnosis and increased mortality. Recognizing these subsets is crucial for tailored treatment approaches and improving patient prognosis. Further prospective studies are needed to refine diagnostic and therapeutic strategies.

espanolLa arteritis de celulas gigantes (ACG) es la vasculitis sistemica primaria mas frecuente en adultos mayores de 50 anos. Puede presentarse con sintomatologia craneal, afectando la circulacion ocular y cerebral, o extracraneal, con inflamacion de grandes vasos, especialmente la aorta y sus ramas principales. Debido a la gran variabilidad de sintomas y, ocasionalmente, a la escasez de manifestaciones clinicas de la enfermedad, el diagnostico puede ser dificil, pero critico, para prevenir complicaciones isquemicas devastadoras como la ceguera y los accidentes cerebrovasculares. La biopsia de arteria temporal (BAT) sigue siendo el “gold-standard” para el diagnostico de la ACG. Sin embargo, aunque es muy especifico, carece de la sensibilidad adecuada y puede producir resultados falsos negativos en un 39-91%% de los casos. La ecografia de las arterias temporales y de grandes vasos es una modalidad de diagnostico en la ACG que puede identificar la presencia de cambios de la pared de los vasos a lo largo de su longitud. La ecografia es una tecnica no intervencionista, rapida de interpretar y de un coste reducido en comparacion a otras tecnicas. EnglishGiant cell arteritis (GCA) is the most frequent primary systemic vasculitis in adults over 50 years. It may cause cranial vasculitis, affecting vascular territories of the eye or brain or extracranial involvement of large vessels, especially the aorta and its main branches. Due to the great variability of symptoms and, occasionally, due to the scarcity of clinical manifestations of the disease, diagnosis may be difficult, but critical, to prevent devastating ischemic complications such as blindness and stroke. Temporal artery biopsy (BAT) remains the “gold-standard” for the diagnosis of GCA. Although it is very specific, it lacks adequate sensitivity and may have a false negative rate in 39-91%% of cases. Ultrasonography (US) of the temporal artery and large vessels is a diagnostic procedure in GCA able to identify vessel wall changes. US is a non-interventionist technique, quick to interpret and more affordable compared to other techniques. catalaL'arteritis de cel·lules gegants (ACG) es la vasculitis sistemica primaria mes frequent en adults majors de 50 anys. Pot presentar-se amb simptomatologia cranial, afectant la circulacio ocular i cerebral o extracranial amb inflamacio de grans vasos, especialment l'aorta i les seves branques principals. A causa de la gran variabilitat de simptomes i, ocasionalment, a l'escassetat de manifestacions cliniques de la malaltia, el diagnostic pot ser dificil, pero critic, per prevenir complicacions isquemiques devastadores com la ceguesa i els accidents cerebrovasculars. La biopsia d'arteria temporal (BAT) segueix sent el “gold-standard” per al diagnostic de l'ACG, pero, tot i que es molt especific, no te la sensibilitat adequada i pot produir resultats falsos negatius en un 39-91% dels casos. L'ecografia de les arteries temporals i de grans vasos es una modalitat de diagnostic en l'ACG que pot identificar la presencia de canvis de la paret dels vasos al llarg de la seva longitud. L'ecografia es una tecnica no intervencionista, rapida d'interpretar i d'un cost molt mes reduit comparat amb altres tecniques.

Objective. The aim of this study was to quantify the incidence of all clinical fractures, including traumatic and fragility fractures, in patients aged 50 years and older, and to describe their distribution by fracture location, sex and age. Methods. The incidence of clinical fractures at 10 hospitals in Catalonia, with a reference population of 3 155 000 inhabitants, was studied. For 1 week, from 30 May to 5 June 2016, we reviewed the discharge reports of the Traumatology section of the Emergency Department to identify all fractures diagnosed in patients >= 50 years of age. As a validation technique, data collection was carried out for 1 year at one of the centres, from 1 December 2015 to 30 November 2016. The fracture incidence, including the 95% CI, was estimated for the entire sample and grouped by fracture type, location, sex and age. Results. A total of 283 fractures were identified. Seventy per cent were in women, with a mean age of 72 years. The overall fracture incidence was 11.28 per 1000 person-years (95% CI: 11.10, 11.46), with an incidence of traumatic and fragility fractures of 4.15 (95% CI: 4.04, 4.26) and 7.13 per 1000 person-years (95% CI: 6.99, 7.28), respectively. The incidence of fractures observed in the validation sample coincided with that estimated for the whole of Catalonia. The most common fragility fractures were of the hip, forearm, humerus and vertebrae. Conclusion. The results of this study are the first to estimate the incidence of clinical fragility fractures in Spain, grouped by location, age and sex.

