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SAPHO (synovitis, acne, pustulosis, hyperostosis, and osteitis) syndrome is a cluster of osteo-cutaneous manifestations that can lead to serious complications such as thrombosis of the subclavian vein or superior vena cava, mainly in patients with significant inflammatory involvement of the anterior chest wall. The objective of this study was to review the cases published in the medical literature related with the presence of thrombotic complications in patients diagnosed with SAPHO syndrome and to try to determine their possible pathogenic mechanism and risk factors. We analyzed 11 published reports of isolated clinical cases or case series, a total of 144 patients, which described a total of 15 cases of venous thrombosis. The clinical characteristics of these patients, evaluated to determine whether they meet the ASAS criteria for axial and peripheral spondyloarthritis, are analyzed and the need for early diagnosis and treatment is highlighted.

Resumen El sindrome SAPHO (sinovitis, acne, pustulosis, hiperostosis y osteitis) comprende un conjunto de manifestaciones cutaneo-osteoarticulares. Se han descrito algunas complicaciones graves que pueden aparecer durante la evolucion de la enfermedad, como la trombosis venosa, principalmente en pacientes que desarrollan afectacion inflamatoria grave de la pared toracica anterior. El objetivo de la presente revision fue analizar los casos descritos en la literatura medica relacionados con la presencia de complicaciones tromboticas en pacientes diagnosticados de sindrome SAPHO e intentar establecer los probables factores de riesgo y su posible mecanismo patogenico. Se analizaron 11 articulos publicados de casos clinicos aislados o series de casos, con un total de 144 pacientes, que describen en total 15 casos de trombosis venosa. Se exponen las caracteristicas clinicas de estos pacientes, se evalua si cumplen los criterios de clasificacion ASAS para espondiloatritis axial y periferica, y se resalta la necesidad de realizar un diagnostico y tratamiento precoces.

Fundamento: La enfermedad ósea de Paget (EOP) es un trastorno focal del hueso con aumento el número, tamaño y actividad de los osteoclastos. Algunos datos epidemiológicos apoyan la teoría de su relación con agentes ambientales tóxicos o infecciosos. Su interacción con algunas alteraciones genéticas predisponentes conducirían a la EOP. Las glutatión-S-transferasas (GST) intervienen en la metabolización de toxinas, al catalizar el ataque nucleofílico del sustrato fisiológico, glutatión reducido o GSH (g-Glu-Cys-Gly) sobre el centro electrófilo de un gran número de estructuras tóxicas. Estudiamos si la variabilidad de los genes GSTM1, GSTP1 y GSTT1 se relaciona con el riesgo a desarrollar EOP.Pacientes y métodos: Analizamos a 148 pacientes diagnosticados de EOP y a 207 individuos controles pareados en sexo y edad sin antecedentes de alteraciones óseas. Con DNA genómico obtenido de sangre periférica se estudió la presencia-ausencia de deleción en los genes GSTM1 y GSTT1, mediante PCR multiplex. El estudio del polimorfismo Ile105Val del gen GSTP1 se llevó a cabo mediante PCR y posterior digestión con la enzima de restricción BsmaI. Se analizó la distribución de genotipos mediante el test chi-cuadrado de Pearson. Cuando se encontraron diferencias estadísticamente significativas, realizamos una regresión logística multivariante para conocer el riesgo que puede generar la presencia de un determinado genotipo. Utilizamos el programa SPSS 21.0. Se consideraron diferencias estadísticamente significativas aquéllas con valores de p

Ankylosing spondylitis (AS) is the prototype of diseases that belong to the category of spondyloarthropathies. AS usually affects the sacroiliac joints and invariably involves the axial skeleton. Peripheral joint involvement, enthesitis, and extraskeletal manifestations are also important clinical and radiographic features of the disease1,2. A 55-year-old man was diagnosed with …

