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.

Incidence of refractory CMV infection with or without antiviral resistance in Spain: A systematic literature review.

INTRODUCTION: Solid organ transplantation (SOT) and hematopoietic stem cell transplantation (HSCT) recipients are susceptible to cytomegalovirus (CMV) infection. The incidence of refractoriness to antivirals, with or without resistance, is unclear. The purpose of this review was to describe the epidemiology of refractory CMV infection in Spain to understand the current unmet needs. METHODS: PubMed, EMBASE, Cochrane and MEDES were searched systematically for relevant articles. We included randomized controlled trials and observational studies published during the period from January 1990 to June 2021. RESULTS: From 212 screened records, we selected 19 papers including 1973 transplant recipients. Refractory infection ranged from 3 to 10% in studies with SOT recipients. The incidence of CMV resistance ranged from 1% to 36% in these patients. The incidence of CMV refractory infection in HSCT recipients ranged from 11 to 50%, while values for resistant infection ranged from 0% to 21%. CONCLUSION: The wide range of definitions and values observed does not allow us to establish the true incidence of refractory CMV infection with or without resistances in SOT and HSCT patients in Spain. This review highlights the gap between clinical practice and clinical trials' definitions which needed to be updated to be easier followed in current clinical practice.

Sonidegib as a Locally Advanced Basal Cell Carcinoma Therapy in Real-life Clinical Setting: A National Multicentre Study. / [Artículo traducido] Sonidegib para el tratamiento del carcinoma basocelular avanzado en práctica clínica real: estudio nacional multicéntrico.

BACKGROUND: Basal cell carcinoma (BCC) is the most prevalent cancer. A minority of BCCs have an aggressive behaviour (laBCC) and may require hedgehog pathway inhibitors such as sonidegib as its treatment. OBJECTIVE: To describe the use of sonidegib in a large number of patients and provide more data on its real-life efficacy and safety profile. METHODS: We conducted a retrospective and multicentric study that included patients treated with sonidegib. Epidemiological, effectiveness and safety data were collected. RESULTS: A total of 82 patients with a mean age of 73.9 years were included. Ten patients had Gorlin syndrome. Median treatment duration was 6 months. Median follow-up duration was 34.2 months. Globally, 81.7% of the patients showed clinical improvement (52.4% partial response and 29.3% complete response), 12.2% clinical stability and 6.1% disease progression. There was no statistically significant difference in clinical improvement between the 24 h and 48 h sonidegib posology. After 6 months of treatment, 48.8% of the patients discontinued sonidegib. Prior vismodegib treatment and recurrent primary BCC were associated with a poorer response to sonidegib. At 6 months of treatment, 68.3% of the patients experienced at least one adverse effect. CONCLUSION: Sonidegib shows good effectiveness and acceptable safety profile in usual clinical practice.

Sonidegib as a Locally Advanced Basal Cell Carcinoma Therapy in Real-life Clinical Setting: A National Multicentre Study.

BACKGROUND: Basal cell carcinoma (BCC) is the most prevalent cancer. A minority of BCCs have an aggressive behaviour (laBCC) and may require hedgehog pathway inhibitors such as sonidegib as its treatment. OBJECTIVE: To describe the use of sonidegib in a large number of patients and provide more data on its real-life efficacy and safety profile. METHODS: We conducted a retrospective and multicentric study that included patients treated with sonidegib. Epidemiological, effectiveness and safety data were collected. RESULTS: A total of 82 patients with a mean age of 73.9 years were included. Ten patients had Gorlin syndrome. Median treatment duration was 6 months. Median follow-up duration was 34.2 months. Globally, 81.7% of the patients showed clinical improvement (52.4% partial response and 29.3% complete response), 12.2% clinical stability and 6.1% disease progression. There was no statistically significant difference in clinical improvement between the 24h and 48h sonidegib posology. After 6 months of treatment, 48.8% of the patients discontinued sonidegib. Prior vismodegib treatment and recurrent primary BCC were associated with a poorer response to sonidegib. At 6 months of treatment, 68.3% of the patients experienced at least one adverse effect. CONCLUSION: Sonidegib shows good effectiveness and acceptable safety profile in usual clinical practice.

Searching for a prognostic index in lupus nephritis.

