Thorough assessment of the effectiveness of belimumab in a large Spanish multicenter cohort of systemic lupus erythematosus patients.

OBJECTIVES: To provide an overview on the current use of belimumab (BLM) in SLE patients in clinical practice and to examine its efficacy in terms of standardized outcomes, drug survival, as well as patient and safety profiles. METHODS: A longitudinal retrospective multicentre cohort including SLE patients treated with BLM at 18 Spanish centers. Data was collected upon initiation of BLM, at 6 and 12 months after initiation, and at the last recorded visit. Changes in SLEDAI-2K, the proportion of patients who achieved LLDAS and DORIS 2021, and number of flares were compared between visits. Changes in damage, glucocorticoids use and employment status pre-BLM and post-BLM were also assessed. RESULTS: A total of 324 patients were included with a mean follow-up of 3.8 (±2.7) years. LLDAS was attained by 45.8%, 62% and 71% of patients, and DORIS by 24%, 36.2% and 52.5% on successive visits, respectively. Twenty-seven-point two percent of patients were in DORIS ≥ 50% of the visits and a 46% in LLDAS-50. Flares and number of flares were significantly lower one year after treatment with BLM and no changes in damage accrual were observed. Mean (±SD) prednisone dose was significantly reduced over time, with 70 (24%) patients discontinuing GC. CONCLUSION: Our study not only demonstrates belimumab´s efficacy in attaining treat-to-target goals in SLE patients, but also confirms its GC-sparing effect, and its prevention of flares and organ damage accrual.

Rheumatoid meningitis: a case series report and review of modern therapeutic schemes and outcome.

INTRODUCTION: Rheumatoid meningitis (RM) is an extremely rare extra-articular complication of rheumatoid arthritis (RA), with approximately 165 cases reported world-wide. RM exhibits a broad range of symptoms, with stroke-like episodes and seizures being the most common manifestations. The primary differential diagnoses include vascular and infectious diseases. The influence of immunomodulatory medications on the pathophysiology of RM remains unclear. There are no consensus guidelines on therapeutic regimen. METHODS: We present four patients with prior history of RA that developed different neurological syndromes in correlation to radiological leptomeningitis. Clinical presentations, comorbid conditions, supplementary diagnostic assessments, treatments, and prognosis are provided. A literature review of recent immunosuppressive management in RM patients was performed. RESULTS: Three patients presented to hospital with recurrent focal seizures. Only two suffered meningism, reporting headache and fever. Magnetic resonance imaging (MRI) showed different grades of leptomeningitis across all cases. Notably, three cases demonstrated bilateral involvement extending to the pachymeninges. Two patients exhibited pronounced CSF mononuclear inflammation while extended microbiological evaluations yielded negative results. Two patients required biopsy for confirmation. The initiation of immunosuppressive therapy marked a turning point for three patients who previously exhibited progressive deterioration. Mortality was absent in all cases. CONCLUSIONS: Our experience remarks the elusive nature of RM. Rigorous exclusionary diagnostics are imperative to differentiate RM from mimicking conditions. Clinical manifestations oscillate between transient episodes and progressive neurological impairments, punctuated by frequent epileptic seizures. In scenarios where clinical worsening persists or where clinical and radiological evaluations are inconclusive, aggressive immunosuppressive therapy is recommended.

Opioids and fibromyalgia: frequency of use and factors associated with increased consumption in patients remitted to a tertiary care center.

