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Objective To determine the current state of Rheumatology in Catalonia and to update the information regarding previous studies. Material and methods Design Observational, descriptive and cross-sectional study. Data collection from January to June 2017. Sample Rheumatologists practising public healthcare activity in Catalonia. An online questionnaire was sent to gather individual data and data from rheumatology services/sections. Results Information was obtained on 109 rheumatologists: 39 men and 70 women; mean age: 47 ± 9 years. The number of rheumatologists has increased by 8% over the past 5 years. One hundred and one (92.7%) doctors qualified as rheumatologists through the MIR. Rheumatology practice was mostly in a hospital setting: 68 (62.4%) physicians. Ninety-six (88.1%) rheumatologists were full-time practitioners. Fifty-four (50%) rheumatologists also practiced in the private sector. Clinical practice was predominant: 76% of daily time was devoted to this area. Of note, it was found that most of the rheumatology services, 24 (705), were dependent hierarchically on other services, namely internal medicine and orthopaedic surgery. There are still 6 hospitals in Catalonia without a rheumatologist. Conclusions The number of rheumatologists in the public health sector of Catalonia has increased over the past 5 years. The mean age of rheumatologists is advanced and there is a clear predominance of female practitioners in the specialty. Many rheumatology services depend on other services. Rheumatology activity is primarily focussed in the city of Barcelona.

Resumen Objetivo Determinar el estado actual de la reumatologia en Cataluna, y actualizar la informacion respecto a los estudios previos. Material y metodos Diseno Estudio observacional, descriptivo y transversal. Recogida de datos de enero a junio de 2017. Muestra Reumatologos que ejercen actividad asistencial publica en Cataluna. Se envio un cuestionario en linea recabando informacion tanto de forma individual como colectiva de los servicios de reumatologia. Resultados Se obtuvo informacion de 34 hospitales y de 109 reumatologos: 39 varones y 70 mujeres; edad media: 47 ± 9 anos. El numero de reumatologos aumento un 8% en los ultimos 5 anos. La obtencion del titulo fue en 101 (92,7%) casos por via MIR. Noventa y seis (88,1%) facultativos tenian dedicacion completa. Un 50% de los reumatologos ejercian ademas en el sector privado. La actividad asistencial suponia un 76% de la jornada laboral. Se constato que 24 (70%) servicios de reumatologia dependian jerarquicamente de otra especialidad. En 6 hospitales de Cataluna no disponen de reumatologo. Conclusiones El numero de reumatologos aumento respecto hace 5 anos. La edad media de los reumatologos es avanzada y existe un claro predominio femenino en la especialidad. Un gran numero de unidades de reumatologia dependen de otros servicios. La actividad reumatologica se centra en Barcelona.

Hintergrund und ziele Die kutane Polyarteriitis nodosa (CPAN) ist eine relativ seltene Vaskulitis, die kleine Arterien und Arteriolen im Unterhautfettgewebe und in der dermo-subkutanen Ubergangszone befallt. Uber ihren Verlauf liegen fur die europaische Bevolkerung nur wenige Daten vor. Ziel dieser Studie ist es, Manifestationen und Prognoseparameter fur ein Rezidiv bei CPAN zu beschreiben. Patienten und methoden Wir stellen eine retrospektive Studie an Patienten mit klinischer und histologischer Evidenz fur CPAN vor, die zwischen 1989 und 2019 in zwei tertiaren Referenzzentren in Spanien behandelt wurden. Ergebnisse Eingeschlossen waren 31 Patienten. Die haufigste Manifestation waren subkutane Knoten (90,3 %). Zum Zeitpunkt der Diagnose traten haufig Ulzera auf (35,5 %). Bei zwei Dritteln der Patienten lag mindestens eine extrakutane Manifestation vor. Siebzehn Patienten (54,8 %) erlitten ein Rezidiv. Der starkste Pradiktor fur ein Rezidiv waren Ulzerationen in der ersten Episode (OR 18,6; 95%-KI 2,73-38; p Schlussfolgerung CPAN ist eine auf die Haut begrenzte Vaskulitis. Jedoch konnen die Symptome auf benachbarte Skelettmuskeln oder periphere Nerven ubergreifen. Die Krankheit ist nicht lebensbedrohlich, jedoch sind Ulzerationen und der Anstieg bestimmter Laborparameter mit einer schlechten Prognose assoziiert.

Elderly-onset rheumatoid arthritis (EORA) and polymyalgia rheumatica (PMR) are common rheumatic diseases in older adults. Oxylipins are bioactive lipids derived from omega-6 (n-6) and omega-3 (n-3) polyunsaturated fatty acids (PUFAs) that serve as activators or suppressors of systemic inflammation. We hypothesized that arthritis symptoms in older adults were related to oxylipin-related perturbations. Arthritis in older adults (ARTIEL) is an observational prospective cohort with 64 patients older than 60 years of age with newly diagnosed arthritis. Patients’ blood samples at baseline and 3 months posttreatment were compared with 18 controls. A thorough clinical examination was conducted. Serum oxylipins were determined by mass spectrometry. Data processing and statistical analysis were performed in R. Forty-four patients were diagnosed with EORA and 20 with PMR. At diagnosis, EORA patients had a mean DAS28CRP (Disease Activity Score 28 using C-reactive protein) of 5.77 (SD 1.02). One hundred percent of PMR patients reported shoulder pain and 90% reported pelvic pain. Several n-6- and n-3-derived oxylipin species were significantly different between controls and arthritis patients. The ratio of n-3/n-6 PUFA was significantly downregulated in EORA but not in PMR patients as compared to controls. The top two candidates as biomarkers for differentiating PMR from EORA were 4-HDoHE, a hydroxydocosahexaenoic acid, and 8,15-dihydroxy-eicosatrienoic acid (8,15-diHETE). The levels of n-3-derived anti-inflammatory species increased in EORA after treatment. These results suggest that certain oxylipins may be key effectors in arthrtis in older adults and that the imbalance between n-6- and n-3-derived oxylipins might be related to pathobiology in this population.

To study retention of biologic disease-modifying anti-rheumatic drugs (bDMARDs) or apremilast and potential predictors of lack of response in patients with psoriatic arthritis (PsA). A single-center retrospective analysis of PsA patients who received ≥ 1 bDMARD or apremilast during 2000–2018. The main endpoint was lack of response (primary or secondary failure). Analyses included retention of DMARDs (Kaplan–Meier curves) and potential predictors of lack of response (bivariate and multivariate logistic regression models). A total of 159 patients with PsA received up to 8 DMARDs: etanercept (34%), adalimumab (30%), infliximab (9%), golimumab (9%), apremilast (7%), ustekinumab (5%), certolizumab (4%), and secukinumab (2%). Therapy was discontinued in 96 cases (60%), mainly owing to secondary failure (37%), followed by primary failure (25%) and adverse effects (24%). Retention was analyzed based on 313 units of analysis. Duration of follow-up was 846.1 treatment-years (maximum 14.8 years, median 2.75 years). A total of 172 DMARDs were discontinued. The probability of continuing the initial treatment was 37% at 5 years, 22% at 10 years, and 12% at 14 years. The longest medium retention time was observed for infliximab (6.2 years) and etanercept (4.5 years). Predictors of lack of response included male sex, number of swollen joints, and, especially, depression (OR = 35.2). The sensitivity and specificity of the model were 86.4% and 85.7%, respectively, with a coefficient of determination (R2) of 45.6 (ROC, 0.912). Rates of discontinuation due to primary and secondary failure are high in PsA. Retention is better for anti-TNF agents than for other agents.

Objective Septic arthritis is a medical emergency and crystal-induced arthritis is a risk factor for its development. If both occur simultaneously, crystal-induced arthritis may mask the diagnosis of infection and delay antibiotic therapy. Method Retrospective analysis of patients with coexistence of septic and crystal-induced arthritis. We included only patients with isolation of crystals in synovial fluid analysis and positive culture of synovial fluid and/or blood culture. Results A total of 25 patients (17 men and 8 women) with a mean age of 67 years. The most commonly affected joint was the knee. In synovial fluid cytological studies, the most frequently identified crystals were monosodium urate. Risk factors included diabetes and chronic renal failure. The most frequently isolated germs were methicillin-sensitive Staphylococcus aureus (48%), methicillin-resistant S. aureus (12%) and Mycobacterium tuberculosis (12%). In all, 36% of subjects required surgical drainage (excluding those caused by M. tuberculosis). Clinical outcome was favourable in 56%, although intercurrent complications were usual (40%). Mortality was 8%. Conclusions Coexistence of septic and crystal-induced arthritis represents a diagnostic challenge and requires a high index of suspicion. Gout was the most prevalent crystal-induced arthritis. S. aureus was the most commonly causative pathogen, with a high rate of methicillin-resistant S. aureus infection. If treated early, the outcome is usually favourable, making synovial fluid microbiological study imperative.