Background Ankylosing spondylitis (AS) is a chronic inflammatory disease that primarily affects the spine, characterized by bone proliferation and increased rigidity. Its prevalence is estimated 0.05- 0.25% of the population, and usually affects men in the second or third decade of life. Is associated with vertebral and hips loss of bone mineral density (BMD) due to inflammation. Both changes in biomechanical properties of the spine as decreased BMD increased bone fragility. Prevalence of vertebral fractures in clinical literature varies between 10-17%, being increased compared to healthy individuals of the same age and sex. Objectives An analysis of the presence of vertebral fractures in patients with AS and their possible relationship with various clinical, dietary and therapeutic factors is performed. Methods Patients with AS were included according to modified New York criteria. In such patients collected epidemiological characteristics (age, gender), year of diagnosis, toxic habits, body mass index (BMI), acute phase reactants (ESR, CRP), disease activity (BASDAI), physical measures (Schober and modified Schober, chest expansion, lateral flexion, occiput-wall distance, swallow-wall, intermalleolar and cervical rotation), function (BASFI), bone densitometry and cervical, dorsal, lumbar spine and sacroiliac radiographs were performed, analyzing the presence of fractures according to Genant semiquantitative method. Patients on modifying drugs in BMD, as well as those with some associated metabolic bone disease were excluded. Results 165 patients were included, with a predominance of males (110 vs 55). The mean age was 48 years and duration of illness of 13 years. 35 patients had a spinal fracture, which corresponds to 21.2%. The most commonly affected vertebra was T7 in the 38.3% of cases, followed by T8 (21.2%), T9 (14.1%) T6 and T11 (7.1% each). The most common type of fracture was moderate wedging (Genant grade 2). We found no statistically significant relationship with the presence of fractures and patients age, sex, smoking, enol, physical measurements or disease activity. Either with increase in acute phase reactants. The only relationship found was between fractures and bone mineral density at the lumbar spine (OR 23.5, 95% CI 1.199 to 463.114) in the first densitometry made at the beginning of the disease. Conclusions The prevalence of fractures in our patients was higher than that found in the literature. Patients with AS and fractures tend to be older than those without fractures, but this relationship did not reach statistical significance. We just found statistically significant relationship between the presence of fractures and lumbar spine bone mineral density in the first densitometry performed at initial diagnosis of AS. The most frequently affected vertebra was T7 with moderate wedging (Genant grade 2). References Caron T, Bransford R, Nguyen Q et al. Spine fractures in patients with ankylosing spinal disorders. Spine 2010; 35(11):E458-E464. Davey-Ranasinghe N and Deodhar A. Osteoporosis and vertebral fractures in ankylosing spondylitis. Curr Opin Rheumatol 2013;25(4):509-16 de Peretti F, Sane JC, Dran G et al. Ankylosed spine fractures with spondylitis or diffuse idiopathic skeletal hyperostosis: diagnosis and complications. Rev Chir Orthop Reaparatrice Appar Mot 2004; 90(5):456-65. Disclosure of Interest : None declared DOI 10.1136/annrheumdis-2014-eular.2991

Background Given the high cost of Biological Therapies (BT) and the possible benefits of its optimization on patients with Rheumatoid Arthritis (RA), Ankylosing Spondylitis (AS) and Psoriatic Arthritis (PA), it has been carried out an observational study. Objectives We analyzed the clinical and economic effect of the dose optimization. Methods It was carried out an observational study in patients in treatment with biological therapy since March to September 2013. For the optimization (reduction of dose and/or dose interval spacing), patients were selected according to clinical remission (DAS28 Results In the time period analyzed, 364 patients were with BT, 204 (56.1%) with RA, 107 with AS (29,4%), 42 (11.5%) with PA. From all patients, 134 (36.8%) were candidates for the dose optimization, 72 patients (53.7%) with RA, 48 (35.8%) with AS, 14 (10.4%) with PA. The average age was 54 years, standard deviation (SD) 13, with female predominance (56%:45%). 84 patients were in clinical remission, 20 patients in low activity, and it was optimized the dose in 30 patients according medical judgment or concomitant pathology. The optimization scheme is shown in the table. At the end of the study all patients maintained the optimized dose, except in two cases where it was necessary to return to their initial regimen for worsening symptoms and increased skin lesions respectively. The patient-year cost (theoretical dose) would be €835,538, while the optimization of dose was €594,102, which represents a saving of €241,435 (29%). The maximum saving was achieved by optimizing the treatments with Adalimumab and Etanercept, with 36% and 39% savings respectively. Conclusions The optimization of BT allows reducing the cost and maintaining the effectiveness and safety of treatments. The spacing in the doses of BT begins with proper selection of patients according to clinical criteria and disease activity and taking into account not only the economic cost. References Reino JG, et al. Consenso SER sobre la gestiόn de riesgo del tratamiento con terapias biolόgicas en pacientes con enfermedades reumaticas. Reumatol Clin. 2011. doi:10.1016/j.reuma.2011.05.002 Tornero J, Sanmarti R, Rodriguez V, Martin E, Marenco JL, Gonzalez-Άlvaro I, et al. Actualizaciόn del Documento de Consenso de la Sociedad Espanola de Reumatologia sobre el uso de terapias biolόgicas en la artritis reumatoide. Reumatol Clin. 2010;6:23–36. Carmona L, Gόmez-Reino J, Gonzalez R, BIOBADASER Gde. Spanish registry for adverse events of biological therapies in rheumatic diseases (BIOBADASER): State report as of January 14th 2005. Reumatol Clin. 2005;1:95-111. Disclosure of Interest None declared DOI 10.1136/annrheumdis-2014-eular.5492