BACKGROUND: Opioids are not recommended for fibromyalgia. OBJECTIVE: To investigate the frequency of opioid use in a large cohort of fibromyalgia patients and to identify factors associated with opioid consumption. METHODS: A retrospective, observational study of a large fibromyalgia cohort in a tertiary care center. We assessed fibromyalgia severity, functional capacity, anxiety, depression, drugs consumption and the patient's impression of change. We compared strong opioid consumers (SOC) and non-SOC. Inferential statistical and logistic regression analysis were used to identify factors associated with opioid consumption, and ANOVA for repeated measurements. RESULTS: We found a prevalence of 9.2% of SOC (100 patients) among 1087 patients in the cohort. During the last four years there was a significant increase on the incidence of SOC up to 12.8% (p = 0.004). There were no differences in demographic variables between SOC and non-SOC. Clinical variables were significantly more severe in SOC, and they consumed more non-opioid drugs (p < 0.0001). Opioid consumption was independently associated with other non-opioid drugs (Odds ratio 1.25, CI: 1.13-1.38), but not with the fibromyalgia severity. At three months, 62% of the patients had opioid withdrawal. There were no statistical differences in the fibromyalgia severity at the initial evaluation, or the patient's impression of change compared with those patients who continued opioids. Coping strategies were better in those patients who withdrew opioids (p = 0.044). CONCLUSIONS: We observed an increase in opioid prescriptions during the last four years. Opioid consumption was associated with concomitant use of non-opioid drugs, but it was not associated with fibromyalgia severity.

Damage in a large systemic lupus erythematosus cohort from the Spanish Society of Rheumatology Lupus Registry (RELESSER) with emphasis on the cardiovascular system: a longitudinal analysis.

OBJECTIVE: To assess organ damage, with emphasis on the cardiovascular system, over the different stages of the disease in a large SLE cohort. METHODS: Multicentre, longitudinal study of a cohort of 4219 patients with SLE enrolled in the Spanish Society of Rheumatology Lupus Registry. Organ damage was ascertained using the Systemic Lupus International Collaborating Clinics/American College of Rheumatology Damage Index (SDI). We longitudinally analysed SDI (globally and for each domain) over time only in the 1274 patients whose dates of damage events had been recorded. RESULTS: During the first year after diagnosis of SLE, 20% of the 1274 patients presented with new damage manifestations. At years 2 and 3, new damage was recorded in 11% and 9% of patients. The annual percentage of patients with new damage after year 5 decreased to 5%. In the first year with the disease, most damage was accumulated in the musculoskeletal, neuropsychiatric and renal systems; in later stages, most damage was in the musculoskeletal, ocular and cardiovascular systems. Considering 'cerebrovascular accident' and 'claudication for 6 months' as cardiovascular items, the cardiovascular system was the second most affected system during the early stages of SLE, with 19% of the patients who presented with damage affected at first year after diagnosis. During the late stages, 20-25% of the patients presenting with new damage did so in this modified cardiovascular domain of the SDI. CONCLUSIONS: New damage occurs mainly during the first year following diagnosis of SLE. Cardiovascular damage is relevant in both the early and the late stages of the disease. Strategies to prevent cardiovascular damage should be implemented early after diagnosis of SLE.

SLESIS-R: an improved score for prediction of serious infection in patients with systemic lupus erythematosus based on the RELESSER prospective cohort.

OBJECTIVE: To develop an improved score for prediction of severe infection in patients with systemic lupus erythematosus (SLE), namely, the SLE Severe Infection Score-Revised (SLESIS-R) and to validate it in a large multicentre lupus cohort. METHODS: We used data from the prospective phase of RELESSER (RELESSER-PROS), the SLE register of the Spanish Society of Rheumatology. A multivariable logistic model was constructed taking into account the variables already forming the SLESIS score, plus all other potential predictors identified in a literature review. Performance was analysed using the C-statistic and the area under the receiver operating characteristic curve (AUROC). Internal validation was carried out using a 100-sample bootstrapping procedure. ORs were transformed into score items, and the AUROC was used to determine performance. RESULTS: A total of 1459 patients who had completed 1 year of follow-up were included in the development cohort (mean age, 49±13 years; 90% women). Twenty-five (1.7%) had experienced ≥1 severe infection. According to the adjusted multivariate model, severe infection could be predicted from four variables: age (years) ≥60, previous SLE-related hospitalisation, previous serious infection and glucocorticoid dose. A score was built from the best model, taking values from 0 to 17. The AUROC was 0.861 (0.777-0.946). The cut-off chosen was ≥6, which exhibited an accuracy of 85.9% and a positive likelihood ratio of 5.48. CONCLUSIONS: SLESIS-R is an accurate and feasible instrument for predicting infections in patients with SLE. SLESIS-R could help to make informed decisions on the use of immunosuppressants and the implementation of preventive measures.

