Clinical characteristics and risk factors associated with lymphoma in patients with systemic lupus erythematosus: a nationwide cohort study.

OBJECTIVES: To assess the characteristics and risk of lymphoma in a large cohort of patients with SLE. METHODS: A case-cohort analysis was performed within a dynamic cohort of SLE patients from the Spanish Society of Rheumatology Lupus Registry (RELESSER). Clinical and analytical features were compared between the lymphoma SLE group and the control SLE group using an independent-sample Student's t-test or Mann-Whitney test for continuous variables and the χ2 test for categorical variables with Fisher's exact test if necessary. The multivariate analysis was based on a generalized linear model. RESULTS: Twenty-one patients with SLE and lymphoma and 3965 non-lymphoma controls with SLE were studied. Most lymphomas were of B cell origin (n = 15/21), with diffuse large B cell lymphoma being the most frequent histological type (8/21, 38.1%). As in the general population, the risk of lymphoma in SLE was higher in male than in female patients and increased with age. In the lymphoma SLE group, bivariate analysis showed a significantly higher percentage of pericarditis, organic brain syndrome, seizures, vasculitis, haemolytic anaemia, splenomegaly, venous thrombosis and mean modified (excluding lymphoma) SLICC/ACR damage index. In contrast, renal involvement, positive anti-dsDNA, and antimalarials ever were less frequent. CONCLUSIONS: In this large multicentre Spanish cohort, we identified characteristics of SLE that are associated with a higher risk of lymphoma. Antimalarials were significantly negatively associated with risk of lymphoma in SLE patients. Nevertheless, further prospective studies are needed to clarify these findings.

Long-term survival of biological therapy in psoriatic arthritis: 18-year analysis of a cohort in a tertiary hospital.

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.

Estado actual de la reumatología en el sector sanitario público de Cataluña / Rheumatology Manpower in the Public System in Catalonia (Spain)

Objetivo: Determinar el estado actual de la reumatología en Cataluña, y actualizar la información respecto a los estudios previos. Material y métodos Diseño: Estudio observacional, descriptivo y transversal. Recogida de datos de enero a junio de 2017. Muestra: Reumatólogos que ejercen actividad asistencial pública en Cataluña. Se envió un cuestionario en línea recabando información tanto de forma individual como colectiva de los servicios de reumatología. Resultados: Se obtuvo información de 34 hospitales y de 109 reumatólogos: 39 varones y 70 mujeres; edad media: 47±9 años. El número de reumatólogos aumentó un 8% en los últimos 5 años. La obtención del título fue en 101 (92,7%) casos por vía MIR. Noventa y seis (88,1%) facultativos tenían dedicación completa. Un 50% de los reumatólogos ejercían además en el sector privado. La actividad asistencial suponía un 76% de la jornada laboral. Se constató que 24 (70%) servicios de reumatología dependían jerárquicamente de otra especialidad. En 6 hospitales de Cataluña no disponen de reumatólogo. Conclusiones: El número de reumatólogos aumentó respecto hace 5 años. La edad media de los reumatólogos es avanzada y existe un claro predominio femenino en la especialidad. Un gran número de unidades de reumatología dependen de otros servicios. La actividad reumatológica se centra en Barcelona.(AU)

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.(AU)

Rheumatology manpower in the public system in Catalonia (Spain).

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.

Relevance of gastrointestinal manifestations in a large Spanish cohort of patients with systemic lupus erythematosus: what do we know?

OBJECTIVE: SLE can affect any part of the gastrointestinal (GI) tract. GI symptoms are reported to occur in >50% of SLE patients. To describe the GI manifestations of SLE in the RELESSER (Registry of SLE Patients of the Spanish Society of Rheumatology) cohort and to determine whether these are associated with a more severe disease, damage accrual and a worse prognosis. METHODS: We conducted a nationwide, retrospective, multicentre, cross-sectional cohort study of 3658 SLE patients who fulfil ≥4 ACR-97 criteria. Data on demographics, disease characteristics, activity (SLEDAI-2K or BILAG), damage (SLICC/ACR/DI) and therapies were collected. Demographic and clinical characteristics were compared between lupus patients with and without GI damage to establish whether GI damage is associated with a more severe disease. RESULTS: From 3654 lupus patients, 3.7% developed GI damage. Patients in this group (group 1) were older, they had longer disease duration, and were more likely to have vasculitis, renal disease and serositis than patients without GI damage (group 2). Hospitalizations and mortality were significantly higher in group 1. Patients in group 1 had higher modified SDI (SLICC Damage Index). The presence of oral ulcers reduced the risk of developing damage in 33% of patients. CONCLUSION: Having GI damage is associated with a worse prognosis. Patients on a high dose of glucocorticoids are at higher risk of developing GI damage which reinforces the strategy of minimizing glucocorticoids. Oral ulcers appear to decrease the risk of GI damage.