BACKGROUND AND OBJECTIVE:Giant cell arteritis (GCA) is the most frequent systemic vasculitis in adults. In recent years, the usefulness of temporal artery ultrasound (TAUS) as a diagnostic tool to assess the underlying inflammation of the vascular wall during the inflammatory process has been under clinical investigation.MATERIAL AND METHODS:Observational and descriptive cohort study of 120 TAUS in 60 patients with clinical suspicions of GCA, according to the ACR (American College of Rheumatology) classification criteria.RESULTS:Among all patients who underwent ultrasound, 42.3% met clinical criteria for GCA according to ACR. Sensitivity and specificity of TAUS in our cohort with clinical suspicion was 81.8% and 93.3%, respectively. A PPV of 90.1% and a VPN of 87.5% were observed.CONCLUSION:Our results showed that TAUS as a useful, indolent, fast, and accessible tool with high diagnostic specificity and diagnostic value.

FRAX is a tool that is available online to calculate the risk of fracture from a number of clinical risk factors, whether bone mineral density is known or not. Due to the quality of the data used in its calculation and the methodology employed, FRAX is now probably the preferred method for determining fracture risk. However, this tool has certain limitations and the physician’s clinical judgment remains critical, especially for the assessment of risk factors not included in the algorithm such as lumbar bone mineral density or frequency of falls. The wide acceptance and use of FRAX could identify people at high risk of osteoporotic fracture, who are suitable for early intervention and are so far overlooked. Equally, FRAX could help to reduce the number of unnecessary treatments in patients with a low fracture risk. Another potential use of FRAX would be its use in identifying patients with indications for bone densitometry. & 2010 SER. Published by Elsevier España, S.L. All rights reserved. Las fracturas osteoporóticas o por fragilidad representan un grave problema de salud por sus consecuencias sobre la morbimortalidad de los pacientes y por los costes económicos que generan. Se estima que, en el mundo, unos 200 millones de adultos tienen osteoporosis y, aunque disponemos de técnicas adecuadas para el diagnóstico y de tratamientos efectivos para disminuir el riesgo de fractura, aún es una enfermedad infradiagnosticada e infratratada. La densidad mineral ósea (DMO) determinada por absorciometrı́a fotónica dual de fuente radiológica se asocia estrechamente a la presentación de fracturas: el riesgo de fractura se dobla por cada disminución de una desviación estándar de la DMO y la población por debajo del umbral de diagnóstico de osteoporosis (T-score r 2,5) representa una población de alto riesgo de fractura. Sin embargo, los estudios epidemiológicos y la experiencia clı́nica ponen de manifiesto que una alta proporción de mujeres que presentan una fractura tienen una DMO en el intervalo de osteopenia o, incluso, normal. Mientras que a los 50 años solo un 5% de las mujeres tiene osteoporosis, el 20% de las fracturas se produce entre los 50–60 años. Aparte de la DMO, otros factores contribuyen al riesgo de fractura, algunos de estos dependientes y otros independientes de la DMO, unos modificables y otros no modificables (tabla 1). La edad es un claro ejemplo de factor independiente de riesgo de fractura (la incidencia anual de fractura de la cadera se multiplica aproximadamente por 30 entre los 50–90 años, mientras que por la ARTICLE IN PRESS www.elsevier.es/semreuma Seminarios de la Fundación Española de Reumatologı́a 1577-3566/$ see front matter & 2010 SER. Publicado por Elsevier España, S.L. Todos los derechos reservados. doi:10.1016/j.semreu.2010.03.001 Autor para correspondencia. Correo electrónico: carmen.gomez@bellvitgehospital.cat (C. Gómez Vaquero). Semin Fund Esp Reumatol. 2010;11(3):100–106

Background and objective: Giant cell arteritis (GCA) is the most frequent systemic vasculitis in adults. In recent years, the usefulness of temporal artery ultrasound (TAUS) as a diagnostic tool to assess the underlying inflammation of the vascular wall during the inflammatory process has been under clinical investigation. Material and methods: Observational and descriptive cohort study of 120 TAUS in 60 patients with clinical suspicions of GCA, according to the ACR (American College of Rheumatology) classification criteria. Results: Among all patients who underwent ultrasound, 42.3% met clinical criteria for GCA according to ACR. Sensitivity and specificity of TAUS in our cohort with clinical suspicion was 81.8% and 93.3%, respectively. A PPV of 90.1% and a VPN of 87.5% were observed. Conclusion: Our results showed that TAUS as a useful, indolent, fast, and accessible tool with high diagnostic specificity and diagnostic value. (C) 2018. Published by Elsevier Espana, S.L.U.