BACKGROUND: Currently we do not have an ideal biomarker in lupus nephritis (LN) that should help us to identify those patients with SLE at risk of developing LN or to determine those patients at risk of renal progression. We aimed to evaluate the development of a prognostic index for LN, through the evaluation of clinical, analytical and histological factors used in a cohort of lupus. We have proposed to determine which factors, 6 months after the diagnosis of LN, could help us to define which patients will have a worse evolution of the disease and may be, more aggressive treatment and closer follow-up. METHODS: A retrospective study to identify prognostic factors was carried out. We have included patients over 18 years of age with a clinical diagnosis of systemic lupus erythematosus (SLE) and kidney involvement confirmed by biopsy, who are followed up in our centre during the last 20 years. A multi-step statistical approach will be used in order to obtain a limited set of parameters, optimally selected and weighted, that show a satisfactory ability to discriminate between patients with different levels of prognosis. RESULTS: We analysed 92 patients with LN, although only 73 have been able to be classified according to whether or not they have presented poor renal evolution. The age of onset (44 vs. 32; p = 0.024), the value of serum creatinine (1.41 vs. 1.04; p = 0.041), greater frequency of thrombocytopenia (30 vs. 7%; p = 0.038), higher score in the renal chronicity index (2.47 vs. 1.04; p = 0.015), proliferative histological type (100%) and higher frequency of interstitial fibrosis (67 vs. 32%; p = 0.017) and tubular atrophy (67 vs. 32%; p = 0.018) was observed between two groups. The multivariate analysis allowed us to select the best predictive model for poor outcome at 6 months based on different adjustment and discrimination parameters. CONCLUSION: We have developed a prognostic index of poor renal evolution in patients with LN that combines demographic, clinical, analytical and histopathological factors, easy to use in routine clinical practice and that could be an effective tool in the early detection and management.

Complicaciones de los pacientes con uveítis en un estudio poblacional español: proyecto UveCAM / Complications of uveitis in a Spanish population, UveCAM study

Objetivo Estimar la frecuencia poblacional de complicaciones de uveítis y evaluar sus principales factores de riesgo en los pacientes con uveítis del estudio UveCAM. Pacientes y método Desarrollo de complicaciones de los 386 pacientes con uveítis de toda el área sanitaria de la provincia de Toledo (estudio UveCAM) durante el periodo de un año. Descripción de complicaciones y estudio de los posibles determinantes mediante modelos de regresión multivariante. Resultados Se dispuso de información sobre el desarrollo de complicaciones en 371 de los 386 pacientes del estudio. El 45,8% de los pacientes presentó al menos una complicación, siendo las más frecuentes las sinequias posteriores (19,0%), la hipertensión ocular (14,0%), el edema macular (7,5%), la membrana epirretiniana (6,9%), el glaucoma (6,6%), la atrofia iridiana (5,6%) y las cataratas (5,5%). El riesgo de complicaciones aumenta con la edad, las formas intermedias y panuveítis, y las de evolución crónica o recurrente. Conclusión Las uveítis se asocian con una elevada frecuencia de complicaciones, especialmente en pacientes de edad avanzada, con localización intermedia o posterior del proceso inflamatorio y con evolución crónica o recurrente (AU)

Objective To estimate the population frequency of uveitis complications and to evaluate their main risk factors in the patients with uveitis from the UveCAM study. Patients and methods Development of complications in 386 patients with uveitis in the whole health area of the province of Toledo (UveCAM study) during a period of one year. Description of complications and study of their possible determinants by means of multivariate regression models. Results Information on the development of complications was available in 371 of the 386 patients of the UveCAM study. 45.8% of patients had at least one complication, the most frequent were posterior synechiae (19.0%), ocular hypertension (14.0%), macular oedema (7.5%), epiretinal membrane (6.9%), glaucoma (6.6%), iridian atrophy (5.6%), and atrophy (5.6%) and cataracts (5.5%). The risk of complications increases with age, intermediate and panuveitis locations, and those of chronic or recurrent evolution. Conclusion Uveitis is associated with a high frequency of complications, especially in older patients, with intermediate or posterior localization of the inflammatory process, and chronic or recurrent evolution (AU)

Complications of uveitis in a Spanish population, UveCAM study.

OBJECTIVE: To estimate the population frequency of uveitis complications and to evaluate their main risk factors in the patients with uveitis from the UVECAM study. PATIENTS AND METHODS: Development of complications in 386 patients with uveitis in the whole health area of the province of Toledo (UVECAM study) during a period of 1 year. Description of complications and study of their possible determinants by means of multivariate regression models. RESULTS: Information on the development of complications was available in 371 of the 386 patients of the UVECAM study. The most frequent complications were posterior synechiae (19.0%), ocular hypertension (14.0%), macular edema (7.5%), epirretinal membrane (6.9%), glaucoma (6.6%), iridian atrophy (5.6%) and cataract (5.5%). The risk of complications increases with age, intermediate and panuveitis locations, and those of chronic or recurrent evolution. CONCLUSION: Uveitis is associated with a high frequency of complications, especially in older patients, with intermediate or posterior localization of the inflammatory process and chronic or recurrent evolution.

Prevalence and Incidence of Uveitis: A Systematic Review and Meta-analysis.