Do all antiphospholipid antibodies confer the same risk for major organ involvement in systemic lupus erythematosus patients?

OBJECTIVES: We aimed to investigate the association between the different antiphospholipid antibodies (aPL) and both systemic lupus erythematosus (SLE) and antiphospholipid syndrome (APS) manifestations. METHODS: Patients from the RELESSER registry, a Spanish retrospective, cross-sectional, forty-five hospital registry of adult SLE patients, were included. RESULTS: Out of a total of 3,658 SLE patients, 1372 were aPL positive (555 of them fulfilled criteria for APS). All aPL types showed a negative association with cutaneous SLE manifestations. Lupus anticoagulant (LA) and anticardiolipin antibodies (aCL) were both associated with haematological, ophthalmological and neuropsychiatric manifestations. IgG isotypes were associated with a higher risk of lupus manifestations compared with IgM. We found that the risk of neuropsychiatric and ophthalmological manifestations significantly increased with a higher number of positive aPL whereas the risk of cutaneous symptoms showed a negative correlation. All types of aPL, and more strongly LA, were associated with non-criteria antiphospholipid syndrome (APS) manifestations such as thrombocytopenia and haemolytic anaemia. Moreover, LA and aCL (particularly IgG isotype) were also associated with Libman-Sacks endocarditis and cognitive impairment. This association was stronger with more than one positive aPL. All types of aPL were also associated with classic APS manifestations, although LA, IgG isotypes, and patients with more than one aPL displayed a higher risk. CONCLUSIONS: There is a hierarchy for aPL and the risk of APS and SLE manifestations. aCL, and especially LA, confer a higher risk for major organ involvement in SLE. IgG isotypes seem to have a more important role. The load of aPL confer a higher risk for APS and certain SLE manifestations.

Desarrollo de una aplicación para teléfonos móviles (app) basada en la colaboración Sociedad Española de Reumatología/Sociedad Española de Medicina de Familia y Comunitaria para derivación de enfermedades autoinmunes sistémicas / Development of an application for mobile phones (App) based on the collaboration between the Spanish Society of Rheumatology and Spanish Society of Family Medicine for the referral of systemic autoimmune diseases from primary care to rheumatology

El diagnóstico y tratamiento de las enfermedades autoinmunes sistémicas (EAS) constituye un reto. Aunque infrecuentes, afectan a cientos de miles de pacientes en España. El médico de familia (MF) se enfrenta a síntomas o signos inespecíficos que hacen sospechar EAS al inicio del proceso, y tiene que decidir a quiénes debería derivar. Para facilitar su reconocimiento y mejorar su derivación, expertos de la Sociedad Española de Medicina de Familia y Comunitaria y de la Sociedad Española de Reumatología seleccionaron 26 síntomas/signos-guía y alteraciones analíticas. Se escogieron parejas de MF y reumatólogo para elaborar algoritmos diagnósticos y de derivación. Posteriormente se revisaron y adaptaron al formato de aplicación para móviles (app) descargable. El resultado es el presente documento de derivación de EAS para MF en formato de papel y app. Contiene algoritmos de fácil manejo utilizando datos de la anamnesis, exploración física y pruebas analíticas accesibles en atención primaria para orientar el diagnóstico y facilitar la derivación a reumatología o a otras especialidades

Management of systemic autoimmune diseases is challenging for physicians in their clinical practice. Although not common, they affect thousands of patients in Spain. The family doctor faces patients with symptoms and non-specific cutaneous, mucous, joint, vascular signs or abnormal laboratory findings at the start of the disease process and has to determine when to refer patients to the specialist. To aid in disease detection and better referral, the Spanish Society of Rheumatology and the Spanish Society of Family Medicine has created a group of experts who selected 26 symptoms, key signs and abnormal laboratory findings which were organized by organ and apparatus. Family doctors and rheumatologists with an interest in autoimmune systemic diseases were selected and formed mixed groups of two that then elaborated algorithms for diagnostic guidelines and referral. The algorithms were then reviewed, homogenized and adapted to the algorithm format and application for cell phone (apps) download. The result is the current Referral document of systemic autoimmune diseases for the family doctor in paper format and app (download). It contains easy-to-use algorithms using data from anamnesis, physical examination and laboratory results usually available to primary care, that help diagnose and refer patients to rheumatology or other specialties if needed

Antiphospholipid syndrome (APS) in patients with systemic lupus erythematosus (SLE) implies a more severe disease with more damage accrual and higher mortality.

