Can anticoagulation be withdrawn in APS patients after aPL negativization?

Long-term vitamin K antagonist (VKA) anticoagulation is the cornerstone of the management of subjects with thrombotic antiphospholipid syndrome (APS). Recent investigations have opened up new discussion points regarding the potential for stopping anticoagulant medication in patients with a history of thrombotic APS who no longer have detectable aPL (the so called aPL negativization). Despite the lack of unanimous agreement, some experts agreed on defining aPL negativization as the presence of two negative determinations, 1 year apart. What to do in order to optimize the management of these subjects with thrombotic APS when aPL turn negative is still a matter of debate. In this review, we aim to summarize the main evidence highlighting the magnitude of aPL negativizing among patients with APS and the features to keep in mind when considering (or not) stopping anticoagulation.

Steroids in Lupus: Enemies or Allies.

Glucocorticoids are the gold standard treatment for reducing immune activation and inflammation in a wide range of inflammatory and systemic autoimmune diseases. Glucocorticoids have potent and fast actions that quickly relieve some symptoms and lower mortality in some life-threatening conditions, but they also have side effects that limit the duration of treatment and the dose used. Systemic lupus erythematosus (SLE) is a systemic autoimmune disease characterized by the involvement of numerous organs and systems and the production of autoantibodies. Most current treatments include the use of corticosteroids and immunosuppressive medications. Glucocorticoids in SLE have been classically used not only to induce remission or treat an acute situation but also as maintenance therapy. During the last decades, new approaches to managing SLE have emerged, but corticosteroids continue to be part of all therapeutic regimes. There is more and more evidence about the side effects related to the use (or abuse) of steroids and their relationship with the accrual damage. In this manuscript, we try to make a critical review of the published literature about the benefit and side effects/damage that can be attributed to the use of glucocorticoids.

Juvenile idiopathic arthritis in the young adult. Methodology, objectives, and initial data from the JUVENSER registry.

OBJECTIVES: To describe the methodology, objectives, and initial data of the registry of young adult patients diagnosed with Juvenile Idiopathic Arthritis (JIA), JUVENSER. The main objective of the project is to know the sociodemographic and clinical characteristics, and disease activity of patients with JIA reaching the transition to adulthood. MATERIAL AND METHOD: Longitudinal, prospective, multicentre study, including patients between 16 and 25 years old, with a diagnosis of JIA in any of its categories. The main objective is to determine the characteristics and activity of JIA in the young adult. It includes sociodemographic variables, clinical variables, disease activity and joint damage rates, data on the use of health resources, and treatments used. The total duration of the project will be 3 years. A cohort of 534 young adult patients was obtained. CONCLUSIONS: The JUVENSER registry will constitute a cohort of young adults with JIA, which will allow the evaluation of the clinical characteristics and response to treatment of patients with disease onset in childhood, moving to adult clinics.

Metotrexato en artritis idiopática juvenil: efectos adversos y factores asociados / Methotrexate in juvenile idiopathic arthritis. Adverse effects and associated factors

INTRODUCCIÓN: El metotrexato (MTX) es el fármaco sistémico más utilizado en el tratamiento de pacientes con artritis idiopática juvenil. Su efectividad viene limitada por el desarrollo de efectos adversos (EA). PACIENTES Y MÉTODOS: Estudio observacional descriptivo retrospectivo de la frecuencia y tipo de EA asociados a MTX en pacientes con artritis idiopática juvenil seguidos en un hospital terciario en el periodo 2008-2016. RESULTADOS: Se estudió a 107 pacientes, 71/107 mujeres (66,3%) con edad al diagnóstico de 6,4 años (RIC 3,1-12,4) durante una mediana de seguimiento de 45,7 meses (RIC 28,8-92,4). El 44,9% (48 pacientes) tenía oligoartritis y el 24,3% (n = 26) poliartritis factor-reumatoide negativo. El 48,6% (52/107) desarrolló EA, siendo los más frecuentes los síntomas gastrointestinales y los trastornos conductuales (35,6% cada uno). La edad mayor de 6 años al inicio del tratamiento aumentaba el riesgo de desarrollar EA, tanto en el estudio univariable (OR = 3,5; IC95% 1,5-7,3) como en el multivariable (aumento del riesgo del 12% por año). La dosis, vía de administración o forma clínica no presentaban relación con el desarrollo de EA. Veinte niños precisaron cambio de dosis o vía de administración, resolviéndose el EA en 11 (55%). MTX se suspendió en 37/107 pacientes (34,6%) por EA, principalmente por hipertransaminasemia (n = 14; 37,8%), síntomas gastrointestinales (n = 9; 24,3%) y trastornos conductuales (n = 6; 16,3%). CONCLUSIONES: MTX es el tratamiento de elección de niños con artritis idiopática juvenil pero produce EA en prácticamente el 50% de los pacientes. Aunque estos EA no son graves, obligan a interrumpir el tratamiento en el 35%