Background: Although the impact of uveitis on people's lives is clear, the frequency of this condition is unclear.Objective: To estimate the prevalence and incidence of uveitis.Methods: A systematic review with meta-analysis was conducted. Medline, Embase, and Cochrane Library were searched from inception to January 2019. The quality of the included studies was critically appraised with a grading system based on the Oxford Levels of Evidence. A detailed description of the populations studied and of factors affecting estimates was undertaken. Pooled analyses were conducted using a random-effects approach and expressed as incidence rates per 100,000 with 95% confidence intervals. Subgroup analyses by geographical region were conducted along with meta-regression to analyze possible factors for heterogeneity.Results: A total of 49 studies were included and critically appraised. Twenty-two were population-based, and 27 hospital-based. Heterogeneity was substantial in terms of populations studied, methods for ascertaining uveitis, including definitions, and reporting of results. This was especially important in prevalence studies, with data ranging from 9 to 730 cases per 100,000. For incidence studies, the meta-analysis yielded a pooled incidence of 50.45 per 100.000. The meta-regression showed the geographic region as an important explanatory factor of the heterogeneity between studies.Conclusion: Population-based estimates of the epidemiology of uveitis vary widely, owing to methodologies employed, definitions of uveitis and geographical regions; the representativeness and generalizability of many epidemiological studies of uveitis are limited.

Índices de gravedad en la artritis reumatoide: una revisión sistemática / Severity indices in rheumatoid arthritis: A systematic review

Objetivo: Identificar herramientas diseñadas para evaluar la gravedad global de los pacientes con artritis reumatoide (AR) para su uso en la investigación de marcadores pronósticos de artritis precoz. Métodos: Revisión sistemática de estudios cuyo objetivo fuera el desarrollo o validación de índices de gravedad en AR. Se valoró la calidad metodológica mediante la lista de comprobación COSMIN. Además, se evaluó la claridad de definición, viabilidad y probabilidad de estar presente durante los 2 primeros años de evolución. Resultados: Después de revisar 3.519 artículos, se identificaron 3 índices de gravedad. La Patient Activity Scale (PAS) valoró si el tratamiento previo o actual predecía la gravedad de la AR, medida mediante el patient-reported PAS. Las variables de tratamiento no permitieron distinguir entre los cuartiles superior e inferior de la PAS. El CIRAS incluye las variables edad, sexo, síndrome de Felty, número de visitas al reumatólogo y al rehabilitador, factor reumatoide (FR), recuento de plaquetas, marcadores inflamatorios y paneles bioquímicos solicitados. Su correlación fue baja (r=0,56), con un índice previamente validado por el mismo grupo investigador, el RARBIS, con el DAS28-PCR (r = 0,07) y el Multidimensional Health Assesment Questionnaire (MD-HAQ) (r=0,008). Por último, el RARBIS, utilizado para validar el CIRAS, fue ideado como un índice de gravedad de AR basado en registros médicos. Incluye como dominios cirugía, radiología, manifestaciones extraarticulares, clínica y variables de laboratorio, elegidas previamente por un panel de expertos. Este índice presentó una correlación débil con la intensidad de tratamiento (r = 0,35) y con el DAS 28 (r = 0,41). Conclusión: No existe ningún índice para valorar la gravedad de la AR sobre la base del curso evolutivo de los 2 primeros años de seguimiento y que se adapte a la estrategia terapéutica actual. Por lo tanto, creemos razonable el desarrollo de un nuevo índice de gravedad ad hoc para pacientes con artritis de reciente comienzo

Objective: To identify tools designed to evaluate the severity of patients with rheumatoid arthritis (RA) in order to use them in the investigation of prognostic markers in early arthritis. Methods: We conducted a systematic review of studies that developed/validated an index for RA disease severity. They were analyzed using the COSMIN checklist to assess their methodological quality. In addition, all the variables included were evaluated for their clarity of definition, feasibility and probability of being present in each outcome during the first 2 years of the disease course. To estimate redundancy, variables were grouped by domains. Results: After reviewing 3,519 articles, 3 studies were included. The first study, the PAS, assessed whether current and lifetime treatment with disease-modifying antirheumatic drugs and/or biologics accurately predicted RA severity, as measured by the patient-reported PAS. Treatment variables did not fully distinguish patients in the highest and lowest quartiles of PAS scores. Another severity index, the Claims-Based Index for RA Severity (CIRAS), included the variables age, sex, Felty's syndrome, number of rehabilitation and rheumatology visits, test for inflammatory markers, number of chemistry panels/platelet counts ordered and rheumatoid factor test. The correlation was low (r=0.56) with an index previously validated by the same research group, the RA medical records-based index of severity (RARBIS), with Disease Activity Score-C-reactive protein (DAS28-PCR) (r=0.07) and Multidimensional Health Assessment Questionnaire (MD-HAQ) (r=0.008). Finally, the RARBIS, used to validate the CIRAS, was devised as an RA severity index based on medical records. It includes as domains surgery, radiology, extra-articular manifestations, clinical and laboratory variables, previously chosen by an expert panel. RARBIS had a weak correlation with treatment intensity (r=0.35) and with DAS28 (r=0.41). Conclusion: There is no index to assess the severity of RA based on the course of the first 2 years of follow-up that is adapted to the current strategy of therapeutic management of this disease. Therefore, we believe it is reasonable to develop a new ad hoc severity index for patients with early arthritis

Severity indices in rheumatoid arthritis: A systematic review. / Índices de gravedad en la artritis reumatoide: una revisión sistemática.