Importancia de la planificación familiar en pacientes con enfermedades inflamatorias inmunomediadas: un abordaje multidisciplinar / Importance of family planning in patients with immune-mediated inflammatory diseases: a multidisciplinary approach

Antecedentes y objetivo: El reto terapéutico que supone para los equipos asistenciales la planificación familiar en mujeres con enfermedades inflamatorias inmunomediadas remarca la necesidad de armonizar la evidencia disponible para proporcionar a las pacientes información objetiva y consensuada. Este artículo refleja el trabajo realizado por un equipo multidisciplinar de revisión de la evidencia científica disponible y la estrategia de actuación consensuada en la planificación familiar, embarazo, posparto y lactancia materna de pacientes con enfermedades inflamatorias inmunomediadas. Métodos: Se realizó una búsqueda bibliográfica, se estructuró la información a lo largo de las diferentes etapas (preconcepción, embarazo, posparto y lactancia materna) y se realizó una reunión presencial para consensuar dicha información en la que participaron tanto pacientes como profesionales de la salud. Resultados: Se desarrollaron materiales específicos incluidos en este trabajo y que pueden servir de guía en la toma de decisiones consensuada entre pacientes y profesionales de la salud. Conclusión: Estos materiales responden a la necesidad de que los profesionales responsables del manejo de pacientes con enfermedades inflamatorias inmunomediadas posean información validada y actualizada sobre las actuaciones y el uso de fármacos indicados para estas enfermedades.(AU)

Background and objectives: Family planning in women with immune-mediated inflammatory diseases is a challenge for healthcare teams, highlighting the need for standardized available evidence to provide patients with objective and agreed information. This study reflects the work performed by a multidisciplinary team in reviewing available scientific evidence, and the strategy agreed for family planning, pregnancy, postpartum, and breastfeeding in patients with immune-mediated inflammatory diseases. Methods: A literature search was conducted, information was structured across the different stages (preconception, pregnancy, postpartum and breastfeeding), and an on-site meeting was convened, in which patients and healthcare providers participated. Results: Specific materials, which are included in this work, were developed to guide clinical decisions to be agreed upon by patients and healthcare providers. Conclusion: These materials meet the need for validated and updated information on the approach and use of indicated drugs for professionals responsible for the management of immune-mediated inflammatory diseases.(AU)

Impacto económico asociado a eventos obstétricos en mujeres en edad fértil con artritis psoriásica, artritis reumatoide, espondiloartritis axial y psoriasis en España / Economic Impact of Obstetric Events on Women of Reproductive Age Living With Psoriatic Arthritis, Rheumatoid Arthritis, Axial Spondyloarthritis and Psoriasis in Spain