INTRODUCTION: Methotrexate (MTX) is the drug of choice for juvenile idiopathic arthritis. Its clinical efficacy is limited due to the development of adverse effects (AEs). PATIENTS AND METHODS: A retrospective observational study was conducted on the AEs associated with MTX therapy in children diagnosed with juvenile idiopathic arthritis followed-up in a tertiary hospital between 2008 and 2016. RESULTS: The study included a total of 107 patients, of whom 71 (66.3%) were girls (66.3%). The median age at diagnosis was 6.4 years (IQR 3.1-12.4), with a median follow-up of 45.7 months (IQR 28.8-92.4). There were 48 patients (44.9%) with oligoarthritis, and 26 children (24.3%) with rheumatoid-factor negative polyarthritis. Of these, 52/107 (48.6%) developed AEs, with the most frequent being gastrointestinal symptoms (35.6%) and behavioural problems (35.6%). An age older than 6 years at the beginning of therapy increased the risk of developing AEs, both in the univariate (OR = 3.5; 95% CI: 1.5-7.3) and multivariate (12% increase per year) analyses. The doses used, administration route, or International League of Associations for Rheumatology (ILAR) classification, were not associated with the development of AEs. Twenty children required a dosage or route of administration modification, which resolved the AE in 11 (55%) cases. MTX was interrupted due to the development of AEs in 37/107 patients (34.6%), mainly due to increased plasma transaminases (n = 14, 37.8%), gastrointestinal symptoms (n = 9, 24.3%) and behavioural problems (n = 6, 16.3%). CONCLUSIONS: MTX is the therapy of choice for patients with juvenile idiopathic arthritis, but 50% of the children develop some form of AE. Although the AEs are not severe, they lead to interruption of therapy in 35% of the children

[Methotrexate in juvenile idiopathic arthritis. Adverse effects and associated factors]. / Metotrexato en artritis idiopática juvenil: efectos adversos y factores asociados.

INTRODUCTION: Methotrexate (MTX) is the drug of choice for juvenile idiopathic arthritis. Its clinical efficacy is limited due to the development of adverse effects (AEs). PATIENTS AND METHODS: A retrospective observational study was conducted on the AEs associated with MTX therapy in children diagnosed with juvenile idiopathic arthritis followed-up in a tertiary hospital between 2008 and 2016. RESULTS: The study included a total of 107 patients, of whom 71 (66.3%) were girls (66.3%). The median age at diagnosis was 6.4 years (IQR 3.1-12.4), with a median follow-up of 45.7 months (IQR 28.8-92.4). There were 48 patients (44.9%) with oligoarthritis, and 26 children (24.3%) with rheumatoid-factor negative polyarthritis. Of these, 52/107 (48.6%) developed AEs, with the most frequent being gastrointestinal symptoms (35.6%) and behavioural problems (35.6%). An age older than 6 years at the beginning of therapy increased the risk of developing AEs, both in the univariate (OR=3.5; 95% CI: 1.5-7.3) and multivariate (12% increase per year) analyses. The doses used, administration route, or International League of Associations for Rheumatology (ILAR) classification, were not associated with the development of AEs. Twenty children required a dosage or route of administration modification, which resolved the AE in 11 (55%) cases. MTX was interrupted due to the development of AEs in 37/107 patients (34.6%), mainly due to increased plasma transaminases (n=14, 37.8%), gastrointestinal symptoms (n=9, 24.3%) and behavioural problems (n=6, 16.3%). CONCLUSIONS: MTX is the therapy of choice for patients with juvenile idiopathic arthritis, but 50% of the children develop some form of AE. Although the AEs are not severe, they lead to interruption of therapy in 35% of the children.