OBJECTIVE: To identify tools designed to evaluate the severity of patients with rheumatoid arthritis (RA) in order to use them in the investigation of prognostic markers in early arthritis. METHODS: We conducted a systematic review of studies that developed/validated an index for RA disease severity. They were analyzed using the COSMIN checklist to assess their methodological quality. In addition, all the variables included were evaluated for their clarity of definition, feasibility and probability of being present in each outcome during the first 2 years of the disease course. To estimate redundancy, variables were grouped by domains. RESULTS: After reviewing 3,519 articles, 3 studies were included. The first study, the PAS, assessed whether current and lifetime treatment with disease-modifying antirheumatic drugs and/or biologics accurately predicted RA severity, as measured by the patient-reported PAS. Treatment variables did not fully distinguish patients in the highest and lowest quartiles of PAS scores. Another severity index, the Claims-Based Index for RA Severity (CIRAS), included the variables age, sex, Felty's syndrome, number of rehabilitation and rheumatology visits, test for inflammatory markers, number of chemistry panels/platelet counts ordered and rheumatoid factor test. The correlation was low (r=0.56) with an index previously validated by the same research group, the RA medical records-based index of severity (RARBIS), with Disease Activity Score-C-reactive protein (DAS28-PCR) (r=0.07) and Multidimensional Health Assessment Questionnaire (MD-HAQ) (r=0.008). Finally, the RARBIS, used to validate the CIRAS, was devised as an RA severity index based on medical records. It includes as domains surgery, radiology, extra-articular manifestations, clinical and laboratory variables, previously chosen by an expert panel. RARBIS had a weak correlation with treatment intensity (r=0.35) and with DAS28 (r=0.41). CONCLUSION: There is no index to assess the severity of RA based on the course of the first 2 years of follow-up that is adapted to the current strategy of therapeutic management of this disease. Therefore, we believe it is reasonable to develop a new ad hoc severity index for patients with early arthritis.

2013 ACR/EULAR systemic sclerosis classification criteria in patients with associated pulmonary arterial hypertension.

OBJECTIVE: To analyze the performance of the 1980 ACR and new 2013 ACR/EULAR criteria for systemic sclerosis (SSc) in cutaneous SSc (lcSSc) patients, especially those affected by lcSSc and pulmonary arterial hypertension (PAH). METHODS: All patients with a clinical lcSSc diagnosis from a prospective observational SSc cohort were included. Sociodemographic and disease-related variables were collected, and PAH confirmed by right heart catheterization (RHC). Performance of the 2013 and 1980 SSc criteria was analyzed in terms of clinical diagnosis. Descriptive and between-group analyses were performed as to the fulfillment of criterion sets, including comparison of survival. RESULTS: Overall, 321 patients were included, 63% of whom fulfilled the 1980 ACR and 93% the 2013 ACR/EULAR criteria. Agreement between both criteria sets proved poor (κ = 0.23). LcSSC patients fulfilling both criterion sets were significantly younger at diagnosis, whilst presenting organ involvement, calcinosis, fingertip digital ulcers, and pitting scars more frequently than those who met the 2013 criteria only. Patients who fulfilled the 2013 but not the 1980 criteria presented a higher degree of ACA positivity and PAH. Nearly 12% of patients developed PAH. Patients who did not meet the 1980 criteria were affected by a milder disease from but demonstrated higher pulmonary vascular resistance and lower cardiac index than those fulfilling both criterion sets. Whereas patients with PAH met the 2013 criteria, only 47% fulfilled the 1980 criteria. Regardless of criterion set fulfillment, high mortality was observed in PAH patients, with no significant between-patient difference based on criterion set. CONCLUSION: The new 2013 ARC/EULAR criteria prove more accurate than the former 1980 ACR criteria in identifying and differentiating patients with lcSSc, especially those with associated PAH. Since PAH exhibits a better prognosis if treated early, all SSc patients should undergo PAH screening.

Validation of the 2013 American College of Rheumatology/European League Against Rheumatism classification criteria for systemic sclerosis in patients from a capillaroscopy clinic.