Background Ankylosing spondylitis (AS) is a chronic inflammatory disease that primarily affects the axial skeleton. It associated loss of vertebral and hips bone mineral density (BMD) due to inflammation. Both changes in biomechanical properties of the spine as decreased BMD condition increased bone fragility. The prevalence of vertebral fractures in clinical literature varies between 10-17%. Nonsteroidal antiinflammatory drugs (NSAIDs) are drugs with analgesic and anti-inflammatory effect considered the mainstay of treatment in AS. In Spain, data from the Registry of Spondyloarthopaties of the Spanish Society of Rheumatology (REGISPONSER) 2006 showed that 95% were taking NSAIDs and 61% consumed them daily. The administration of NSAIDs could be prevented continuously radiographic progression in AS as well as the risk of fractures according to the literature. Objectives We analyzed longitudinally the impact of taking NSAIDs in BMD and the presence of fractures in a sample of 69 patients with AS. Methods Patients with AS were included according to modified New York criteria. In such patients epidemiological characteristics (age, gender), year of diagnosis, treatment with NSAIDs (daily vs only if pain is present), toxic habits, body mass index (BMI), ESR, CRP, disease activity (BASDAI), physical measurements, function (BASFI), bone density and the presence of fractures (according to Genant semiquantitative method) were collected. We exclude patients treated with biological therapies and those treated with modifying BMD drugs or with some associated metabolic bone disease. Results 69 patients (47 men and 22 women) were analyzed. The mean age was 49 years (23-87), with a time of disease duration of 13.4 years. The use of NSAIDs was only if the patient had pain in 66.7% of cases. Regarding BMD, 45% of patients had a decrease in BMD at the spine and 40.6% at the hip, but we didn9t found statistically significant differences in the way of taking NSAIDs (p=0.61 and p=0.42 respectively). They had vertebral fractures 11 to 69 patients, with no statistically significant differences among those taking NSAIDs continuously or on demand (p=0.998). These differences were still not significant after adjustment for confounders. Conclusions In our patients, the use of NSAIDs is mainly only if there9s pain, which contrasts with the revised literature. We observed a decrease in BMD of our patients, considering that it is young population, although no differences were found in patients as the dose of NSAIDs. The incidence of fractures in our patients is 16%, with no difference between groups as taking NSAIDs. Thus we can conclude that in our AS patients taking NSAIDs daily or only if they9ve pain does not affect BMD or the presence of fractures. References Per Aspenberg. Drugs and fracture repair. Acta Orthopaedica 2005; 76(6): 741-748. Mayor Gonzalez M y Batlle Gualda E. ?Cόmo hay que administrar los AINE en la espondilitis anquilosante? Semin Fund Esp Reumatol 2008;9:137-43. Vosse D, Landewe R, van der Heijde D et al. Ankylosing spondylitis and the risk of fracture: results from a large primary care-based nested case-control study. Ann Rheum Dis 2009;68:1839-42. Disclosure of Interest None declared DOI 10.1136/annrheumdis-2014-eular.3006