Características del líquido sinovial en pacientes con artritis idiopática juvenil / Characteristics of synovial fluid in patients with juvenile idiopathic arthritis

Introducción: El análisis del líquido sinovial (LS) es una herramienta importante en el diagnóstico de pacientes con artritis idiopática juvenil (AIJ). Pacientes y métodos: Análisis retrospectivo de las características citológicas del LS obtenido de pacientes con AIJ en el periodo 2008-2016. Resultados: Se analizaron 102 LS de 59 pacientes. El 66% fueron mujeres y la forma clínica más frecuente fue la AIJ oligoarticular persistente (52,5%). La mediana de edad al inicio fue de 5 años (RIC 2,4-11,8). El LS generalmente era de características inflamatorias (mediana leucocitos 11.757/mm3; RIC 4.543-18.800) con predominio de polimorfonucleares (PMN, 61%; RIC 30-75). Ocho pacientes (14%) presentaron recuentos inferiores a 2.000 cél/mm3, con predominio de mononucleares (80%), mientras que 3 pacientes (5%) presentaron recuentos superiores a 50.000cél/mm3, con predominio de PMN (90%). No se encontraron diferencias en los recuentos celulares entre las distintas formas de AIJ. La mediana del recuento de leucocitos de pacientes positivos para ANA fue un 20% inferior a la de niños negativos para ANA (9.340 vs. 11.600/mm3; p = 0,23). La proporción de PMN en LS tendía a aumentar conforme se incrementaba la VSG (p < 0,001) y/o la PCR (p = 0,03). No existe correlación del índice JADAS-10 con el recuento en LS (p = 0,4). El LS en artrocentesis simultáneas de diferentes articulaciones mostró una correlación significativa (p = 0,001). Conclusiones: El LS de pacientes con AIJ generalmente tiene características inflamatorias, aunque un 19% presentó recuentos inferiores a 2.000 o superiores a 50.000cél/mm3. Los recuentos en pacientes positivos para ANA tendían a ser menores que en los negativos para ANA (no significativo). La proporción de PMN aumentaba con los reactantes

Introduction: Synovial fluid (SF) analysis is an important tool for the diagnosis of patients with juvenile idiopathic arthritis (JIA). Patients and methods: A retrospective analysis was carried out of cytological features of SF samples obtained from patients with JIA during the period 2008-2016. Results: A total of 102 SF samples from 59 patients were analysed. JIA was more common in females (66%). The most frequent form was persistent oligoarticular JIA (52.5%). The median age at onset was 5 years (IQR 2.4-11.8). SF usually showed an inflammatory pattern (median white blood cells count 11,757/ mm3; IQR 4,543-18,800), with a predominance of polymorphonuclear (PMN) cells (61%; IQR 30-75). Eight patients (14%) had white blood cells counts of less than 2,000 cells/mm3, with predominance of mononuclear cells (80%), whereas 3 patients (5%) had white blood cells counts higher than 50,000 cells/mm3, with a predominance of PMN cells (90%). Synovial white blood cells count did not show significant differences among the different forms of JIA. The median synovial white blood cells count in ANA-positive patients was 20% lower than in ANA-negative (9,340 vs. 11,600/mm3; P = .23). The proportion of PMN increased with increasing levels of ESR (P < .001) and/or CRP (P = .03). No significant correlation was found between JADAS-10 and synovial white blood cells count (P = .4). SF obtained from different joints in simultaneous arthrocentesis showed a significant correlation P = .001). Conclusion: SF from JIA patients usually had inflammatory characteristics, although 19% of the patients showed white blood cells counts below 2,000cells/mm3 or higher than 50,000 cells/mm3. SF cell count was non-significantly lower in ANA-positive patients, and the proportion of PMN increased with increasing levels of ESR/CRP

Enfermedad de Still del adulto asociada a neoplasia gástrica / Adult-onset Still's disease associated with gastric cancer

Resumen El diagnóstico de enfermedad de Still del adulto requiere la exclusión de cuadros infecciosos, tumorales y autoinmunes. Sin embargo, un proceso neoplásico poco expresivo clínicamente y en las pruebas complementarias puede pasar desapercibido al diagnóstico o comenzar con posterioridad, habiéndose descrito numerosos casos de enfermedad de Still asociada a tumores. Presentamos el caso de una paciente de 84 años con diagnóstico previo de enfermedad de Still del adulto, que desarrolló un tumor gástrico de evolución fatal 2 años después del diagnóstico del cuadro reumatológico.