Objetivo: Estimar el coste anual asociado a eventos obstétricos que pueden experimentar mujeres en edad fértil con enfermedades inflamatorias inmunomediadas, desde la perspectiva del Sistema Nacional de Salud. Métodos: Se desarrolló un análisis de costes para estimar el impacto anual por paciente asociado a eventos obstétricos en mujeres en edad reproductiva con artritis psoriásica (APs), artritis reumatoide (AR), espondiloartritis axial (EspAax) y psoriasis (PsO). Se consideraron eventos durante la fertilidad, la concepción, el embarazo y el posparto. Todos los parámetros fueron validados y consensuados por un panel multidisciplinar de expertos. Los costes unitarios (€, 2019) se obtuvieron de bases de datos nacionales. Resultados: Durante la fertilidad y concepción, se estimó un coste anual/paciente de 229€ para una consulta preconcepcional en pacientes con PsO, de 3.642€ en pacientes con APs, AR y EspAax y de 4.339€ para reproducción asistida. En el embarazo, el coste anual/paciente fue de 1.214€ para un aborto espontáneo en el 1.er trimestre, 4.419€ para un aborto tardío en el 2.o trimestre, 11.260€ para preeclampsia, 3.188€ para crecimiento intrauterino retardado y 12.131€ para amenaza de parto prematuro. En el posparto, se estimó un coste anual/paciente de 120.364, 44.709 y 5.507€ para prematuridad de<28, 28-32 y 33-37 semanas, respectivamente. Conclusiones: Este análisis facilita información sobre la carga económica de los eventos en mujeres en edad reproductiva con APs, AR, EspAax y PsO. Una adecuada planificación junto con la individualización del manejo, el tratamiento y la monitorización adicional pueden reducir el riesgo y la carga de estos eventos.(AU)

Objective: To estimate the annual cost associated with obstetric events in women of reproductive age with immune-mediated inflammatory diseases, from the perspective of the National Healthcare System. Methods: A cost-analysis was developed to estimate the impact associated with obstetric events in women of reproductive age with psoriasis (PSO), psoriatic arthritis (PsA), rheumatoid arthritis (RA) and axial spondyloarthritis (axSpA). The analysis considered complications during fertility and conception, in pregnancy and in the postpartum. All parameters were validated and agreed by a multidisciplinary expert panel. Unitary costs (€, 2019) were obtained from national, local databases. Results: During fertility and conception, an annual cost per patient of €229 was estimated for a preconception consultation in a patient with PSO, of €3,642 for a preconception consultation in patients with PsA, RA and axSpA and €4,339 for assisted reproduction. Women with complications in pregnancy had an annual cost per patient of €1,214 for a miscarriage in the first trimester, €4,419 for a late miscarriage in the second trimester, €11,260 for preeclampsia €3,188 for restricted intrauterine growth and €12,131 for threat of premature delivery. In the postpartum, an annual cost per patient of €120,364, €44,709, and €5,507 were estimated associated with admissions to neonatology of premature infants of <28, 28-32 and 33-37 weeks, respectively. Conclusions: This analysis provides insight on the economic burden of complications associated with women of reproductive age for immune-mediated diseases (PSO, PsA, RA, axSpA). Individualization of treatment, additional and close monitoring may reduce the risk and burden of these complications.(AU)

Impacto clínico de la capilaroscopia periungueal en la práctica clínica diaria / Clinical impact of nailfold capillaroscopy in daily clinical practice

Introducción: La capilaroscopia periungueal (CP) es útil en la evaluación del fenómeno de Raynaud tanto primario como secundario, y en el seguimiento de los pacientes con esclerosis sistémica. Nuestro estudio evalúa el impacto de la CP en el diagnóstico, en función del motivo de solicitud y el perfil de autoanticuerpos en la práctica clínica diaria. Material y métodos: Se incluyeron todos los pacientes con al menos una CP entre junio de 2012 y diciembre de 2017. Se revisaron las historias clínicas y se determinó de forma dicotómica (sí/no) si la CP contribuyó al diagnóstico realizado en la consulta posterior a la realización de la CP. Se recogieron datos demográficos, clínicos y de laboratorio, motivo de solicitud de la CP y su relación con los patrones CP. Resultados: De 530 pacientes con una primera CP, 266 se realizaron como estudio de un fenómeno de Raynaud. De estos, en 20 pacientes (3,8%) se realizó un diagnóstico de enfermedad del tejido conectivo en la consulta posterior a la CP; 15 fueron diagnosticados de esclerosis sistémica, 4 de conectivopatía indiferenciada y uno de enfermedad mixta. Salvo un paciente diagnosticado de conectivopatía indiferenciada, el resto tenía anticuerpos antinucleares positivos y 11 de ellos, además, anticuerpos específicos (9 anticentrómero, uno anti-Scl70 y otro, anti-RNPC). La positividad de anticuerpos antinucleares se asoció con una mayor probabilidad de presentar una CP de esclerodermia, y ningún paciente diagnosticado de una enfermedad reumática tras la CP tenía un patrón normal. Conclusión: La CP es una técnica útil, pero con impacto limitado en el diagnóstico de enfermedades del tejido conectivo. La positividad de los anticuerpos se relaciona con una mayor probabilidad de presentar patrones patológicos en la CP.(AU)