OBJECTIVE: To validate the 2013 ACR/EULAR classification criteria for systemic sclerosis (SSc) in patients from a capillaroscopy clinic. METHODS: All patients attended in a capillaroscopy clinic were included. Sociodemographic and SSc-related variables were collected. Using as gold standard for SSc the clinical judgement, the performance (sensitivity, specificity, predictive values, and likelihood ratios) of the 2013 ACR/EULAR criteria were analyzed. Receiver operating characteristic (ROC) curve and the area under the curve (AUC) were calculated for the global score and individual items, and the best cutoffs were obtained. RESULTS: We included 327 patients (84% women, mean age at capillaroscopy 48 years). Main reasons for capillaroscopy referral were Raynaud's phenomenon (39%) and SSc evaluation (27%). The most frequent final clinical diagnosis were SSc (32.4%) and primary Raynaud's phenomenon (25.7%). The 2013 ACR/EULAR SSc classification criteria were met by 116 patients (35.5%). Sensitivity and specificity of the new criteria were 98.1% and 94.6%, respectively, and positive and negative predictive values were 89.7% and 99.1%. The individual variables with the best sensitivity were Raynaud's phenomenon (99.1%) and abnormal nailfold capillaries (81.1%). All the individual variables, except Raynaud's phenomenon, puffy fingers and sclerodactily showed high specificity values, over 90%. The best cutoffs of the total score were ≥8, ≥9, and ≥10, and the AUC = 0.993. CONCLUSIONS: We validated the new ACR/EULAR classification criteria for SSc in unselected patients from a capillaroscopy clinic. Global score and individual items included in the new criteria show high diagnostic accuracy and discriminatory capacity.

A comparative study of the effectiveness of cisplatin and 5-fluorouracil on cutaneous squamous human carcinoma cell line: Potential chemotherapy alternative to surgery.

Surgery as treatment for local invasive cutaneous squamous cell carcinoma (cSCC) is not always feasible due to the age and/or the health status of patients. Thus, the investigation of new strategies to improve the quality of life of them is required. The aim of this work is to investigate two chemotherapy agents individually on cSCC cells with the purpose to provide a better understanding of the effectiveness underlying each one. The cisplatin effectiveness is compared at different times with that observed for the 5-fluorouracil treatment. The effectiveness of both was assessed by using flow cytometry to determine the survival cell ratio, and QBlue test to study the cell recovery ability after treatments. A significant increase in the number of apoptotic cells, especially 48 hours after treatments, has been detected. Despite this, cisplatin arises as the most promising agent for the treatment of local invasive cutaneous squamous cell carcinoma due to the fact that a lower concentration and time are required to observe a higher effectiveness on cells with respect to the 5-fluorouracil. An optimal cisplatin-based chemotherapy might provide a better outcome for patients affected by a local invasive cSCC rather than surgery.

Masa subcutánea pétrea en el brazo de una mujer joven / Stony-Hard Subcutaneous Mass on the Arm of a Young Woman

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Analysis of disease activity and response to treatment in a large Spanish cohort of patients with systemic lupus erythematosus.

OBJECTIVES: The objectives of this paper are to study the impact of disease activity in a large cohort of patients with systemic lupus erythematosus (SLE) and estimate the rate of response to therapies. METHODS: We conducted a nationwide, retrospective, multicenter, cross-sectional cohort study of 3658 SLE patients. Data on demographics, disease characteristics: activity (SELENA-SLEDAI), damage, severity, hospitalizations and therapies were collected. Factors associated with refractory disease were identified by logistic regression. RESULTS: A total of 3658 patients (90% female; median SLE duration (interquartile range): 10.4 years (5.3-17.1)) were included. At the time of their last evaluation, 14.7% of the patients had moderate-severe SLE (SELENA-SLEDAI score ≥6). There were 1954 (53.4%) patients who were hospitalized for activity at least once over the course of the disease. At some stage, 84.6% and 78.8% of the patients received glucocorticoids and antimalarials, respectively, and 51.3% of the patients received at least one immunosuppressant. Owing to either toxicity or ineffectiveness, cyclophosphamide was withdrawn in 21.5% of the cases, mycophenolate mofetil in 24.9%, azathioprine in 40.2% and methotrexate in 46.8%. At some stage, 7.3% of the patients received at least one biologic. A total of 898 (24.5%) patients had refractory SLE at some stage. Renal, neuropsychiatric, vasculitic, hematological and musculoskeletal involvement, a younger age at diagnosis and male gender were associated with refractory disease. CONCLUSIONS: A significant percentage of patients have moderately-to-severely active SLE at some stage. Disease activity has a big impact in terms of need for treatment and cause of hospitalization. The effectiveness of the standard therapies for reducing disease activity is clearly insufficient. Some clinical features are associated with refractory SLE.