Abstract The diagnosis of Adult-onset Still's disease (AOSD) requires the exclusion of infectious, malignant, and autoimmune diseases. However, a poorly symptomatic neoplastic process can easily be overlooked, or even onset later during the course of the disease. Therefore, numerous cases of Adult-onset Still's disease associated with malignancy have been reported. The case is reported of an 84-year old woman with previous diagnosis of AOSD who developed a gastric tumour with fatal outcome 2 years after the diagnosis of her rheumatic disease.

[Characteristics of synovial fluid in patients with juvenile idiopathic arthritis]. / Características del líquido sinovial en pacientes con artritis idiopática juvenil.

INTRODUCTION: Synovial fluid (SF) analysis is an important tool for the diagnosis of patients with juvenile idiopathic arthritis (JIA). PATIENTS AND METHODS: A retrospective analysis was carried out of cytological features of SF samples obtained from patients with JIA during the period 2008-2016. RESULTS: A total of 102 SF samples from 59 patients were analysed. JIA was more common in females (66%). The most frequent form was persistent oligoarticular JIA (52.5%). The median age at onset was 5 years (IQR 2.4-11.8). SF usually showed an inflammatory pattern (median white blood cells count 11,757/mm3; IQR 4,543-18,800), with a predominance of polymorphonuclear (PMN) cells (61%; IQR 30-75). Eight patients (14%) had white blood cells counts of less than 2,000 cells/mm3, with predominance of mononuclear cells (80%), whereas 3 patients (5%) had white blood cells counts higher than 50,000 cells/mm3, with a predominance of PMN cells (90%). Synovial white blood cells count did not show significant differences among the different forms of JIA. The median synovial white blood cells count in ANA-positive patients was 20% lower than in ANA-negative (9,340 vs. 11,600/mm3; P=.23). The proportion of PMN increased with increasing levels of ESR (P<.001) and/or CRP (P=.03). No significant correlation was found between JADAS-10 and synovial white blood cells count (P=.4). SF obtained from different joints in simultaneous arthrocentesis showed a significant correlation P=.001). CONCLUSION: SF from JIA patients usually had inflammatory characteristics, although 19% of the patients showed white blood cells counts below 2,000cells/mm3 or higher than 50,000cells/mm3. SF cell count was non-significantly lower in ANA-positive patients, and the proportion of PMN increased with increasing levels of ESR/CRP.

Uso de rituximab para trombocitopenia refractaria en pacientes con anticuerpos antifosfolípidos / Rituximab therapy for refractory thrombocytopenia in patients with antiphospholipid antibodies

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Osteomielitis crónica no bacteriana: experiencia en un hospital terciario / Non-bacterial chronic osteomyelitis: Experience in a tertiary hospital

INTRODUCCIÓN: La osteítis crónica no bacteriana (OCNB) es una enfermedad autoinflamatoria que cursa con brotes de inflamación ósea en ausencia de aislamiento microbiológico. Su diagnóstico es de exclusión. El tratamiento se basaba en la utilización de antiinflamatorios no esteroideos (AINE) y esteroideos aunque cada vez con mayor frecuencia se utilizan bifosfonatos o fármacos contra el factor de necrosis tumoral α (anti-TNFα) con buenos resultados. El objetivo es revisar nuestra experiencia en el diagnóstico y tratamiento de estos pacientes. PACIENTES Y MÉTODOS: Revisión retrospectiva de las historias clínicas de los pacientes diagnosticados de OCNB entre 2008 y 2015 en un hospital terciario. RESULTADOS: De un total de 7 pacientes, 4 eran mujeres, con una mediana de edad de 10 años (RIQ 2). El motivo más frecuente de consulta fue dolor que interfería con el sueño en 5 pacientes. Seis presentaron lesiones multifocales al diagnóstico. En 6 se realizó biopsia ósea que demostró un infiltrado neutrofílico o linfocitario y esclerosis. Cuatro pacientes recibieron tratamiento antibiótico y AINE sin respuesta clínica. Cinco pacientes recibieron prednisona, consiguiéndose control sintomático que solo mantuvo uno tras su suspensión. Cinco recibieron bifosfonatos con remisión de la enfermedad en 3. Dos pacientes presentaron una respuesta insuficiente a pamidronato, por lo que recibieron terapia anti-TNFα (etanercept, infliximab o adalimumab) y se mantienen asintomáticos en la actualidad. CONCLUSIONES: Nuestra serie, aunque limitada, confirma la efectividad y seguridad de la terapia con bifosfonatos y fármacos biológicos en pacientes con OCNB