Introduction: Nailfold capillaroscopy (NC) is useful in the evaluation of Raynaud's phenomenon, associated with some connective tissue diseases and in the follow-up of patients with systemic sclerosis. Our study evaluates the impact of NC in the diagnosis, according to the reason for the request and profile of autoantibodies in daily clinical practice. Material and methods: All patients that undergone at least one NC between June 2012 and December 2017 were included. Clinical records were reviewed and analysed in a dichotomous way (yes/no), to see whether the NC contributed to a change of diagnosis in subsequent consultations. In addition, demographic, clinical and laboratory data were collected, and the relationship with NC patterns evaluated. Results: Of the 530 patients who had undergone at least one NC, 266 had Raynaud's phenomenon as primary indication for the technique. Of those, 20 patients (3.8%) had a diagnostic change in the post-NC consultation; 15 were diagnosed with systemic sclerosis, 4 with undifferentiated connective tissue disease and one with mixed connective tissue disease. All patients had, except for one patient diagnosed with undifferentiated connective tissue disease, positive antinuclear antibodies titres, 11 of them had disease specific antibodies (9 anti-centromere, one anti-Scl70 and other anti-RNPC). The positivity of antinuclear antibodies titres was associated with a higher probability of presenting a scleroderma pattern in the NC, and all patients with a specific rheumatological diagnosis had an abnormal NC. Conclusion: NC is a useful technique, but with limited impact in the diagnosis of connective tissue diseases. Autoantibody positivity is associated with a greater likelihood of presenting pathological NC patterns.(AU)

Una reflexión sobre el anticoagulante lúpico: cómo lo definimos, determinamos e interpretamos / A reflection on how we define, determine and interpret the finding of lupus anticoagulant

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Salivary gland ultrasound in clinical practice: What is its real usefulness? / Utilidad en práctica clínica de la ecografía de glándulas salivales

Background: Salivary gland ultrasound (SGU) provides information about structural gland abnormalities that can be graded and used for primary Sjögren's syndrome (pSS) diagnosis. Its potential role as a prognostic marker for detecting patients at high risk of lymphoma and extra-glandular manifestations is still under evaluation. We aim to assess the usefulness of SGU for SS diagnosis in routine clinical practice and its relationship with extra-glandular involvement and lymphoma risk in pSS patients. Methods: We designed a retrospective observational single-center study. Data was collected using the electronic health records of patients referred to an ultrasound outpatient clinic for evaluation over a 4-year period. Data extraction included demographics, comorbidities, clinical data, laboratory tests, SGU results, salivary gland (SG) biopsy, and scintigraphy results. Comparisons were made between patients with and without pathological SGU. The external criterion for comparison was the fulfillment of the 2016 ACR/EULAR pSS criteria. Results: A total of 179 SGU assessments were included from this 4-year period. Twenty-four cases (13.4%) were pathological. The most frequently diagnosed conditions prior to SGU-detected pathologies were pSS (9.7%), rheumatoid arthritis (RA) (13.1%), and systemic lupus (4.6%). One hundred and two patients (57%) had no previous diagnosis (sicca syndrome work-up); of these, 47 patients (46.1%) were ANA positive and 25 (24.5%) anti-SSA positive. In this study, the sensitivity and specificity of SGU for SS diagnosis were 48% and 98% respectively, with a positive predictive value of 95%. There were statistically significant relationships between a pathological SGU and the presence of recurrent parotitis (p=.0083), positive anti-SSB antibodies (p=.0083), and a positive sialography (p=.0351)...(AU)