Resumen Objetivo La artritis septica es una urgencia medica y la artritis microcristalina es un factor de riesgo para su aparicion. Si ambas cursan de forma simultanea, la identificacion de microcristales puede enmascarar el diagnostico de la infeccion y causar un retraso en la instauracion del tratamiento antibiotico. Metodo Analisis retrospectivo de pacientes con coexistencia de artritis septica y microcristalina. Se incluye unicamente a los enfermos con aislamiento del germen en liquido articular y/o hemocultivo e identificacion de cristales en el liquido articular. Resultados Se identificaron un total de 25 pacientes (17 varones y 8 mujeres) con una media de edad de 67 anos. La articulacion que se afecto con mayor frecuencia fue la rodilla. Los cristales de urato monosodico fueron los que con mayor frecuencia se identificaron en el estudio citologico del liquido sinovial. Los factores de riesgo mas frecuentes fueron la diabetes mellitus y la insuficiencia renal cronica. El germen aislado con mayor frecuencia fue el Staphylococcus aureus sensible a meticilina (48%), seguido del Staphylococcus aureus resistente a meticilina (12%) y Mycobacterium tuberculosis (12%). El 36% de los pacientes precisaron desbridamiento quirurgico (excluyendo los causados por M. tuberculosis). La evolucion fue favorable en el 56% de los pacientes, aunque la presencia de complicaciones intercurrentes fue habitual (40%). La mortalidad fue del 8%. Conclusiones La coexistencia de artritis septica y microcristalina representa un reto diagnostico y requiere un alto indice de sospecha. La artropatia por cristales de urato monosodico es la mas prevalente y S. aureus el germen causal mas frecuente, con una tasa elevada de infeccion por S. aureus resistente a meticilina. Si se instaura de forma precoz el tratamiento adecuado, la evolucion suele ser favorable, por lo que el estudio microbiologico del liquido sinovial es imperativo. Objective Septic arthritis is a medical emergency and crystal-induced arthritis is a risk factor for its development. If both occur simultaneously, crystal-induced arthritis may mask the diagnosis of infection and delay antibiotic therapy. Method Retrospective analysis of patients with coexistence of septic and crystal-induced arthritis. We included only patients with isolation of crystals in synovial fluid analysis and positive culture of synovial fluid and/or blood culture. Results A total of 25 patients (17 men and 8 women) with a mean age of 67 years. The most commonly affected joint was the knee. In synovial fluid cytological studies, the most frequently identified crystals were monosodium urate. Risk factors included diabetes and chronic renal failure. The most frequently isolated germs were methicillin-sensitive S. aureus (48%), methicillin-resistant S. aureus (12%) and Mycobacterium tuberculosis (12%). In all, 36% of subjects required surgical drainage (excluding those caused by M. tuberculosis). Clinical outcome was favorable in 56%, although intercurrent complications were usual (40%). Mortality was 8%. Conclusions Coexistence of septic and crystal-induced arthritis represents a diagnostic challenge and requires a high index of suspicion. Gout was the most prevalent crystal-induced arthritis. S. aureus was the most commonly causative pathogen, with a high rate of methicillin-resistant S. aureus infection. If treated early, the outcome is usually favorable, making synovial fluid microbiological study imperative.

Objectives Recent data suggest that some adult patients with autoimmune rheumatic diseases may develop cardiac conduction and repolarization abnormalities mediated by anti-Ro/SSA antibodies. We aim to investigate the utility of a cardiac screening in patients with systemic lupus erythematous (SLE) and anti-Ro/SSA positivity. Methods SLE patients who consecutively attended a Rheumatology clinic during 1 year where evaluated for the presence and levels of anti-Ro/SSA antibodies, and clinical and biological markers of organ damage and disease activity. All participants underwent a cardiovascular anamnesis and physical examination, ECG, echocardiography, and 24-hour Holter. Results Of the 145 recruited patients, 49 (32%) had anti-Ro/SSA positivity. None had any degree of atrioventricular block in the ECG or Holter monitoring. No significant differences were observed between anti-Ro/SSA–positive vs. negative patients in terms of PR, QRS or QTc intervals. No clinically significant arrhythmias were recorded during Holter monitoring and no differences in average heart rate, heart rate variability, or atrial or ventricular ectopy burden were observed. Finally, no differences were found in echocardiographic measurements. Conclusions In this study of SLE patients, anti-Ro/SSA positivity was not associated with significant alterations in ECG, echocardiography, or 24-hour Holter. These findings do not support ordinary cardiac evaluation in these patients. ( Clinicaltrials.gov registration number: NCT02162992).

BACKGROUND AND OBJECTIVES Cutaneous polyarteritis nodosa (CPAN) is a comparatively rare form of vasculitis that affects small arteries and arterioles in the panniculus and dermo-subcutaneous junction. Limited information is available regarding its course in the European population. The aim of this study is to characterize the manifestations and prognostic markers of recurrence in CPAN. PATIENTS AND METHODS We report a retrospective study of patients with clinical and histopathologic evidence of CPAN, which was treated at two tertiary referral centers in Spain between 1989 and 2019. RESULTS 31 patients were included. The most frequent manifestation was subcutaneous nodules (90.3 %); ulcers were frequent at diagnosis (35.5 %). Two thirds of the patients had at least one extracutaneous manifestation. Seventeen patients (54.8 %) experienced relapse. The strongest predictor of recurrence was ulceration in the initial episode (OR 18.6; 95 % CI 2.73-38; p

We report a case of acute-onset multifocal vertebral osteitis with a marked impact on the patient's general health. The radiological, scintigraphic and magnetic resonance findings made it necessary to carry out a differential diagnosis to distinguish it from an infiltrative neoplastic process and determine whether it had an infectious or an inflammatory etiology. The presence of noninfectious multifocal osteitis and sternoclavicular arthritis and the subsequent development of plantar pustulosis pointed to SAPHO syndrome. Treatment with infliximab led to improvement in the clinical symptoms, laboratory values and radiological abnormalities.

We report a case of acute-onset multifocal vertebral osteitis with a marked impact on the patient's general health. The radiological, scintigraphic and magnetic resonance findings made it necessary to carry out a differential diagnosis to distinguish it from an infiltrative neoplastic process and determine whether it had an infectious or an inflammatory etiology. The presence of noninfectious multifocal osteitis and sternoclavicular arthritis and the subsequent development of plantar pustulosis pointed to SAPHO syndrome. Treatment with infliximab led to improvement in the clinical symptoms, laboratory values and radiological abnormalities.

Background:SAPHO syndrome (synovitis, acne, pustulosis, hyperostosis and osteitis) is an acronym created with the aim of encompassing within the same entity the findings present in a heterogeneous group of patients with various osteoarticular and cutaneous disorders. For years it has been debated whether it is an entity itself or if, on the contrary, it is a specific phenotype of psoriatic arthritis.Objectives:Determine the relationship between SAPHO syndrome and psoriatic arthritis by comparing the classic criteria of SAPHO with CASPAR in patients previously diagnosed with SAPHO.Methods:A retrospective study where patients with a diagnosis of SAPHO in the same center (1984-2018) were reviewed. Of a total of 95 patients that met the criteria of Benhamou et al., 46 were excluded due to lack of information to complete the minimum necessary data, so 39 patients were finally included in the study. Demographic data were registered, age at diagnosis, CASPAR criteria (active psoriasis, history of own or familial psoriasis, nail psoriasis, negative RF, dactylitis and new formation/juxta-articular bone proliferation), classical criteria of SAPHO and HLAB27. For the statistical analysis, a Chi-square is applied to determine the differences between the groups with / without CASPAR criteria.Results:Of the sample of 39 subjects diagnosed with SAPHO, 15 patients (38%) met CASPAR criteria (4M/11W), with a median age at diagnosis of 42 years (range 21-50). Of them, 8 (60%) had active cutaneous psoriasis, 10 (73%) had a family or personal history of psoriasis, 6 (40%) had psoriatic nail dystrophy, 14 (93%) had negative FR, 3 (20%) had presented some episode of dactylitis and 10 (73%) had juxta-articular new bone formation. Of the 15 patients who met CASPAR criteria, 9 (67%) had synovitis, 2 (13%) acne, 4 (26%) pustulosis, 13 (87%) hyperostosis and 12 (80%) osteitis. HLAB27 was positive in 1 patient (2.5%) of the group that met CASPAR criteria. Of the patients who did not fulfill CASPAR criteria (9M/15W), the median age at diagnosis was 44.5 years (range 10-70). None of them had active cutaneous psoriasis, psoriatic nail distrophy or dactylitis, 1 (4%) had a family or personal history of psoriasis, all had RF- and 3 (12.5%) had juxta-articular bone new formation (Table 1). Synovitis was observed more frequently in patients who met CASPAR criteria than in those who did not (67% vs 25%, p 0.01). In contrast, osteitis was present more frequently in patients who did not meet CASPAR criteria (80% vs 96% p 0.05) and pustulosis, although it was not statistically significant (50% vs 26%, p 0.07). Among patients who did not meet CASPAR criteria only 1 met the 5 classic SAPHO criteria and another 3 met 4.Table 1.Conclusion:Approximately one third (38%) of patients diagnosed with SAPHO meet criteria for psoriatic arthritis, the most notable variables being active psoriasis or a history of psoriasis. Synovitis manifests more frequently in patients with CASPAR criteria and osteitis more present in patients who did not meet them.Acknowledgments:Rheumatology Service of the Germans Trias i Pujol HospitalDisclosure of Interests:None declared

Background Myositis specific antibodies have gained special importance in last years. Its knowledge has allowed stratifying patients in different clinical phenotypes, predicting with greater accuracy prognosis and establishing a clinical attitude to follow. SAE1/2 antibody (anti-small ubiquitin-like modifier activating enzyme) was first described in 2007 in patients with amyopathic dermatomyositis with cutaneous and digestive involvement. Its prevalence ranges from 8% in European cohorts to 3% in asians. Objectives Describing myositis specific antibodies (MSA) in a cohort of inflammatory myopathies. To characterize the clinical phenotype of SAE1/2 antibody and to compare it with the rest of MSA. Methods Patients diagnosed of dermatomyositis in a tertiary hospital from 1978-2018, according to the criteria of Bohan and Peter (1975) and according to Dalakas classification criteria (2015). Clinical and analytical data, including the immunological profile were collected, as well as the treatments received and the evolution of the disease. Results Out of 46, 41 dermatomyositis had positive antinuclear antibodies (ANA). 55% percent had aNA titles≥640, being the fine speckled pattern the most frequent (43%) followed by coarse speckled and homogeneous (13.6% each). 72% had MSA, the most frequent being antiJo1 (27.3%) followed by MDA5 (18.2%) and SAE1/2 (15%). Up to 40% had two or more antibodies, being the association with antibodies Ro52 and Ro60, the most frequent. 5 patients presented positivity against SAE antibody. In comparison to the rest of MSA, 80% presented with cutaneous debut (p = 0.00), being the most frequent manifestations heliotrope erythema (p = 0.00), Gottron papules (p = 0.10) and skin rash (p = 0.00). 60% had pathological capillaroscopy compared to 15% (p = 0.00). Muscular balance was preserved in 60% of patients. Sixty percent had dysphagia vs 9% (p = 0.00). Two of them had lung involvement (alveolar hemorrhage and rapidly progressive pneumonitis). In comparison with SAE negative group, patients presented more pulmonary hypertension (44.5vs34 mmHg), without reaching significant differences. No significant differences were found between muscle enzyme levels’ neither acute phase reactants. As complications, one patient presented a myocarditis with quickly rapidly progressive pneumonitis that required high doses of corticotherapy and another one alveolar hemorrhage, which was treated the same way. All patients required corticotherapy at doses of mg/kg, requiring two of them to be treated with DMARDs (methotrexate and dolquine) and one of them with immunoglobulins due to cutaneous involvement. Mortality rate was 40% due to rapidly progressive lung affectation and cardiorespiratory arrest. Conclusion Higher prevalence of SAE1/2 antibody in our cohort may be explained due to the use of amplified myositis antibodies kit that specifically includes this antibody. These patients typically present with cutaneous involvement and dysphagia but also lung affectation as a complication. Studies with larger samples should be performed in order to know the prognosis of this antibody specificity. Disclosure of interests None declared