Registro nacional de pacientes con lupus eritematoso sistémico de la Sociedad Española de Reumatología: objetivos y metodología / National registry of patients with systemic lupus erythematosus of the Spanish Society of Rheumatology: objectives and methodology

Objetivo. Describir en detalle los objetivos y aspectos metodológicos del registro de lupus eritematoso sistémico (LES) de la Sociedad Española de Reumatología (RELESSER). Métodos. Registro multicéntrico, de base hospitalaria, con recogida retrospectiva de datos de una amplia muestra representativa de adultos con LES (criterios ACR 1997) procedentes de servicios de reumatología españoles. Incluye datos demográficos, manifestaciones clínicas frecuentes e infrecuentes (< 1%), actividad, daño, gravedad, comorbilidad, tratamientos y mortalidad, totalizando 359 variables por paciente, con definiciones altamente estandarizadas. Se ha realizado un análisis descriptivo preliminar de los datos. Resultados. Han participado 45 centros e incluido 4.024 pacientes con LES o LES incompleto (91% con ≥ 4 criterios ACR). El 90% son mujeres y el 93% caucásicos, con una mediana de edad al diagnóstico de 33 años; mediana de duración de la enfermedad: 120 meses; seguimiento medio: 104 meses. Se encuentran en seguimiento activo 3.222 pacientes (81%) y 591 (14%) han sido perdidos para seguimiento. Las medianas del índice de actividad SELENA-SLEDAI, índice de daño de SLICC/ACR y de gravedad de Katz han sido 2, 1 y 2, respectivamente. Un total de 211 pacientes (6%) han fallecido. Conclusiones. RELESSER representa el registro de LES europeo con mayor número de pacientes construido hasta la fecha, disponiendo de abundante información actualizada y fiable sobre manifestaciones del LES, situación de enfermedad, comorbilidad y tratamientos en condiciones de práctica clínica real. RELESSER se constituye como herramienta de gran potencialidad para la investigación clínica multicéntrica en el LES(AU)

Objective: To describe the objectives, design and methods of the Spanish Society of Rheumatology systemic lupus erythematosus (SLE) registry (RELESSER). Methods: Multicenter, hospital-based registry, with retrospective collection of data from a large representative sample of adult patients with SLE (1997 ACR criteria) attending Spanish rheumatology services. The registry includes demographic data, frequent and infrequent (< 1%) clinical manifestations, information about activity, damage, severity, comorbidity, treatments and mortality, collecting 359 variables per patient, with highly standardized definitions. We performed a preliminary descriptive analysis of the data. Results: Forty-five centers were involved and 4,024 SLE patients (91% with >= 4 ACR criteria) have been included; 90% are women and 93% caucasians, with a median age at diagnosis of 33 years, median disease duration: 120 months, median follow-up duration: 104 months; 3,222 (81%) of the patients are in active follow-up and 591 (14%) were lost to follow-up. The median values of the SELENA-SLEDAI score, SLICC/ACR damage index and Katz severity index have been 2, 1 and 2, respectively. A total of 211 patients (6%) died. Conclusions: RELESSER represents the largest European SLE registry built to date, providing comprehensive and reliable information on SLE manifestations, disease status, comorbid conditions and treatments in daily clinical practice. RELESSER is constituted as a tool of great potential for multicenter clinical research in SLE (AU)

National registry of patients with systemic lupus erythematosus of the Spanish Society of Rheumatology: objectives and methodology.

OBJECTIVE: To describe the objectives, design and methods of the Spanish Society of Rheumatology systemic lupus erythematosus (SLE) registry (RELESSER). METHODS: Multicenter, hospital-based registry, with retrospective collection of data from a large representative sample of adult patients with SLE (1997 ACR criteria) attending Spanish rheumatology services. The registry includes demographic data, frequent and infrequent (<1%) clinical manifestations, information about activity, damage, severity, comorbidity, treatments and mortality, collecting 359 variables per patient, with highly standardized definitions. We performed a preliminary descriptive analysis of the data. RESULTS: Forty-five centers were involved and 4,024 SLE patients (91% with ≥ 4 ACR criteria) have been included; 90% are women and 93% caucasians, with a median age at diagnosis of 33 years, median disease duration: 120 months, median follow-up duration: 104 months; 3,222 (81%) of the patients are in active follow-up and 591 (14%) were lost to follow-up. The median values of the SELENA-SLEDAI score, SLICC/ACR damage index and Katz severity index have been 2, 1 and 2, respectively. A total of 211 patients (6%) died. CONCLUSIONS: RELESSER represents the largest European SLE registry built to date, providing comprehensive and reliable information on SLE manifestations, disease status, comorbid conditions and treatments in daily clinical practice. RELESSER is constituted as a tool of great potential for multicenter clinical research in SLE.