INTRODUCTION: Non-bacterial chronic osteomyelitis (NBCO) is an autoinflammatory disease that presents with recurrent bouts of bone inflammation in the absence of microbiological isolation. It is a diagnosis of exclusion. Its treatment was classically based on the use of non-steroidal anti-inflammatory drugs (NSAIDs) and corticosteroids, although nowadays bisphosphonates or anti-tumour necrosis factor-α (anti-TNF) drugs are frequently used with good results. The objective of the study is to describe our experience in the diagnosis and treatment of patients with NBCO. PATIENTS AND METHODS: Retrospective chart review of patients with NBCO followed up in a tertiary centre between 2008 and 2015. RESULTS: A total of 7 patients with NBCO were recorded. Four were female and the median age was 10 years (IQR 2). The most common complaint was pain that interfered with sleep in 5 of the patients. Six patients had multifocal lesions at diagnosis. Bone biopsy demonstrated neutrophilic or lymphocytic infiltration and sclerosis in 6 patients. Four patients received antibiotics and NSAIDs without clinical response. Five received a short course of prednisone with an adequate control of symptoms, but only one of them maintained remission after corticosteroid suspension. Five patients received bisphosphonates with disease remission in 3 of them. The other 2 showed an inadequate response to pamidronate and were started on anti-TNF therapy (etanercept, infliximab or adalimumab), remaining asymptomatic at present. CONCLUSIONS: Our series, although limited, confirms the effectiveness and safety of bisphosphonate and anti-TNF therapy for children with NBCO

Subcutaneous Immunoglobulin in Refractory Juvenile Dermatomyositis.

Juvenile dermatomyositis (JDM) is the most common form of juvenile idiopathic inflammatory myopathy. We report a child with steroid-dependent JDM refractory to hydroxychloroquine and subcutaneous methotrexate who experienced systemic reactions to intravenous immunoglobulin and was successfully treated with subcutaneous immunoglobulin. This form of therapy has been shown to be safe, has a very low rate of adverse effects, does not require hospital admission, reduces the number of missed school days, and decreases the costs associated with treatment.

[Non-bacterial chronic osteomyelitis: Experience in a tertiary hospital]. / Osteomielitis crónica no bacteriana: experiencia en un hospital terciario.

INTRODUCTION: Non-bacterial chronic osteomyelitis (NBCO) is an autoinflammatory disease that presents with recurrent bouts of bone inflammation in the absence of microbiological isolation. It is a diagnosis of exclusion. Its treatment was classically based on the use of non-steroidal anti-inflammatory drugs (NSAIDs) and corticosteroids, although nowadays bisphosphonates or anti-tumour necrosis factor-α (anti-TNF) drugs are frequently used with good results. The objective of the study is to describe our experience in the diagnosis and treatment of patients with NBCO. PATIENTS AND METHODS: Retrospective chart review of patients with NBCO followed up in a tertiary centre between 2008 and 2015. RESULTS: A total of 7 patients with NBCO were recorded. Four were female and the median age was 10 years (IQR 2). The most common complaint was pain that interfered with sleep in 5 of the patients. Six patients had multifocal lesions at diagnosis. Bone biopsy demonstrated neutrophilic or lymphocytic infiltration and sclerosis in 6 patients. Four patients received antibiotics and NSAIDs without clinical response. Five received a short course of prednisone with an adequate control of symptoms, but only one of them maintained remission after corticosteroid suspension. Five patients received bisphosphonates with disease remission in 3 of them. The other 2 showed an inadequate response to pamidronate and were started on anti-TNF therapy (etanercept, infliximab or adalimumab), remaining asymptomatic at present. CONCLUSIONS: Our series, although limited, confirms the effectiveness and safety of bisphosphonate and anti-TNF therapy for children with NBCO.