Background: Rheumatoid arthritis (RA) patients have a higher insulin resistance (IR) and some studies report that it is present at diagnosis. Systemic inflammation has been pointed out as the reason. However, data on polymyalgia rheumatica (PMR) are controversial. Objectives: To analyse IR in a group of untreated patients with a recent diagnosis of RA and PMR, and to establish predictive factors related with IR. Methods: Longitudinal observational study of patients older than 60 years, newly diagnosed with elderly-onset AR (ACR/EULAR 2010) and PMR (ACR/EULAR 2012). Comparison with healthy control group of the same age. Inclusion: consecutive and voluntary. Exclusion: patients with insulin-dependent diabetes. Follow-up time: 12 months. The clinical-epidemiological, anthropometric and analytical characteristics were collected. IR was calculated by HOMA-IR [(homeostatic model assessment of insulin resistance) = glucose (mg/dL) * insulin (mUI/L)/405] baseline and at 12 months. HOMA-IR>2.75 was considered IR (according to Spanish data). The statistical study was performed with Stata 15.1. Results: We recruited 42 patients with RA, 18 with PMR and 18 healthy controls. None of them had received treatment with corticosteroids or with DMARD at the baseline visit. Baseline characteristics are summarized in the table. At baseline visit, 66.7% of patients with elderly-onset RA had IR, compared with 33.3% of controls (p=0.024) and 27.8% of PMR (p=0.006). Therefore, the prevalence of IR in patients with RA doubled that of controls and patients with PMR before starting treatment. After 12 months of evolution and treatment, patients with RA and IR decreased from 66.7% to 51.2%, not being statistically significant (p=0.179). On the other hand, the percentage of patients with PMR and IR remained the same (27.8%). The differences in IR between RA and PMR at 12 months remained statistically significant (p=0.048). Given the results we decided to analyze the predictive factors related with IR only in the 42 patients with RA. In the univariate logistic regression analysis, the predictors of presenting IR were the BMI, the abdominal perimeter and the scapular girdle involvement. Specifically for BMI, for each of 2 kg/m2 the probability of having IR was 1.24 times higher (OR=1.24, IC95%: 1.12-1.37). Patients with scapular girdle involvement had a 6-fold increased risk of developing IR (OR = 6.0, 95% CI: 1.3-26.6). And for every 5 centimetres of abdominal perimeter the risk increased almost 4 times more (OR=3.9, 95% CI: 2.9-5.1). In the multivariate analysis, the only independent factor to increase the IR was the abdominal perimeter (aOR = 1.23, IC95%: 1.07-1.41). Conclusion: Patients with elderly-onset RA have a higher IR than the general population. High IR in RA is present at diagnosis. IR in AR is not exclusively mediated by systemic inflammation, since patients with PMR do not have this increase. The predictors of presenting IR in late onset AR were BMI, scapular girdle involvement and abdominal perimeter. Only the abdominal perimeter was shown as an independent factor. Disclosure of Interests: None declared

Background: Adult-onset Still’s disease (AOSD) is an uncommon disease with an unpredictable clinical course and variable prognosis. Sometimes, it requires biologic treatment in early phases. A prognosis score has been described, which has never been applied in a Spanish case series. Objectives: To apply the prognosis score described by Pouchot et al (Systemic Score System (SSS)) on a 64 cases series diagnosed with AOSD in Spanish population and to determine if SSS high values registered at the onset of the pathology are related to AOSD clinical patterns (monocyclic, polycyclic and chronic course), requirement of biologic treatment along the disease’s course and development of AOSD clinical complications. To establish the relationship between its value and the AOSD-related mortality. Methods: Retrospective study realized in two University Hospitals. Clinical, laboratory, AOSD-related complications data, administered biologic treatments and number of deaths (AOSD related or not) were recorded. Each patient was characterized for the presence of AOSD-related complications such as macrophage activation syndrome (MAS), myocarditis, lung involvement (pulmonary hypertension, interstitial infiltrate), renal involvement (tubulointerstitial nephritis, acute renal failure), secondary amyloidosis and AOSD-related death. SSS was applied at the onset of the disease development, assigning a point to each of the next 12 variables: fever, exanthema, pleuritis, pneumonitis, pericarditis, alteration of liver tests or hepatomegaly, splenomegaly, lymphadenopathy, odynophagia, leukocytosis >15,000/mm3, myalgia and abdominal pain. A ≥7 score has been validated on other populations as the one which identifies the patients with high risk of complications. The relationship between SSS value and the next parameters was determined: clinical course, complications, biologic treatments administered and AOSD-related mortality. Results: Data from 64 patients was analyzed (40,6% men, mean age 37 years). SSS values of Conclusion: The prognosis score described by Pouchot et al could be useful to identify those patients with high risk of developing clinical complications and those who will need biologic treatment along the course of their disease. It is necessary a higher number of patients to determine if the score could be useful to estimate the death risk related to AOSD complications. References: [1] Ruscitti, et al. Adult-onset Still’s disease: evaluation of prognostic tools and validation of the systemic score by analysis of 100 cases from three centers. BMC Medicine (2016)14:194 Disclosure of Interests: Ivette Casafont-Sole: None declared, Susana Holgado: None declared, J. Narvaez Consultant for: Bristol-Myers Squibb, Maribel Mora: None declared, Josep Roca: None declared, Anahy Brandy-Garcia: None declared, Lourdes Mateo: None declared, Melania Martinez-Morillo: None declared, Laia Gifre: None declared, Maria Aparicio Espinar: None declared, Agueda Prior-Espanol: None declared, Anne Riveros: None declared, Clara Sanguesa: None declared, Jordi Camins-Fabregas: None declared, Annika Nack: None declared, Joan Miquel Nolla: None declared, Alejandro Olive: None declared

Background Polyarthritis at the elderly people usually has a similar onset with an acute inflammatory character and scapular girdle involvement. Differentiating elderly onset rheumatoid arthritis (EORA) and polymyalgia rheumatica (PMR) can be a diagnostic challenge. Objectives To analyse the clinical and analytical differences between EORA and PMR. Methods Longitudinal observational study of patients older than 60 years newly diagnosed with EORA (ACR/EULAR 2010) and PMR (ACR/EULAR 2012). Inclusion: consecutive and voluntary. Follow-up time: 12 months. A single rheumatologist made all follow-up visits. The clinical-epidemiological and analytical characteristics were collected. The statistical study was performed with Stata 15.1. Results 45 EORA were recruited (53% women; mean age 74.8 ± 7.5) and 20 PRM (85% women; mean age 76.6±5.0). 75% of EORA had scapular girdle involvement, but only 44% of the pelvic girdle. All had peripheral arthritis, and the small joints of the hands were involved in 93.3%, with edema in 46.7%. Forty percent of EORA patients were seropositive (RF> 20 IU/mL and/or ACPA> 20 U/mL): 33% RF positive (132.8 ± 126 IU/mL), 28.9% for ACPA (2 cases [15.4%] from 100 to 250 U/mL and 11 cases [84.6%] above 250 U/mL) and in 10 patients [22.2%] double positive). All patients with PMR patients had shoulder girdle involvement and 90% of the pelvic girdle. None of them had peripheral arthritis. RF was positive in one patient (73 IU/mL) and ACPA in 2 patients (titers between 20-40 U/mL). No patient was double positive. Table 1 and 2. After 12 months of follow-up, there was no difference between the dose of glucocorticoids received in patients with EORA and PMR (p = 0.684). There were also no differences in glucocorticoids adverse effects according to the diagnosis (p = 0.734). Regarding the use of immunosuppressors, this was higher in patients with EORA (91% EORA and 20% PMR), according to the usual clinical practice guidelines. The percentage of remission in PMR at 12 months was 95%. However, using DAS 28-VSG, only 40.9% of patients with EORA were in remission at 12 months (p=0.003). Conclusion The female predominance was higher in PMR than in EORA. The scapular girdle involvement, but especially the pelvic girdle, was more frequent in PMR. In contrast, involvement of peripheral joints and edema were more frequent in EORA. RF and ACPA were more frequent in EORA. There were no other analytical differences that would help their differential diagnosis. The mean and accumulated doses of glucocorticoids during the first 12 months were similar, as well as the percentage of side effects. Immunosuppressors are more frequently used in EORA than in PMR. Remission is achieved more commonly in PMR than in EORA. References [1] Olivieri I, Pipitone N, D’ Angelo S, Padula A, Salvarani C. Late-onset rheumatoid arthritis and late-onset spondyloarthritis. Clin Exp Rheumatol 2009;27(4 Suppl 55):S139-145. [2] Pease CT, Haugeberg G, Montague B, Hensor EMA, Bhakta BB, Thomson W, et al. Polymyalgia rheumatica can be distinguished from late onset rheumatoid arthritis at baseline: results of a 5-yr prospective study. Rheumatology 2009;48(2):123-7. Disclosure of Interests None declared