Abhijeet Danve Albert Selva-O’Callaghan Alejandro Escudero Alfred H. J. Kim Amir Sharabi Andreas Goules Angel Luis Guerrero Ayten Yazıcı Babatunde Olaosebikan Basilio Rodriguez-Diez Bernard Ng Beth Wallace Bünyamin Kısacık Carme Moragues Cengiz Korkmaz Cesar Diaz-Torne Chrysanthi Staveri Daniel Roig Vilaseca Roig David Castro Corredor Dimitrios Daoussis Dimitrios Vassilopoulos Elena A. Kovrazhkina Elena Riera Emil Thachil Erkan Alpsoy Estrada Alarcon Paula Valentina Fernando Perez-Ruiz Figen Ayhan Filiz Cebeci Ganesh Dharmshaktu Georgina Salvador Gezmiş Kimyon Gianluigi Bajocchi Gideon Flusser Gözde Yıldırım Çetin Gustavo Balbi Gülcan Güleç Gülşen Akman Demir Hakan Emmungil Haner Direskeneli Hèctor Corominas Hollis Krug James T. Rosenbaum Jennifer Jooha Lee John Case Katherine Wysham Kwi Young Kang Lara Valor M. Sait Dağ Marc C. Genovese Meiko Hashimato Maeda Meltem Koray Michael Horn Miriam Álvarez Álvarez Mónica Fernandez-Castro Orhan Küçükşahin Panagiota Anyfanti Patricia Moya Piero Portincasa Prasandeep Rath Priyankar Pal Ragnar Gunnarson Ramadan Özmanevra Rıdvan Mercan Robert Fox Özgür Kasapçopur Samardeep Gupta Schivanand Gamanagetti Seung-Geun Lee Seung Min Jung Su-Jin Moon Süleyman Serdar Koca Takao Nagashima Tian Wang Timuçin Kaşifoğlu Tomohiro Koga Tsai-Ching Hsu Vera Ortiz-Santamaria Vladimir Ognenovski Weiqian Chen Yair Molad Yehuda Shoenfeld

Background PsA has a higher heritability than PsV, indicating the existence of additional PsA-specific genetic factors. To date, however, the specific genetic basis underlying PsA is poorly understood. Objectives The objective the present study was to identify new genetic variation specifically associated with PsA risk. Methods In order to characterize the genetic basis of PsA, we performed a GWAS meta-analysis at the single-marker level as well as at the pathway level (GWPA). A cohort of 835 PsA patients and 1,558 controls from the Spanish population was genotyped for >550,000 SNPs. GWAS data from a second cohort of 1,430 PsA patients and 1,417 controls from the North American population was also used. In order to confirm the specificity of the new genetic variation associated with PsA risk, we analyzed the association with purely cutaneous psoriasis (PsC, n=614) and rheumatoid arthritis (RA, n=1,191). We performed a pharmacogenetic analysis to investigate the new PsA-specific pathways as a source for drug discovery in PsA. Results GWAS meta-analysis identified a new association between B3GNT2 gene and PsA (P<5e-08). In the GWAS pathway analysis, we identified and validated a total of 14 genetic pathways associated with PsA risk. From these, the glycosaminoglycan (GAG) metabolism pathway was also found to be significantly associated with PsA risk when directly contrasted to the PsC cohort as well as the RA cohort. At the functional level, we detected a significant differential expression of GAG metabolism pathway genes in blood samples from PsA patients compared to PsC patients. The pharmacogenetic analysis identified several FDA-approved drugs likely to modify the GAG pathway. Conclusions The present study represents an important step towards the characterization of the genetic factors specific to PsA risk. Disclosure of Interest None declared

In March 2008, FRAX, developed by Kanis and collaborators in the University of Sheffield and supported by the World Health Organization, became available online to calculate absolute risk of osteoporotic fracture in the next 10 years.