Antecedentes y objetivo: La ecografía de glándulas salivales (EGS) proporciona información acerca de las anomalías en la estructura glandular, y puede ser utilizado para el diagnóstico del síndrome de Sjögren (SS). Además, su potencial valor pronóstico para detectar pacientes con riesgo de manifestaciones extra-glandulares, así como el riesgo de linfoma se encuentra aún bajo estudio. El objetivo de nuestro estudio es evaluar la utilidad de la EGS para el diagnóstico del SS en la práctica clínica habitual, y su relación con la afectación extra-glandular, así como el riesgo de linfoma en pacientes con síndrome de Sjögren primario (pSS). Métodos: Realizamos un estudio retrospectivo y observacional en un único centro. La información fue recolectada de la historia clínica electrónica del paciente tras un seguimiento de 4 años. Esta información incluye variables demográficas, comorbilidades, datos clínicos, análisis de laboratorio, los resultados de la EGS, biopsia de glándulas salivales y gammagrafía. Se efectuaron comparaciones entre los pacientes que tenían una EGS patológica con aquellos que tenían un resultado normal. El criterio para establecer la comparación fue cumplir los criterios de ACR/ELUAR 2016 para el diagnóstico de pSS. Resultados: Se realizaron un total de 179 EGS durante el período de 4 años. De estas, 24 (13,4%) resultaron ser patológicas. Las enfermedades más frecuentemente identificadas tras realizar la EGS fueron pSS (9,7%), artritis reumatoide (AR) (4,6%) y lupus eritematoso sistémico (LES) (4,6%). Ciento dos pacientes (57%) no tenían diagnóstico previo (estudio de síndrome seca); de estos, 47 (46,1%) tenían ANA positivo y 25 (24,5%) tenían anti-Ro positivo. La sensibilidad y la especificidad de la EGS para detectar el SS en nuestro estudio fueron del 48 y 98%, respectivamente; con un valor predictivo positivo del 95%...(AU)

Hechos y controversias en la enfermedad mixta del tejido conectivo / Facts and controversies in mixed connective tissue disease

La enfermedad mixta del tejido conectivo (EMTC) es una enfermedad reumática autoinmunitaria sistémica (ERAS) caracterizada por la asociación de manifestaciones clínicas de lupus eritematoso sistémico (LES), esclerosis sistémica cutánea (ESC) y polimiositis-dermatomiositis en presencia de títulos elevados de anticuerpos anti-U1-RNP en el suero de los pacientes. Sus principales síntomas son la poliartritis, el edema de manos, el fenómeno de Raynaud, la esclerodactilia, la miositis y la hipomotilidad esofágica. Actualmente, la mayoría de los autores acepta que la EMTC es una entidad independiente, pero algunos mantienen que estos pacientes podrían presentar una ERAS, definida en su fase precoz como LES o ESC, o ser, en realidad, un síndrome de solapamiento de la ERAS (AU)

Mixed connective tissue disease (MCTD) is a systemic autoimmune rheumatic disease (SARD) characterised by the combination of clinical manifestations of systemic lupus erythematosus (SLE), cutaneous systemic sclerosis (SSc) and polymyositis-dermatomyositis, in the presence of elevated titers of anti-U1-RNP antibodies. Main symptoms of the disease are polyarthritis, hand oedema, Raynaud's phenomenon, sclerodactyly, myositis and oesophageal hypomobility. Although widely discussed, most authors today accept MCTD as an independent entity. Others, however, suggest that these patients may belong to subgroups or early stages of certain definite connective diseases, such as SLE or SSc, or are, in fact, SARD overlap syndromes (AU)