Background Denosumab (Dmab), an anti-receptor activator of nuclear factor kappa-B ligand (RANKL) monoclonal antibody, has been shown to increase bone mineral density (BMD) at lumbar spine and proximal femur up to 21.6% and 9.1% respectively at 10 years of treatment. Additionally, Dmab has shown a marked decrease of vertebral, nonvertebral and femoral fractures during treatment. Nowadays, the existence of inadequate response to Dmab treatment remains unknown. Objectives: to describe the clinical, analytical and densitometric characteristics of patients with an inadequate response (IR) to Dmab treatment. IR was defined as the presence of a new fragility fracture during Dmab treatment or a significant decrease in BMD (≥5% at lumbar spine or ≥4% at proximal femur) within at least 12 months of therapy. Methods: retrospective study including patients with osteoporosis with an IR to Dmab. Therapeutic compliance was checked by clinical anamnesis and the electronic prescription. Risk factors for osteoporosis, history of fragility fractures, previous anti-osteoporotic treatment, densitometric and analytical data were collected before and at the moment when IR was diagnosed. Results Fourteen patients were included (12 women and 2 men) with mean age of 75 ± 9 years. The causes of osteoporosis were: postmenopausal (n=8, 57.14%), induced by glucocorticoids (n=3, 21.43%), alcoholic (n = 1, 7.14%) and multifactorial (n=2; 14.28%). Nine patients (64.28%) had been previously treated with oral or intravenous bisphosphonates for a mean of 5.8 ± 2.76 years. Nine patients (64.28%) had previous vertebral fractures (median 2, range 1-8), 2 of them had also presented a femoral fracture. During Dmab treatment, 7 patients (50%) presented a decrease in BMD (mean loss: proximal femur -3.5%, p=0.09; lumbar spine - 5.8%, p=0.046;) and 7 had incidental fractures: 5 vertebral (median 1, range 1-4), 1 humerus and 1 femur. The duration of treatment with Dmab was 3.82 ± 1.85 years in patients who sustained fragility fractures and 2.39 ± 1.4 years in patients with a BMD decrease. A multiple myeloma was diagnosed in a patient with vertebral fractures during Dmab treatment. After The identification of Dmab RI most patients mantained same treatment. Of the patients with incidental fragility fractures, 2 started combined treatment with teriparatide and Dmab, 1 changed to teriparatide and 2 maintained the same treatment. Of the 7 patients with BMD only 1 changed to zoledronic acid. Conclusion Most patients with IR to Dmab treatment had previous fragility fractures and had been previously treated with bisphosphonates for a mean duration of 5 years. The patients with a significant decrease in BMD had lesser duration of Dmab treatment than those who sustained fractures during Dmab treatment. Only one patient had a clinical cause for the IR development. Disclosure of Interests None declared

Background Cutaneous Polyarteritis Nodosa (cutaneous PAN) is a rare form of necrotizing vasculitis involving predominantly medium and small arteries. It is limited to the skin. The clinical course is mostly chronic, however it has a more favourable prognosis than systemic PAN. Cutaneous PAN may be preceded by infection, drugs intake or may reflect underlying inflammatory or autoimmune disease. The diagnostic criteria are controversial and in some cases overlapping with those of systemic PAN. Cutaneous PAN is limited to the skin, but occasionally patients present extracutaneous symptoms, namely: myalgia, arthralgia or multineuritis Objectives To describe the clinical manifestations of cutaneous PAN, emphasizing the extracutaneous manifestations, as well as comorbid associations. Methods Retrospective study (1989-2018) of patients with cutaneous PAN seen in a tertiary center: referral area 800.000 inhabitants. All the cases were reviewed regarding epidemiological, clinical, electromyographic, histopathological and laboratory findings. Infections and comorbid diseases were also reviewed. Results Twenty-three cases were recruited: 8 male (34.78%) and 15 female (65.22%) patients. Mean age at onset: 47 years (range 18-76). Mean follow-up period :105 months (range 12-380). Cutaneous manifestations included: subcutaneous nodules (95.65%), ulcers (52.17%), livedo reticularis (60.87%), livedo racemosa (8.70%), atrophie blanche (39.13%) and purpura (4.34%). Cutaneous PAN most commonly occurred on the legs (100%), followed by the thighs (47.83%), the upper limbs (39.13%) and the trunk (17.39%). The most frequent extracutaneous symptom were asthenia (78.26%). Extracutaneous manifestations, limited to the same area of cutaneous lesions, were myalgia (60.87%) and paraesthesia (56.52%). An electromyography was performed in 14/23 patients and mononeuritis multiplex was observed in 7 patients. Only one patient progressed to systemic PAN, with development of renal disease 12 month after the diagnosis. The following infections were identified: hepatitis C (2), tuberculosis (1), Streptococcus pyogenes (1) and rickettsia (1). Comorbid diseases included: autoimmune hypothyroidism (2), cutaneous psoriasis (1), recidivant polychondritis (1), adult onset Still’s disease (1), ulcerative colitis (1), pulmonary idiopathic fibrosis (1) and acute myeloid leukaemia (1). Glucocorticoids were used at medium-high doses (20-40 mg/day) in 20 patients as induction therapy, and NSAIDs at full doses in the remaining 3. Thirteen patients (56.52%) developed relapsing forms. Of this, 12 patients (52%) needed associated immunosuppressive treatment to prevent relapse. The most widely used immunosuppressant was methotrexate, followed by mycophenolate or azathioprine. Conclusion A long-term follow-up, with careful anamnesis and examination, including electromyography, is necessary to better characterize the cutaneous and extracutaneous manifestations of cutaneous PAN. The progression to systemic involvement is infrequent and cutaneous PAN has a more benign course from a clinical, and prognostic point of view. References [1] Diaz-Perez JL, De Lagran ZM, Diaz-Ramon JL, Winkelmann RK. Cutaneous polyarteritis nodosa. Semin Cutan Med Surg. 2007Jun;26:77–86. [2] Morgan AJ, Schwartz RA. Cutaneous polyarteritis nodosa: a comprehensive review. Int J Dermatol. 2010Jul;49:750–6. [3] Daoud MS, Hutton KP, Gibson LE. Cutaneous periarteritis nodosa: a clinicopathological study of 79 cases. Br J Dermatol. 1997May;136:706–13. [4] Nakamura T, Kanazawa N, Ikeda T, Yamamoto Y, Nakabayashi K, Ozaki S, et al. Cutaneous polyarteritis nodosa: revisiting its definition and diagnostic criteria. Arch Dermatol Res. 2009Jan;301:117–21. Disclosure of Interests None declared

Background Elderly onset rheumatoid arthritis (EORA) has several peculiarities. Which activity index should we use in this age group is still an unresolved issue. The use of ESR in DAS28 is widespread, but it is well known that ESR could increase with age, overestimating activity. DAS28-PCR or SDAI, which uses PCR, or CDAI that does not use analytical data, could be more suitable alternatives. Objectives To describe disease activity scores evolution in EORA and to analyse the correlation and concordance between them. Methods Longitudinal observational study in naive treatment EORA patients (ACR/EULAR 2010 criteria). Study visits were carried out basally (pre-treatment) and at month 1, 3, 6 and 12. DAS28 VSG, DAS28 PCR, SDAI and CDAI were calculated at each visit, and ACR/EULAR remission index at 12 months. The same rheumatologist made all visits and explorations. Correlations were analysed using the Spearman or Pearson correlation index (p value ≤ 0.001). The concordance was evaluated with the kappa index. The statistical study was performed with Stata 15.1. Results 45 patients with EORA were enrolled. Table 1 and 2 summarize the clinical and analytical characteristics. All the scores had a very good linear correlation both at baseline and at follow-up (correlation score: 0.83-0.98). A moderate concordance was observed from the baseline visit between DAS28-VSG and DAS28-PCR (k=0.43-0.52, p Conclusion The correlation between the different scores was good. However, the concordance between all the scores decreases as time passes, according to a higher percentage of patients in remission or low activity. This low-moderate concordance is demonstrated even between DAS28-PCR and DAS28-VSG, and when these are compared with CDAI and SDAI. CDAI and SDAI are the only ones that maintain a good concordance between them, even when the number of patients in remission or low activity increases. ACR/EULAR only maintains an acceptable concordance with the most restrictive scores, SDAI and CDAI. By contrast, agreement is very low with DAS28, especially by DAS-PCR. Disclosure of Interests None declared