Autoevaluación de salud mental en pacientes con psoriasis moderada a grave: un estudio multicéntrico observacional de 1.164 pacientes en España (el estudio VACAP) / Mental Health Self-Assessment in Patients With Moderate to Severe Psoriasis: An Observational, Multicenter Study of 1164 Patients in Spain (The VACAP Study)

Antecedentes: La gravedad de la psoriasis parece estar relacionada con una pobre autoevaluación de la salud mental. Objetivos: Evaluar el impacto que tiene la gravedad de la psoriasis sobre los trastornos de ansiedad y del estado de ánimo. Metodología: Estudio prospectivo, observacional y multicéntrico realizado por 123 dermatólogos en España. Los pacientes con psoriasis moderada a grave (n = 164; edad media [DE] 45,11 [13,92] años; 60,8% hombres) fueron valorados al inicio del estudio y 4 meses más tarde. Para medir la gravedad de la psoriasis se usó el Índice de Severidad y Área de Psoriasis (PASI) con un rango de puntuación entre 0 (leve) y 72 (grave); el área de superficie corporal afectada (BSA) y las puntuaciones de la evaluación global del médico (PGA) entre 1 (leve) y 7 (grave). La salud mental se evaluó utilizando la escala hospitalaria de ansiedad y depresión (HADS), con una puntuación total entre 0 y 42 (los valores más altos representan peor salud mental). Se compararon la media de las puntuaciones obtenidas en la primera y segunda visita. Resultados: La media (DE) de las puntuaciones mejoraron entre la primera y la segunda visita de la siguiente manera: de 13,24 (9,50) a 5,07 (6,03) para el PASI; de 12,52 (7,92) a 10,78 (7,32) para el HADS global, de 7,83 (4,55) a 6,85 (4,21) para el HADS subescala de ansiedad y de 4,72 (4,12) a 3,95 (3,76) para el HADS subescala de depresión (P < 0,001 en todos los casos). El análisis multivariante mostró que los principales factores relacionados con la ansiedad fueron la gravedad de la psoriasis, el género y la finalización de los estudios de licenciatura. Las variables independientes incluidas en el modelo de estudio para la depresión fueron la gravedad de la psoriasis, el género y la psoriasis localizada en la cabeza. Conclusiones: La reducción en la gravedad de la enfermedad mejora la autoevaluación de los trastornos del estado de ánimo y de la ansiedad en pacientes con psoriasis de moderada a grave (AU)

Background: Poor self-assessed mental health appears to be related to the severity of psoriasis. Objective: To evaluate the impact of psoriasis severity on mood and anxiety disorders. Methods: A prospective, observational, multicenter study was conducted by 123 dermatologists in Spain. Patients (n = 164; mean [SD] age, 45.11 [13.92] years; 60.8% males) with moderate to severe psoriasis were evaluated at baseline and 4 months later. Psoriasis severity was measured using the Psoriasis Area and Severity Index (PASI), with a score range of 0 (mild) to 72 (severe); body surface area involvement (BSA); and physician global assessment (PGA) scores, with a range of 1 (mild) to 7 (severe). Mental health was assessed using the Hospital Anxiety and Depression Scale (HADS), with a total possible score of 0–42 (higher scores representing worse mental health). Mean first and second visit scores were compared. Results: Mean (SD) scores improved between the first and second visit as follows: 13.24 (9.50) to 5.07 (6.03) for PASI, 12.52 (7.92) to 10.78 (7.32) for overall HADS, 7.83 (4.55) to 6.85 (4.21) for the HADS anxiety subscale, and 4.72 (4.12) to 3.95 (3.76) for the HADS depression subscale (P < 0.001 in all cases). Multivariate analyses showed that the main factors related to anxiety were psoriasis severity, sex, and completion of graduate studies. The independent variables included in the model for depression were psoriasis severity, sex, and psoriasis located on the head. Conclusions: Reductions in disease severity improve self-assessed mood and anxiety disorders in patients with moderate to severe psoriasis (AU)

Características demográficas y calidad de vida relacionada con la salud en pacientes con psoriasis moderada a grave: Estudio vacap / Demographic characteristics and health-related quality of life of patients with moderate-to-severe psoriasis: The VACAP study