Background Giant cell arteritis (GCA) is the most frequent vasculitis in adulthood. The delay in diagnosis sets back treatment and can lead to serious consequences. Diagnosis is complex, and is followed by the classification criteria according to the American College of Rheumatology (ACR). The is an increasing interest on the utility of temporal artery ultrasound (TAUS) as a tool to evaluate inflammation on the vessel wall. Objectives to evaluate the utility of TAUS in GCA. Methods During 2016, 120 TAUS were carried out in 60 patients with clinical suspicion for GCA. The TAUS was carried to completion by rheumatologist with experience. The symptoms that lead to a TAUS was either one or more of these clinical scenarios: 1) cranial symptoms (recent onset headache, mandibular claudication, visual disturbances) 2) polymyalgic syndrome 3) toxic o febril unspecific syndrome 4) vertebrobasilary (VB) stroke. Demographic and laboratory data were collected, and a follow-up was done to learn the final diagnosis. As for TAUS, the “halo” sign was considered positive if an anechoic image surrounded the vessel was present, and measured >0,30 mm in both, longitudinal and transverse cuts. Other more unspecific signs as stenosis or occlusion were also registered. A temporal artery biopsy was performed whenever the physicians considered necessary, based on clinical criteria, every case in no more than 30 days. Results Fifty-two percent were women, mean age 76±7.8 years old. Mean laboratory parameters: eritrosedimentation rate 85±41.9 mm/h, C-reactive protein 77±80 mg/L, Haemoglobin 11.4±2.2 g/L, white blood count 10,228±3,520, platelet count 310,603±123,918. The symptoms that motivated requesting the TAUS were: cranial symptoms (62.2%), toxic, unspecific, febrile syndrome (44%), polymyalgic syndrome (30%), VB stroke (5%). A temporal artery biopsy was carried out in 45% of patients (N=27); it was positive in 40.7%, negative in 40.7% and unspecific (given it reported an inflammatory histologic pattern, but without the characteristic giant cells) in 18.5%. From all 60 patients in whom a TAUS was performed, 36% were diagnosed with GCA, based on ACR criteria. The sensibility and specificity for TAUS was 80% and 94% respectively, with a posivite predictive value of 88.9% and a negative predictive value of 89.2% Conclusions TAUS is a useful, non-invasive, fast, accessible tool for evaluating temporal arteries with a great diagnostic valu Disclosure of Interest None declared

Corominas, Hèctor MD, PhD; Villareal, Johan MD; Estrada, Paula MD; Roig-Vilaseca, Daniel MD, PhD; Torrente-Segarra, Vicenç MD; Reina, Delia MD; Cerdà-Gabaroi, Dacia MD, PhD; García-Diaz, Silvia SRN; Vidal, David MD, PhD Author Information

Primary Sjögren syndrome (PSS) is a chronic inflammatory autoimmune disease. Interstitial lung disease (ILD) can be an extraglandular complication.

OBJECTIVE:Diagnosis and therapy of patients with early onset rheumatoid arthritis (RA) is influenced by accessibility to specialized care devices. We attempted to analyze the impact of their availability. METHODS:We analyzed time related to diagnosis delay measuring: 1) Time from first clinical symptoms to the first visit with the Rheumatologist; 2) Time from referral to the first visit of Rheumatology; 3) Time between first symptom until final diagnosis; 4) time between first symptom until the initiation of the first disease-modifying antirheumatic drug (DMARD). The presence of these 6 rheumatology devices was defined: 1) early arthritis monographic clinics, 2) RA monographic clinics, 3) Mechanisms for fast programming, 4) Algorithms for referral from primary care (PC), 5) rheumatology consultation services in PC and 6) consulting services in PC. RESULTS:The mean time from onset of symptoms to diagnosis or the establishment of a DMARD in RA patients in Catalonia is very long (11 months). Patients seen in rheumatology devices such as RA monographic clinics, rheumatology consultation in PC and specially in early arthritis clinics are treated early with DMARDs. CONCLUSION:the existence of monographic clinics or consulting in primary care centers is essential to improve early care of RA patients.

Background Copy-Number Variants (CNV) have been associated to the risk to develop multiple autoimmune diseases. Objectives Our objective was to identify CNVs associated with the risk to develop Psoriatic Arthritis (PsA) using a genome-wide analysis approach. Methods A total of 835 PsA patients and 1,498 controls were genotyped for CNVs using the Illumina HumanHap610 BeadChip. The most significant CNV associations with PsA risk were independently tested in a sample of 1,133 PsA patients and 1,831 controls. In order to test for the specificity of the variants with PsA etiology, we also analyzed the association to a cohort of 822 purely cutaneous psoriasis (PsC) patients. Results A total of 165 common CNVs were identified in the GWAS. We found a highly significant association of an intergenic deletion between ADAMTS9 and MAGI1 genes (p=0.00014). Using the independent patient and control cohort we validated the association between ADAMTS9-MAGI1 deletion and PsA risk (p=0.032). Using next-gen sequencing we characterized the associated deletion. Finally, analyzing the PsC cohort we found a lower frequency of the deletion compared to PsA (p=0.0088) and similar to that of controls (p>0.3). Conclusions The GWAS for CNVs has identified a new deletion associated with PsA risk and which is also differential from PsC risk. Disclosure of Interest None declared