Background Elderly-onset RA (EORA), which is defined as rheumatoid arthritis (RA) with an onset at >60 years of age, and polymyalgia rheumatica (PMR) are common rheumatic diseases in the elderly. Eicosanoids are biological lipids that serve a specific role as either activators or suppressors of systemic inflammation and have been involved in the development and progression of arthritis. Objectives We hypothesized that eicosanoid-related perturbations are related to arthritic symptoms in the elderly, and that by defining this eicosanoid profile, we might be able to define elements of inflammation pathobiology in this population. Methods Arthritis in the Elderly (ARTIEL) is a recent collection cohort with patients older than 60 years of age who have newly diagnosed arthritis. Blood samples were collected from these patients at baseline (pre-treatment) and 3 months after treatment, along with physician and patient outcome measures through 12 months. These patients were also compared with randomly-selected control individuals of the same age and gender. A thorough clinical examination was conducted and patients completed a health assessment questionnaire (HAQ). Disease activity score (DAS)28CRP was also calculated. Serum eicosanoids were determined by mass spectrometry at baseline and after 3 months of treatment and were classified into groups according to their eicosanoid precursors: eicosapentaenoic acid (EPA), docosohexanoic acid (DHA) or arachidonic acid (AA). Data processing and statistical analysis were performed in R. Results 64 patients (average: 75.15, standard deviation (SD) 6.80) and 18 controls (average: 75.39, SD, 6.04) were analyzed. Of these, 44 were diagnosed with RA and 20 with PMR. At the start of the study, patients had a mean DAS28CRP of 5.72 (SD, 1.05) and a mean HAQ of 1.64 (SD, 0.73). In addition, 84% of the patients reported scapular pain and 56% reported pelvic pain at baseline. After three months of treatment, patients had a mean DAS28CRP of 2.38 (SD, 1.23) and a mean HAQ of 0.36 (SD, 0.41). Several eicosanoids, especially anti-inflammatory species derived from EPA and DHA, were significantly downregulated in both RA and PMR patients at the start of the study as compared to normal controls (Figure 1). Three months after treatment, the levels of anti-inflammatory eicosanoid species derived from EPA and DHA went back to normal only in patients who responded to therapy. Conclusion These results suggest that certain eicosanoids may be key effectors in arthritis in the elderly and that the imbalance between pro and anti-inflammatory eicosanoids before and after treatment might be related to clinical and therapeutic outcomes in this population. References [1] A systematic review on the role of eicosanoid pathways in rheumatoid arthritis. Hoxha M. Adv Med Sci. 2018 Mar;63(1):22-29. [2] Kobak S, Bes C. An autumn tale: geriatric rheumatoid arthritis. Therapeutic advances in musculoskeletal disease. 2018 Jan;10(1):3-11. PMID: 29290762 [3] Quehenberger O, Dennis EA. The human plasma lipidome. New England Journal of Medicine. 2011 Nov 10;365(19):1812-23. PMID: 22070478 Disclosure of Interests Roxana Coras: None declared, Rekha Narasimhan: None declared, Arthur Kavanaugh Grant/research support from: UCB Pharma, Lourdes Mateo Soria: None declared, Oswald Quehenberger: None declared, Monica Guma: None declared, Melania Martinez-Morillo: None declared

Background Clinical manifestations of systemic lupus erythematosus (SLE) and infections sometimes are difficult to distinguish. In clinical practice low complement and anti(ds)DNA levels are used to assess lupus activity but its determination usually requires some days. Leukocyte count, CRP and ESR cannot discriminate SLE from infectious processes. Calprotectin could be a good biomarker to assess lupus activity since it is more specific than CRP and ESR and faster to analyse than anti(ds)DNA. Objectives Our aim is to determine serum calprotectin levels in patients with SLE, and its correlation with analytical and clinical manifestations, especially with disease activity. Methods A total of 148 patients were included. All patients included fulfilled the SLE criteria (SLICC 2012). A quantitative ELISA analysis was performed to assess levels of serum calprotectin (CALPRO AS, Norway). Other biomarkers of lupus disease activity were also assessed (levels of anti(ds)DNA, hypocomplementemia, ESR and CRP). Clinical variables and activity/damage index (SLEDAI/SLICC) were also evaluated. The study was approved by the Clinical Research Ethics Committee of the hospital and all patients signed an informed consent. The results were compared with a healthy control group of similar age and sex (n=20). Results 134 patients (92%) were women with a mean age of 46±12 years and an average SLE evolution of 12±7 years. Mean SLEDAI was 2±2 (105 inactive [ 13]). Mean SLICC was 0.31±0.70. No significant differences were observed in serum calprotectin levels between patients with SLE and healthy controls (2.93±2.35 vs 2.17±1.49 µg/mL, p=0.160). Calprotectin was positively correlated with CRP (r=0.447, p= 100UI/mL) had higher calprotectin compared to patients with lower anti(ds)DNA (3.20±2.63 vs 2.42±1.57 µg/mL; p=0.027), however this pattern was not observed with hypocomplementemia. Contrary to what we expected, we did not observe significant differences on calprotectin levels depending on SLEDAI index classification (cutoff at 4 and 12). Moreover, no differences were observed on calprotectin levels between those patients with/without clinical manifestations such as serositis, arthritis or glomeruloneprhitis. Patients with antiphospholipid antibodies had higher calprotectin levels (3.75±2.04 vs 2.77±2.38 µg/mL;p=0.045). Conclusion Serum calprotectin levels were positively correlated with CRP levels and leukocyte count. Patients with higher anti(ds)DNA levels had higher calprotectin levels, however we did not observe significant differences depending on SLEDAI index or the presence of arthritis, serositis neither glomerulonephritis. Even that calprotectin determination is faster than anti(ds)DNA levels and could be helpful in assessing inflammatory activity. There is an interesting relation between antiphospholipid antibodies and calprotectin. This study should be continued in a larger sample of active SLE patients to assess its utility in clinical practice as a discriminating biomarker for flares and even infection Disclosure of Interests None declared

Background: Dermatomyositis (DM) is an idiopathic inflammatory myopathy. The recent years has increased its knowledge thanks to best characterization of myositis-specific antibodies that correlate with different clinical phenotypes.with different clinical phenotypes. Objectives: To describe the clinical and analytical features of a series of dermatomyositis: clinical debut, clinical manifestations as well as the treatments received and the evolution of the disease. Methods: Patients diagnosed of dermatomyositis in a tertiary hospital between the years 1978-2018 according to the criteria of Bohan and Peter (1975) and according to Dalakas’ classification criteria (2015). Clinical, analytical and immunological profile data were collected, as well as treatments received and the evolution of the disease. Results: A total of 59 inflammatory myopathies diagnosed between the years 1985 and 2018 were included. 46 were dermatomyositis (78%), 9 polymyositis (15%) and 4 necrotizing myositis (7%). 69% were women and 22% were smokers. Clinic started at 54 ± 17 years. The initial manifestation was pulmonary in 26.7% followed by cutaneous manifestations (24.4%) and the muscular (22.2%), while a 17.8% started skin and muscular manifestations at the same time. 45.5% behaved like a myopathic DM while a 28% as amyopathic DM and antisynthetase syndrome respectively. 38% presented interstitial involvement being the most common non-specific interstitial pneumonitis (76%)followed by usual interstitial pneumonitis (17%). In these patients, DLCO was decreased (mean of 58.9% and 13.2) as well as the FVC (average of 58%, 2.5L). 73.3% presented cutaneous involvement being the most common manifestations the Gottron papules (37.8%) and the heliotrope rash (35.6%), and up to 27% had cuticular affectacion. 64% had muscle involvement, afecting proximal and symmetrical. Neck flexors were affected in a 38% of patients while 20% had dysphagia.Only 3 patients presented dysphonia (7%) and 2 myocarditis Analytical data Conclusion: 80% received corticotherapy at a dose of mg/kg/day and 20% required high doses of metilprednisolone due to muscular involvement or pulmonary. 18% immunoglobulins and 11% cyclophosphamide. As maintenance 80% received disease modifiying antirheumatic drug in addition to corticosteroid therapy in descending doses (azathioprine 22%, dolquine 15%, tacrolimus 13.3%, rituximab 11%) due to muscle (29.9%), cutaneous (24.4%) and pulmonary involvement (22.2%). As complications, 2 cases of the syndrome were registered hemophagocytic Virtually all patients they presented pulmonary hypertension (mean 34 ± 12 mmHg). 5 patients (11.1%) were diagnosed with neoplasia, two of them after the diagnosis of DM. The mortality was 24%. 3 patients died due to a rapidly progressive pneumonitis, another 2 due to alveolar hemorrhage and three of them due to complication of the neoplastic disease. Dermatomyositis occurs in a variable way, with predominance of pulmonary, skin and muscle manifestations. They require corticotherapy and immunosuppressive treatment for maintenance, even so, mortality is high. Disclosure of interests: None declared

Background Extra-articular manifestations (EAM) in rheumatoid arthritis (RA) are more prevalent in patients with seropositive, nodular and long term disease. Around 40% of patients with RA can develop EAM, although the incidence has decreased with the “treat to target” strategy. Objectives To describe EAM in a cohort of patients with rheumatoid arthritis. Methods Retrospective descriptive study of patients diagnosed with RA between 2000 and 2018 who are currently under active biological treatment. Cardiovascular risk factors, baseline characteristics of RA and EAM during their evolution are collected. Statistical analysis is performed with SPSS 24. Results We recruited 108 patients, 81.5% female, with mean age at onset of RA of 50 ±15 years (range 17-82). RF was positive in 84.3% and ACPA in 81.3%. Erosions were observed on hands or feet at diagnosis in 35.2%. Mean follow up period: 12±8 years. The most common initial treatment was methotrexate (82.4%) and 44.4% received other DMARDs: leflunomide (32.3%), hydroxychloroquine (16.2%) and others (9.1%). DAS28VSG at diagnosis was 4.15±1.18 (1.89-6.02) and at the introduction of the first biological drug it was 5.02 ±1.16 (mean years since diagnosis 6±5). The first biological drug was etarnecept (40.7%), followed by adalimumab (17.6%), abatacept (15.7%), rituximab (7.4%), tocilizumab (7.4%) and infliximab (4.6%). 57.4% discontinued the first biologic: secondary failure (58.1%) or adverse effects (20.6%). 65.7% (71 patients) developed some EAM during disease evolution and 14.8% appeared during the first year. During this first year, secondary Sjogren (37.5%) was the most frequent, followed by rheumatoid nodules (18.7%), EPID (18.7%) and osteoporosis (18.7%). Two or more EAMs were present in 33.3% of patients. Fifty-four patients (56.3%) did not present any EAM at the beginning of the biological treatment and 29.6% (21 patients) developed EAM afterwards, with an average of 1±3 years. Pulmonary involvement was the first EAM to appear after the onset of biological treatment, followed by anemia (both in the first year). Summarizing the data of all patients over time, the most frequent EAMs were: osteoporosis (28.7%, mean appearance: 5 years from diagnosis); secondary Sjogren syndrome (27.8%, mean 6 years); normochromic normochromic anemia (21.3%, mean 8 years) and pulmonary involvement (17.6%, mean 4 years), presented as EPID (63.3%), NINE (25%), NIU (8.3%) and pulmonary nodules (8.3%). Other EAM observed were: cutaneous involvement in the form of rheumatoid nodules (15.7%, mean 5 years); ophthalmological involvement (6.5%, mean 18 years) (PUK 2.8%, uveitis 1.9%, scleritis 0.9%, episcleritis 0.9%); amyloidosis (3.7%, mean 16 years) and vasculitis (2.4%, mean 14 years). Conclusion EAM have a high prevalence in RA patients receiving biologic treatment (76.6%). A third part of patients had 2 or more EAMs. 14.8% develop the first EAM during the first year of evolution of RA and 30% in the first 5 years. The most frequent are osteoporosis and Sjogren’s syndrome, according to what is described in the literature. Pulmonary involvement is the most rapid during the evolution of RA with an average of 4 years. References [1] Marcucci E, Bartoloni E, Alunno A, Leone MC, Cafaro G, Luccioli F, et al.Extra-articular rheumatoid arthritis. Reumatismo. 2018Dec20;70(4):212-224 Disclosure of Interests None declared