Antecedentes: La psoriasis se asocia a un deterioro de la Calidad de Vida Relacionada con la Salud (CVRS) de los pacientes. El objetivo de este estudio fue valorar la CVRS en pacientes con psoriasis moderada a grave. Métodos: Se llevó a cabo un estudio prospectivo observacional (Estudio VACAP) sobre 1217 pacientes distribuidos en 123 centros de España. Los pacientes fueron evaluados al inicio del estudio (visita 1 [V1]) y de nuevo 4 meses más tarde (visita 2 [V2]). Para determinar la gravedad de la psoriasis se emplearon los siguientes índices: a) el índice de gravedad y de área de la psoriasis (Psoriasis Area and Severity Index [PASI]) (valores entre 0-72, las puntuaciones más altas indican una mayor gravedad de la enfermedad); b) el índice de superficie corporal afectada (Body Surface Area [BSA]), y c) la evaluación general efectuada por el médico (Physicians Global Assessment [PGA]) (intervalo entre 1–7: los valores más altos son indicativos de una enfermedad más grave). Para evaluar la CVRS se utilizaron 4 tipos de cuestionarios: a) el Cuestionario de Calidad de Vida SF-36 (SF-36) (escala entre 0–100, los valores más altos indican una mejor CVRS); b) el EuroQol (EQ-5D) (intervalo comprendido entre 1–3, cuanto más bajos sean los resultados obtenidos mejor es la CVRS); c) el Índice de Calidad de Vida en Dermatología (Dermatology Life Quality Index [DLQI]) (intervalo entre 0–30; de mejor a peor CVRS); y d) el Índice de Discapacidad de la Psoriasis (Psoriasis Disability Index [PDI]) (escala de puntuación entre 0–45, los datos más altos muestran una mejor CVRS). Resultados: La edad media de los pacientes (desviación estándar [DS]) en la V1 fue de 45,11 (13,92) años. La edad media de inicio de la psoriasis fue de 26,08 (14,19) años. La mayoría de los pacientes eran mujeres (61%) y trabajadoras (68%). El valor medio del PASI fue de 13,24 (9,59) en la V1 y de 5,07 (6,03) en la V2 (p < 0,001). Las puntuaciones de los cuestionarios de CVRS genéricos (EQ-5D, SF-36) mejoraron significativamente en todas las dimensiones evaluadas entre las 2 visitas (p < 0,001). Los cuestionarios específicos de enfermedad también revelaron la mejoría general de la calidad de vida a lo largo del tiempo: la puntuación media total del DLQI fue de 8,97 (7,28) en la V1 y de 4,76 (5,72) en la V2 (p < 0,001), y los valores medios totales del PDI fueron de 9,24 (8,76) en la V1 y 4,88 (6,65) en la V2 (p < 0,001). El análisis multivariado, siendo el PDI la variable dependiente, mostró que los principales factores relacionados con la CVRS eran la gravedad de la psoriasis, medida por PASI, (p < 0,001) y el sexo (p = 0,048). Conclusiones: El principal factor relacionado con la CVRS en pacientes con psoriasis es la gravedad de la enfermedad (AU)

Background: Psoriasis is associated with a deterioration in the health-related quality of life (HRQoL) of affected patients. The aim of this study was to assess the HRQoL of patients with moderate-to-severe psoriasis. Methods: A prospective observational study (the VACAP Study) was carried out in 123 centers in Spain with 1217 patients. Patients were evaluated at baseline (visit 1 [V1]) and again four months later (visit 2 [V2]). The severity of psoriasis was determined using the following indices: (I) Psoriasis Area and Severity Index (PASI) (score range 0–72, higher score indicates more severe disease), (II) the body surface area (BSA) affected, and (III) the Physicians Global Assessment (PGA) (range 1–7, higher score indicates more severe disease). Four questionnaires were used for the assessment of the HRQoL: (I) the Short-Form 36 quality-of-life questionnaire (SF-36) (score range 0–100, higher score indicates better HRQoL); (II) Euroqol (EQ-5D) (range from 1 to 3, lower score indicates better HRQoL); (III) Dermatology Life Quality Index (DLQI) (ranges 0–30; from best to worst HRQoL); and (IV) Psoriasis Disability Index (PDI) (ranges 0–45; higher score indicates better HRQoL). Results: The mean (SD) age of the patients was 45.11 ( 13.92) years at V1. The mean age at the onset of psoriasis was 26.08 (14.19) years. The majority of patients were female (61%) and were employed (68%). The mean PASI score was 13.24 (9.50) at V1 and 5.07 (6.03) at V2 (P < 0.001). Scores from the generic HRQoL questionnaires (EQ-5D, SF-36) showed significant improvement between visits in all dimensions measured (P < 0.001). The disease-specific questionnaires also revealed overall improvements in quality of life over time: the DLQI mean total score was 8.97 (7.28) at V1 and 4.76 (5.72) at V2 (P < 0.001), and the PDI mean total score was 9.24 (8.76) V1 and 4.88 (6.65) at V2 (P < 0.001). Multivariate analysis using PDI as the dependent variable showed that the principal factors related to HRQoL were severity of psoriasis as measured by PASI (P < .001), and gender (P = 0.048). Conclusions: The principal factor related to HRQoL in patients with psoriasis is the severity of the disease (AU)