INTRODUCTION: Antiphospholipid antibodies (aPL) have been associated with organ damage and certain features in systemic lupus erythematosus(SLE) patients. Our aim was to investigate the differences between SLE patients according to the presence of aPL and/or clinical antiphospholipid syndrome (APS). MATERIALS AND METHODS: Patients from the RELESSER-T registry were included. RELESSER-T is a Spanish multicenter, hospital-based, retrospective, SLE registry. RESULTS: We included 2398 SLE patients, 1372 of whom were positive for aPL. Overall 1026 patients were classified as SLE, 555 as SLE-APS and817 as SLE-aPL. Regarding cardiovascular risk factors, SLE-APS patients had higher rates of hypertension, dyslipidemia and diabetes than those with SLE-aPL and SLE (p < 0.001). SLE-APS patients showed higher rates of neuropsychiatric, cardiac, pulmonary, renal and ophthalmological manifestations than the other groups (p < 0.001). SLE-APS patients presented greater damage accrual with higher SLICC values (1.9 ± 2.2 in SLE-APS, 0.9 ± 1.4 in SLE-aPL and 1.1 ± 1.6 in SLE, p < 0.001) and more severe disease as defined by the Katz index (3 ± 1.8 in SLE-APS, 2.7 ± 1.7 in SLE-aPL and 2.6 ± 1.6 in SLE, p < 0.001). SLE-APS patients showed higher mortality rates (p < 0.001). CONCLUSIONS: SLE-APS patients exhibited more severe clinical profiles with higher frequencies of major organ involvement, greater damage accrual and higher mortality than SLE-aPL and SLE patients.

Rheumatology Manpower in the Public System in Catalonia (Spain). / Estado actual de la reumatología en el sector sanitario público de Cataluña.

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.

Associated factors to serious infections in a large cohort of juvenile-onset systemic lupus erythematosus from Lupus Registry (RELESSER).

OBJECTIVE: To assess the incidence of serious infection (SI) and associated factors in a large juvenile-onset systemic lupus erythematosus (jSLE) retrospective cohort. METHODS: All patients in the Spanish Rheumatology Society Lupus Registry (RELESSER) who meet ≥4 ACR-97 SLE criteria and disease onset <18 years old (jSLE), were retrospectively investigated for SI (defined as either the need for hospitalization with antibacterial therapy for a potentially fatal infection or death caused by the infection). Standardized SI rate was calculated per 100 patient years. Patients with and without SI were compared. Bivariate and multivariate logistic and Cox regression models were built to calculate associated factors to SI and relative risks. RESULTS: A total of 353 jSLE patients were included: 88.7% female, 14.3 years (± 2.9) of age at diagnosis, 16.0 years (± 9.3) of disease duration and 31.5 years (±10.5) at end of follow-up. A total of 104 (29.5%) patients suffered 205 SI (1, 55.8%; 2-5, 38.4%; and ≥6, 5.8%). Incidence rate was 3.7 (95%CI: 3.2-4.2) SI per 100 patient years. Respiratory location and bacterial infections were the most frequent. Higher number of SLE classification criteria, SLICC/ACR DI score and immunosuppressants use were associated to the presence of SI. Associated factors to shorter time to first infection were higher number of SLE criteria, splenectomy and immunosuppressants use. CONCLUSIONS: The risk of SI in jSLE patients is significant and higher than aSLE. It is associated to higher number of SLE criteria, damage accrual, some immunosuppressants and splenectomy.

Tocilizumab in giant cell arteritis: differences between the GiACTA trial and a multicentre series of patients from the clinical practice.

OBJECTIVES: A potential point of concern among clinicians is whether results derived from the clinical trials can be reasonably applied or generalised to a definable group of patients seen in real world. It can be the case of the GiACTA study that is a phase III randomised controlled trial of tocilizumab (TCZ) in giant cell arteritis (GCA). To address this question, we compared the clinical features and the responses to TCZ from the GiACTA trial patients with those from a series of GCA seen in the daily clinical practice. METHODS: Comparative study of clinical features between patients from the GiACTA trial (overall n=251) and those from a multicentre series of real-world GCA patients undergoing TCZ therapy (n=134). The diagnosis of GCA in the GiACTA trial was established by the ACR modified criteria whereas in the series of real-world patients it was made by using the ACR criteria, a positive biopsy of temporal artery or the presence of imaging techniques consistent with large-vessel vasculitis in individuals who presented cranial symptoms of GCA. GiACTA trial patients received subcutaneous TCZ (162 mg every 1 or 2 weeks) whereas those from the clinical practice series were treated using standard IV dose (8 mg/kg/month) or subcutaneous (162 mg/week). RESULTS: Real-life patients undergoing TCZ were older with longer disease duration and higher values of ESR and had received conventional immunosuppressive therapy (mainly methotrexate) more commonly than those included in the GiACTA trial. Despite clinical differences, TCZ was equally effective in both GiACTA trial and clinical practice patients. However, serious infections were more commonly observed in GCA patients recruited from the clinical practice. CONCLUSIONS: Despite clinical differences with patients recruited in clinical trials, data from real-life patients confirm the efficacy of TCZ in GCA.

La voz pasiva de la fibromialgia / Passive voice of fibromyalgia

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