Resumen La enfermedad de Behcet es una vasculitis caracterizada por ulceras bucales y genitales. La afectacion neurologica o neuro-Behcet es una manifestacion infrecuente, de predominio en el genero masculino y que aparece de 2 a 4 anos despues de la primera manifestacion clinica. El neuro-Behcet cursa ocasionalmente lesiones cerebrales pseudotumorales. Presentamos 2 casos de pacientes diagnosticados de neuro-Behcet tras la deteccion de lesiones cerebrales pseudotumorales y se realiza una revision de la literatura.

Background:There are several studies assessing bone mass and osteoporosis (OP) development of patients with giant cell arteritis (GCA) during glucocorticoid treatment, however data at time of diagnosis is scarce.Objectives:To analyze bone metabolism, bone mineral density (BMD) and trabecular microarchitecture by measuring trabecular bone score (TBS) in patients with recent diagnosis of GCA.Methods:Retrospective observational study in which we included all patients with GCA and bone metabolism assessment at diagnosis. We recorded: risk factors for OP (previous fractures and anti-OP treatments), bone metabolism (calcium, phosphate, PTH, 25-vitaminD [25-OHD]) and the DXA results (Lunar, DPX) at the time of diagnosis and during the follow-up. The statistic analysis was made with SPSS.Results:36 patients (27 W: 9M) with a mean age of 76±9 years were included. The clinical characteristics of the GCA at diagnoses were: headache (81%), constitutional syndrome (58%), allodynia (42%), visual loss (42%, binocular in 2 patients), jaw claudication (39%), polymyalgic syndrome (28%), amaurosis fugax (25%) and lingular necrosis (n=1, 3%). Additional diagnosis tests included: temporal biopsy (86%, being pathologic in 55%), temporal ultrasound (97%, pathologic 34%), extra-cranial arteries ultrasound (81%, pathologic 7%) and PET/CT (19%, pathologic 86%). Up to 61% received three metilprednisolone bolus (mean dose 1700mg). 7 patients (19%) had previous OP and 3 had already suffered a fragility fracture (Colles, vertebral and femur). Only 4 patients were receiving anti-OP treatment (2 oral bisphosphonate, 2 zoledronate).At time of diagnosis of GCA, the mean of 25OHD was 17±13ng/mL (88%, Conclusion:Low bone mass and vitamin D insufficiency are frequent in patients with recent diagnoses of GCA and up to 38% have poor bone microarchitecture at baseline. Thus, it’s important to perform a bone mass assessment in all patients with recent diagnose of ACG and initiate therapeutic or preventive anti-OP treatment if required.Disclosure of Interests:None declared

Background:The incidence and factors related to an inadequate response to denosumab (Dmab) treatment remain unclear.Objectives:To describe clinical, analytical and densitometric characteristics of patients with inadequate response (IR) to Dmab in clinical practice. IR was defined as the presence of a new fracture [fxs-IR] or a significant decrease in BMD (≥5% lumbar or ≥4% femoral) [BMD-IR].Methods:retrospective study of patients with IR to Dmab treatment. Data of demographic variables, risk factors for osteoporosis, history of fractures, previous anti-osteoporotic treatment, densitometric and analytical parameters were collected before and after IR.Results:22 patients were included (19W:3M) with mean age of 75±10years. The causes of osteoporosis were: postmenopausal (50%), induced by glucocorticoids (22.7%), alcoholic (9.09%) and multifactorial (18.8%). Most patients were previously treated with bisphosphonates (59.09%, duration 5.2±2.6y) and had previous vertebral fractures (54.54% %, median 3).During Dmab treatment, 10 patients presented a BMD-IR (with a mean bone loss up to -3.5% at femur and -5.8% at lumbar spine) and 12 had fxs-IR (vertebral [n=8], humerus [n=1], pelvis [n=1], tibia [n=1]). No significant differences were observed in duration of Dmab between both IR groups (Fxs-IR: 3,2±1,9 vs BMD-IR: 2,4 ± 1,2y). In the BMD-IR, the BMD loss was higher at lumbar spine than at total hip (-6.6%±3.7 vs -1.9%±4.8). Only 1 patient of the fxs-IR had a secondary cause of IR (mieloma multiple).In the fxs-IR group, most patients started combined treatment with teriparatide (n=4), 1 changed to teriparatide and 7 remained with Dmab. In the BMD-IR group, most mantained Dmab treatment (n=8) and 2 switched to zoledronate.Conclusion:Most patients who developed IR to Dmab had been previously treated with bisphosphonates and had previous fragility fractures and appears within the first 3 years of treatment. BMD loss seems to be more marked at spine than total hip. Only one patient had a secundary cause of IR.Disclosure of Interests:None declared

Background Cardiac block in neonatal lupus is associated with placental transfer of anti-Ro antibodies. The effect of these antibodies on cardiac conduction disorders in adult patients is controversial. The association between anti-Ro antibodies and heart rhythm disorders have been described in isolated cases. However, there are just a few studies that analyse the relationship between autoimmune diseases and electrocardiographic disturbances. Objectives To determine if there are differences in cardiac conduction of SLE patients in presence of anti-Ro antibodies. Methods All patients included fulfilled the SLE criteria, SLICC 2012 and they were followed up in a single centre. The inclusion was consecutive and voluntary. Patients who took drugs that altered the conduction (except antimalarial drugs), and those who had heart or thyroid disease were discarded. All patients were assessed blindly by a cardiologist who performed an interrogation and physical examination, an electrocardiogram, an echocardiogram and a 24 hour Holter study. Besides, a rheumatologist performed a clinical and analytical assessment including a qualitative analysis by immunoblotting of anti-Ro Ab and a quantification by chemiluminescence of the anti-Ro52 and Ro60 Ab. The presence of other SLE specific Ab (ANA, DNA, antiphospholipids), was also analysed. Clinical, analytical and, activity and damage indexes, were collected (SLEDAI and SLICC). The data were compared by Student’s t test, Fisher’s test and Chi square using 21 SPSS version. The level of significance was established at 5%. Results 145 patients were included: 91.7% women, average age 45±12, average disease duration 11 years. The patients were undergoing the following treatments: antimalarial 91%, mycophenolate 20%, azathioprine 12%, biological treatment 5% and glucocorticoids 70%. The clinical characteristics are summarised in table 1. There were no significant differences between the group of positive and negative anti-Ro Ab in terms of gender, age, clinical characteristics or cardiovascular risk factors. None of the patients was affected by an atrio-ventricular block and the rest of the electrocardiographic alterations had no clinical significance and did not predominate in the positive Ro Ab group. Additionally, no differences in heart rate, ventricular extrasystoles or PR, QT or QRS intervals were detected between both groups. The echocardiogram’s findings were not relevant and there were no differences between groups. Since the majority of patients with SLE are double positive, differences between the two subspecificities of Ro (52 and 60) could not be analysed. On the other hand, no differences were found in cardiac conduction regarding the treatments received, the activity or damage indexes, or the analytical or clinical characteristics of the patients. Conclusions The study results show that there are no differences in cardiac conduction according to the presence of anti-Ro antibodies in SLE patients. Thus, the cardiac screening in SLE patients with anti-Ro positive antibodies seems not helpful in clinical practice. Disclosure of Interest None declared

Background There are few recent large cohort epidemiological studies that analyse the clinical profile of patients with psoriatic arthritis (PsA) who require biological treatment, as well as pharmacological survival and reasons for the suspension of these treatments. Objectives To describe the clinical, demographic, epidemiological and radiographic characteristics of patients with PsA in our centre, as well as pharmacological survival and reasons for the suspension of different biological treatments. Methods Retrospective analysis of patients with PsA treated between 1985 and 2015 at a University Hospital with a referral area of 8 50 000 inhabitants. Demographic, clinical, laboratory and imaging data, as well as patients’ clinical and medical treatment records. Results The main characteristics of the cohort are summarised in table 1: Approximately one third of patients with PsA (32.3%) required treatment with a biological drug during the course of their disease. The most frequently prescribed first-line drugs (89.5%) were tumour necrosis factor-α inhibitors (TNFi). During the course of their disease, 27 (21.9%) patients who started biological treatment received a biological drug other than TNFi. Of the 124 patients who started treatment with a biological DMARD, more than half (65, 52.8%) required a change to a second drug, and of these, 27 (41.5%) changed to a third, with up to 7 different biological drugs required in one case. The mean survival time for the first bDMARD was 42.8±42.3 months, with secondary failure the most frequent cause of treatment change (37.8%), followed by adverse effects (27.1%) and primary failure (18.9%). The adverse effects registered were: 20% infections, 20% appearance of neoplasms, 5% allergic reactions and 55% other causes. Conclusions PsA presents a similar distribution by sex and is usually diagnosed at around the age of 50, having been preceded by cutaneous involvement. PsA is an entity of considerable severity and up to a third of patients will require biological treatments during its evolution. More than half of them will receive a second biological treatment. The retention rate of each drug varies but tends to decrease with each drug change. Disclosure of Interest None declared

Background Pulmonary affection is a serious complication in Rheumatoid Arthritis (RA) with an important impact on mortality and morbidity. Objectives To describe the clinical and radiographic characteristics of patients with RA in our centre, and specifically from those with interstitial lung disease (ILD). Methods Retrospective analysis of patients with RA and pulmonary affection from a University Hospital with a referral area of 85.000 inhabitants. Clinical, laboratory, imaging and pulmonary function test (PFT) data was recorded. Results Data from 85 patients, 37 (43.5%) male and 48 (56.5%) female, were recorded. The mean time from RA diagnosis to pulmonary disease was 9.2 years, with mainly erosive (58.8%) and seropostive (84.7% were positive rheumatoid factor (RF) and 84.2% anti-citrullinated protein antibodies (ACPA)) disease. The 56.5% of the patients were smokers or ex-smokers. PFT results were documented in 75.3% of the patients: diffusing capacity for carbon monoxide (DCLO) baseline values were over 60% in a 38.8% of cases and forced vital capacity (FVC) baseline values were over 50% in a 69.4% of patients. The most frequent High Resolution CT scan pattern was the presence of bronchiectasis (64.7%), followed by ILD (31.8%) and, specifically, the Usual Interstitial Pneumonia pattern (UIP, 66.6%); lung infections (25.8%), rheumatoid lung nodules (22.4%), pleural effusion (15.3%), lung tumours (9.4%) and bronchiolitis (5.8%). The presence of extra articular manifestations as rheumatoid nodules (27%), epieschleritis (2.3%) and vasculitis (9.4%) was registered. The coexistence of Chronic Obstructive Pulmonary Disease (COPD) was recorded too (24.7%). Related to treatment, 84.7% of patients had received Methotrexate and in a 40% of cases, at least one biological disease-modifying antirheumatic drug had been administrated. Though the causes were not registered, a 25.9% of deaths were documented. In parallel we did a subanalysis of ILD related to RA cases, summarised in table 1 (n=27): Conclusions Bronchiectasis is the most common pulmonary manifestation of RA patients in our area. The most frequent clinical profile is a non-smoker woman with seropositive disease. ILD affects approximately one third of RA patients in our area and is an underdiagnosed entity. We consider that it is necessary to establish a screening program to diagnose and treat it early. Disclosure of Interest None declared

Resumen Fundamento y objetivo La amiloidosis secundaria (AA) es una complicacion de enfermedades inflamatorias cronicas tales como las enfermedades reumaticas. El objetivo de este estudio fue evaluar la prevalencia de amiloidosis AA en una serie retrospectiva de pacientes con espondiloartritis. Pacientes y metodo Estudio retrospectivo entre 1984-2013. Se revisaron las historias clinicas de los pacientes con diagnostico de espondiloartritis y amiloidosis AA. Resultados Se identificaron 1.125 pacientes con espondiloartritis. Quince (1,3%) presentaban amiloidosis AA: 14 (93,3%) fueron sintomaticos, en la mayoria de ellos se presento como sindrome nefrotico. Cinco (33,3%) estaban diagnosticados de espondilitis anquilosante (EA), 5 (33,3%) de espondiloartritis asociada a enfermedad inflamatoria intestinal (EII), 4 (26,7%) de artritis psoriasica y uno (6,7%) de artritis reactiva. La media de edad de evolucion desde el diagnostico de espondiloartritis hasta el diagnostico de amiloidosis AA fue de 23,9 anos. La mortalidad al ano y a los 5 anos fue del 30 y 50%, respectivamente. Conclusiones En este estudio retrospectivo de pacientes con espondiloartritis, la prevalencia de amiloidosis AA sintomatica fue del 1,24%. La mayoria fueron varones con una EA o espondiloartritis asociada a EII de larga evolucion. El motivo mas frecuente de sospecha del desarrollo de amiloidosis AA fue el sindrome nefrotico.

Several case reports, small case series, and original research papers have recently suggested that the action of certain auto-antibodies related to connective tissue diseases may be responsible for significant cardiac rhythm disturbances in adults. The relationship between anti-Ro/SSA antibodies and congenital complete atrioventricular block is well recognized in the fetal heart. Herein we review the emerging evidences of the link to increased levels of anti-Ro/SSA antibodies with rhythm disorders of unknown origin in the adult. Confirmation of this distinct etiology may eventually be the basis for new therapies.

Background Septic arthritis (SA) is a rheumatologic emergency as joint destruction occurs rapidly and can lead to significant morbidity and mortality. Accurate diagnosis can be particularly challenging in patients with underlying inflammatory joint disease. Crystal-induced arthritis (CA) is a risk factor for its appearance. When both conditions appear simultaneously, CA may mask diagnosis of infection and delay the antibiotic treatment. Objectives To describe the characteristics of patients with concurrent septic and CA. Methods Retrospective analysis of patients with coexistence of septic and CA attended between 1985 and 2015 in a university hospital with a reference area of 850,000 inhabitants. We collect demographic, clinical, laboratory and imaging data as well as patient medical treatment, complications and evolution records. All patients had positive bacterial culture (blood and/or joint fluid) and crystals in synovial fluid. Results A total of 123 patients with SA were identified. 20.3% (n=25) of them had concomitant CA, with mean age of 67 years (SD 14), 17 (68%) males and 8 (32%) females. Risk factors were: diabetes (24%), diuretic drugs (24%), chronic renal failure (16%) -2 of them undergoing hemodialysis and 4 kidney transplant patients with immunosuppressive treatment. In only 2 cases there was a previous arthrocentesis. The mean diagnostic delay was 14 days (SD 13) (data available in 14 cases). The most commonly affected joint was the knee (48%), followed by the foot (20%) and the hip (12%). In 2 cases several joints were involved at the same time. In synovial fluid cytological studies, the most frequently identified crystals were: urate (60%), calcium pyrophosphate (20%) and hydroxyapatite (8%). In 32% of cases gram staining was positive, but 88% of patients had a positive joint fluid culture, with the most frequently isolated germs being methicillin-sensitive S. aureus (48%), methicillin-resistant S. aureus (MRSA) (12%) and M. tuberculosis (12%). 32% of patients presented positive blood cultures (12% with negative synovial fluid culture), although 48% of patients had fever at the time of diagnosis. It should be noted that 48% had radiological baseline damage. Surgical debridement was performed in 32% of patients. Evolution was successful in 56% of patients; although intercurrent complications were usual (40%). Mortality was 8% -one case due to acute pulmonary edema and the other because of septic shock. Conclusions Coexistence of infectious and CA represents a diagnostic challenge and requires a high suspicion index. It usually appears in elderly patients with comorbidities. Gout was the most prevalent CA. S. aureus was the most commonly causative pathogen, with a high rate of MRSA infection. If it9s treated early the evolution is usually favorable, which makes synovial fluid microbiological study imperative. Disclosure of Interest None declared

Background Anti-Ro antibodies (Ab) are especially directed against two antigens, Ro52 and Ro60, with different structure and function. Studies in systemic lupus erythematosus (SLE) have been described a relationship between the presence of anti-Ro Ab and dry syndrome, subacute cutaneous lupus, myocardial involvement or minor renal involvement. However, studies about the clinical usefulness of determining the 2 specificities of anti-Ro Ab in SLE are not conclusive. It seems that anti-Ro52 could be associated with joint involvement and lung disease, while anti-Ro60 could be related to late-onset SLE. In this paper we evaluate the clinical usefullness of anti-Ro52 and Ro60 Ab chemiluminescence quantification in patients with SLE. Objectives To analyze the presence of anti-Ro52 and Ro60 Ab in a cohort of patients with SLE and evaluate its correlation with clinical, analytical and immunological parameters. Methods 152 patients with SLE (according to the SLICC criteria 2013) attended in an university hospital were included. Qualitative immunoblot analysis of anti-Ro Ab was performed on all of them; those with positive values were also assessed by a chemiluminescence quantification of anti-Ro52 and Ro60 Ac (normality Results 91% (138) were women with a mean age of 46±12 years (range, 20–75) and an average SLE evolution of 12±7 years (range, 1–40). 32% (49/138) of the patients had anti-Ro positive determination by immunoblot: 36 were anti-Ro52 positive (mean value 696±726 CU) and 48 anti-Ro60 positive (mean value 504±696 CU) by chemiluminescence. Only one anti-Ro52 positive patient was anti-Ro60 negative. 10.5% had late-onset SLE. The mean SLEDAI was 1.84±2.48 while the SLICC was 0.32±0.7, with a mean anti(ds)DNA Ac values about 207.87±357.15 IU/ml. 37.5% had hypocomplementemia. Anti-Ro positive patients by immunoblot had a higher value of anti(ds)DNA compared to the patients without Ro positive values and patients with both positivity by chemiluminescence had lower prevalence of APS, however the quantitative values of anti-Ro52 and anti-Ro60 were not related with clinical, analytical or immunological parameters. Conclusions 32% of patients in the cohort were anti-Ro positive being mostly anti-Ro60 positive. The presence of anti-Ro Ab was associated with higher value of antiDNAds and lower prevalence of APS. However, the quantitative values of anti-Ro52 and anti-Ro60 Ab by chemiluminescence were not related to the different parameters analyzed in the study. These results indicate the need to assess the quantification of anti-Ro Ab in a larger cohort of patients with SLE. Disclosure of Interest None declared

Background The analysis of synovial fluid is an important tool for diagnosing joint disease. When synovial fluid is removed, the white cell count (WCC) decreases with time, and an inflammatory liquid could become a false non-inflammatory specimen. Reagent strip testing of urine is a valid tool for the diagnosis of urinary tract infection, via the detection of leukocyte esterase activity. It has been used for the analysis of others body fluids. Synovial fluid test at the site of arthrocentesis using reagent strips could have potential benefits as a screening tool. Objectives To evaluate the performance of leukocyte esterase reagent strips for diagnosis of inflammatory synovial fluid. Methods Prospective single center study. We analyzed synovial fluids samples collected from patients in a tertiary university Hospital (November 2015- December 2016). Synovial fluid samples were tested within 1 hour after collection. We analyzed: The presence of leukocyte esterase using the leukocyte esterase reagent strips test (originally designed for urine test, URI-Clip Test, Menarini Diagnostics). It was recorded semi quantitatively: negative, 1+ (>25 WBC/uL), 2+ (>75 WBC/uL) or 3 +(>500 WBC/uL) by comparison with a standard color chart found on the container9s label. The WCC, formula, glucose level. The WCC was measured by manual leukocyte counting, using saline as diluents. Cultures were also collected. We consider + if leucocyte esterase pad was more or equal than 1+ positive. The cut-off for the WCC (>2000cells/mm3) was used to differentiate between inflammatory and non-inflammatory specimens. We compared the WCC (reference standard diagnostic test) with the presence of leukocyte esterase using the leukocyte esterase reagent. Sensitivity (Se), specificity (Sp), PPV, NPV were determined. P-value smaller than 0.05 were considered significant. Results During the study period, 125 joint fluid specimens were analyzed: 56 (44.8%) mechanical and 69 (55.2%) inflammatory. Of the mechanical fluids 33 (58.9%) were negative by leukocyte esterase reagent and of the inflammatory fluids 67 (97.1%) were positive. The Se and Sp of leukocyte esterase reagent was 97.1% and 58.9% respectively. The PPV was 74.4% and NPV was 94.3%. The 2 false-negative results (negative by leukocyte esterase reagent but more than 2000 WBC/mm3), showed a predominance of mononuclears (> =91%), the median WCC was 2 775/mm and median neutrophil percentage was 8.5%. For inflammatory fluids: semi-quantitative results (negative, 1+, 2+ and 3+) were significantly different regarding the main leukocyte, neutrophil and lymphocyte count (table). Conclusions Our results demonstrate that leukocyte esterase reagent strips are a rapid, cheap, and sensitive tool to identify inflammatory synovial fluid. Leukocyte esterase reagent strips had an excellent Se but a poor Sp, it could be used as a screening tool in primary care practice. A positive result may indicate an inflammatory process, then the patient should be referred to a rheumatologist. Disclosure of Interest None declared

a b s t r a c t Septic pyogenic arthritis of the acromioclavicular joint is a rare entity that occurs in immunosuppressed patients or those with discontinuity of defense barriers. There are only 15 cases described in the literature. The diagnosis is based on clinical features and the isolation of a microorganism in synovial fluid or blood cultures. The evidence of arthritis by imaging (MRI, ultrasound or scintigraphy) may be useful. Antibiotic treatment is the same as in septic arthritis in other locations. Staphylococcus aureus is the microorganism most frequently isolated. Our objective was to describe the clinical features, treatment and outcome of patients diagnosed with septic arthritis of the acromioclavicular joint at a Rheumatology Department. We developed a study with a retrospective design (1989–2012). The medical records of patients with septic arthritis were reviewed (101 patients). Those involving the acromioclavicular joint were selected (6 patients; 6%).

Resumen La artritis septica de la articulacion acromioclavicular (ACV) es una entidad poco frecuente que se presenta en pacientes inmunosuprimidos o con discontinuidad de las barreras de defensa. En la literatura unicamente se han descrito 15 casos hasta la fecha. El diagnostico se basa en la presencia de clinica compatible junto al aislamiento del germen en la articulacion o en los hemocultivos. Las tecnicas de imagen (resonancia magnetica, ecografia o gammagrafia) pueden ser utiles en la localizacion del proceso. El tratamiento antibiotico es el mismo que en la artritis septica de otra localizacion y Staphylococcus aureus es tambien el microorganismo aislado con mas frecuencia. Se describen las caracteristicas clinicas, el tratamiento y la evolucion de los pacientes diagnosticados de artritis septica de la articulacion ACV en un servicio de Reumatologia, mediante estudio retrospectivo de revision de historias clinicas de los pacientes atendidos por artritis septica en dicha unidad (101 pacientes) en el periodo de 1989-2012. Seis enfermos (6%) tuvieron infeccion en la articulacion ACV con confirmacion microbiologica.

Resumen Presentamos el caso de una mujer de 51 anos con antecedentes de lupus eritematoso sistemico no organo especifico de anos de evolucion, que inicia clinica de sindrome toxico junto adenopatias y afectacion de pares craneales. Desarrollamos el diagnostico diferencial. Posteriormente, se muestran la resolucion del caso y la evolucion de la paciente.

Resumen El sindrome de Vogt-Koyanagi-Harada (VKH), tambien conocido como sindrome uveomeningeo, es una panuveitis granulomatosa bilateral y difusa que cursa con desprendimiento de retina seroso y que puede acompanarse de afectacion del sistema nervioso central, alteraciones dermatologicas y auditivas. Su nombre deriva de los autores que la describieron por primera vez. Afecta a adultos de ambos generos, entre los 20 y 50 anos de edad, y presenta una prevalencia aumentada en razas negras. Este sindrome inflamatorio probablemente sea el resultado de un mecanismo autoinmune, influenciado por factores geneticos. La evolucion de la enfermedad se divide en 4 estadios clinicos: prodromico, uveitico agudo, de convalecencia y cronico recurrente. El diagnostico es fundamentalmente clinico, mediante los criterios establecidos por la Sociedad Americana de Uveitis (AUS) publicados en el ano 2001. Es necesario realizar diagnostico diferencial con la oftalmia simpatica, el linfoma primario de celulas B, la escleritis posterior y el sindrome de efusion uveal. El tratamiento precoz y mantenido es la base de una buena evolucion.

Resumen No existe un acuerdo para definir la osteoporosis en mujeres premenopausicas y el diagnostico debe realizarse cuidadosamente y sin basarse unicamente en parametros densitometricos. Hay que tener en cuenta la presencia de otros factores de riesgo como los antecedentes de fracturas por fragilidad, enfermedades o farmacos osteopenizantes. Mas del 50% de las mujeres con osteoporosis premenopausica van a presentar una causa secundaria, el resto seran diagnosticadas de osteoporosis idiopatica. Las consideraciones terapeuticas estan limitadas por los escasos estudios en este grupo de pacientes, sobre todo en lo que se refiere al riesgo de fracturas. Por otro lado, no disponemos del indice de FRAX, ya que no se puede aplicar a pacientes premenopausicas. Este articulo pretende realizar una revision sobre la actitud que se debe seguir segun el tipo de osteoporosis premenopausica basandonos en la evidencia cientifica actual.

a b s t r a c t Background: Granulomatosis with polyangiitis (GP) is a necrotizing vasculitis of unknown etiology that involves small and medium caliber vessels. It is associated with anti neutrophil cytoplasm antibodies (ANCA). It most often affects the respiratory tract and the kidneys and its most important pathologic feature is the presence of necrotizing granulomas. Objectives: To detail the features of 15 patients with GP diagnosed in a university referral center. Patients and methods: Retrospective study: between 1984 and 2009, 15 patients with GP were diagnosed in our center. Epidemiological, clinical, laboratory test as well as pathologic studies and treatment were retrospectively analyzed. Biopsy diagnosis of GP was considered as an inclusion criterion. Results: Fifteen patients were diagnosed: 12 men and 3 women. Mean age at diagnosis: 52.2 years (14-78). 12 patients had a history of smoking. A biopsy was diagnostic in all patients. ANCA were positive in 11 cases, 6 had a cytoplasmic c-ANCA pattern. All patients had pulmonary involvement and seven (40%) had renal involvement. All patients received intravenous glucocorticoids and cyclophosphamide as induction therapy. During the disease progression 5 patients died.

Background Wegener's granulomatosis (WG) is a necrotizing vasculitis of unknown etiology that involves small and medium caliber vessels. It is associated with anti-neutrophil cytoplasm antibodies (ANCA). It most often affects the respiratory tract and the kidneys and its most important pathologic feature is the presence of necrotizing granulomas. Objectives To detail the features of 15 patients with WG diagnosed in a university referral center. Patients and methods Retrospective study: between 1984 and 2009, 15 patients with WG were diagnosed in our center. Epidemiological, clinical, laboratory tests as well as pathologic studies and treatment were retrospectively analyzed. Biopsy diagnosis of WG was considered as an inclusion criterion. Results Fifteen patients were diagnosed: 12 men and 3 women. Mean age at diagnosis: 52.2 years (14–78). Twelve patients had a history of smoking. A biopsy was diagnostic in all patients. ANCA were positive in 11 cases, 6 had a cytoplasmic c-ANCA pattern. All patients had pulmonary involvement and seven (40%) had renal involvement. All patients received intravenous glucocorticoids and cyclophosphamide as induction therapy. During the disease progression 5 patients died. Conclusion The clinical features of this series do not differ from those described by other authors. However, a history of smoking is more common than expected. Frequently used drugs were glucocorticoids and cyclophosphamide (oral and pulse therapy). The course was usually unfavorable, with outbreaks or complications due to immunosuppression, except for those with limited forms. Immunosuppressive therapy should be maintained indefinitely in most cases.

Resumen Los tratamientos biologicos pueden ser el heraldo de la curacion de la artritis reumatoide. Estos tratamientos dirigidos a dianas terapeuticas han cambiado el pronostico de la enfermedad. El tocilizumab es un anticuerpo monoclonal humanizado contra el receptor de la interleucina 6. El tocilizumab se ha aprobado en diferentes paises para el tratamiento de la artritis reumatoide moderada o grave. Se han publicado diferentes trabajos que han puesto de manifiesto la eficacia de tocilizumab en el tratamiento de la artritis reumatoide activa. Esta revision versa sobre la seguridad y la dosificacion del tocilizumab.