Juvenile- and adult-onset systemic lupus erythematosus: a comparative study in a large cohort from the Spanish Society of Rheumatology Lupus Registry (RELESSER).

OBJECTIVES: We aimed to describe juvenile-onset systemic lupus erythematosus (jSLE) features and to establish its differences compared to adult-onset SLE (aSLE) from a large national database. METHODS: Data from patients (≥4 ACR criteria) included in Spanish Society of Rheumatology Lupus Registry (RELESSER) were analysed. Sociodemographic, clinical, serological, activity, treatment, cumulative damage, comorbidities and severity data were collected. Patients with disease onset <18 years were described and compared to those with disease onset ≥18 years. RESULTS: We reviewed 3,428 aSLE patients (89.6% women) and 484 jSLE patients (89.8% girls), 93% Caucasian (both groups). Mean age at diagnosis was 38.1±14 and 16.6±6.3 years (p<0.001) and mean age at the end of follow-up was 48.8±14.3 and 31.5±30 years (p<0.001), respectively. jSLE showed significantly more clinical (including lymphadenopathy, fever, malar rash, mucosal ulcers, pericarditis, pleuritis, Raynaud's phenomenon, lupus nephritis, recurrent nephritis, histologic nephritis changes, thrombocytopenia, haemolytic anaemia, thrombotic thrombocytopenic purpura, seizures, lupus headache and organic brain syndrome) and immunological (a-dsDNA and a-Sm antibodies, hypocomplementaemia) involvement than did aSLE, except for secondary Sjögren's syndrome, a-Ro antibodies, fibromyalgia and osteoporosis. jSLE also showed more SLE family history, longer diagnosis delay, higher SLEDAI and Katz scores, but lower Charlson scores than aSLE. Several specific domains were more frequently involved in SLICC/ACR DI in jSLE. jSLE patients more frequently underwent all SLE-related treatment and procedures, as well as dialysis and kidney transplantations. CONCLUSIONS: jSLE shares many clinical and serological features with aSLE. However, jSLE patients typically manifested more activity, severity, cumulative damage in certain areas, than their aSLE counterparts.

A reduced 12-joint ultrasound examination predicts lack of X-ray progression better than clinical remission criteria in patients with rheumatoid arthritis.

To study the predictive value of clinical remission definitions and ultrasound (US) examination on X-ray progression in rheumatoid arthritis (RA). This was an observational prospective multicenter 1-year follow-up cohort of RA patients with moderate disease activity (3.2 < DAS28 ≤ 5.1) who started anti-TNF therapy. DAS28ESR, DAS28CRP, SDAI, CDAI, and ACR/EULAR remission criteria were applied and reduced 12-joint US examination was performed at baseline and at 6 and 12 months. At baseline and month 12, radiographs of hands and feet were obtained in a subset of patients. A blind independent reader scored radiographs. X-ray progression was defined as Sharp van der Heijde change score >1 and no progression was defined as ≤0. 319 of 357 patients completed the study; patients had a mean (SD) age of 53.5 (13.1) years, with a disease duration of 7.5 (7.1) years. Laboratory, clinical, and US values significantly improved at month 6, except CRP, with additional improvement at month 12. Remission and low disease activity rates increased at follow-up. In the subset of 115 patients with radiological studies, clinical remission by any definition was not significantly associated with X-ray progression. Patients without PD signal at baseline and month 6 were a lower risk of X-ray progression than patients with PD signal, OR 0.197 (95% CI 0.046-0.861) and 0.134 (95% CI 0.047-0.378), respectively. Absence of PD signal, but not clinical remission predicts lack of X-ray progression. A feasible 12-joint US examination may add relevant information to RA remission criteria.

Relationship between damage clustering and mortality in systemic lupus erythematosus in early and late stages of the disease: cluster analyses in a large cohort from the Spanish Society of Rheumatology Lupus Registry.

OBJECTIVES: To identify patterns (clusters) of damage manifestations within a large cohort of SLE patients and evaluate the potential association of these clusters with a higher risk of mortality. METHODS: This is a multicentre, descriptive, cross-sectional study of a cohort of 3656 SLE patients from the Spanish Society of Rheumatology Lupus Registry. Organ damage was ascertained using the Systemic Lupus International Collaborating Clinics Damage Index. Using cluster analysis, groups of patients with similar patterns of damage manifestations were identified. Then, overall clusters were compared as well as the subgroup of patients within every cluster with disease duration shorter than 5 years. RESULTS: Three damage clusters were identified. Cluster 1 (80.6% of patients) presented a lower amount of individuals with damage (23.2 vs 100% in clusters 2 and 3, P < 0.001). Cluster 2 (11.4% of patients) was characterized by musculoskeletal damage in all patients. Cluster 3 (8.0% of patients) was the only group with cardiovascular damage, and this was present in all patients. The overall mortality rate of patients in clusters 2 and 3 was higher than that in cluster 1 (P < 0.001 for both comparisons) and in patients with disease duration shorter than 5 years as well. CONCLUSION: In a large cohort of SLE patients, cardiovascular and musculoskeletal damage manifestations were the two dominant forms of damage to sort patients into clinically meaningful clusters. Both in early and late stages of the disease, there was a significant association of these clusters with an increased risk of mortality. Physicians should pay special attention to the early prevention of damage in these two systems.

Fibromyalgia prevalence and related factors in a large registry of patients with systemic lupus erythematosus.

OBJECTIVES: The objective of this study is to determine the prevalence of fibromyalgia (FM) in systemic lupus erythematosus (SLE) patients and to study its relationship to depression and other SLE-related factors. METHODS: A cross-sectional data analysis from the RELESSER-Transversal Spanish Registry, which includes SLE patients in a national multicentre retrospective charts review, was performed. INCLUSION CRITERIA: patients who fulfilled ≥4 ACR 1997 SLE criteria. Main variables were disease duration, depression, sociodemographics, comorbidities, SLE activity symptoms, serological findings, therapies and different disease status indices. Statistical analyses included a descriptive, associative and logistic regression analyses. A literature review was performed. RESULTS: 3,591 SLE patients were included, 90.1% women, 34.6 years of age at diagnosis (SD 14.6 years) and 93.1% Caucasians. FM prevalence was 6.2%. SLE patients with disease duration >5 years showed more FM than those with duration <5 years: 6.9% vs. 4.0%, respectively (p<0.05). SLE-FM patients showed higher prevalence of depression compared to non-FM-SLE patients: 53.1% vs. 14.6%, respectively (p<0.001). After adjusting by risk factors, the OR (CI) of suffering depression in FM-SLE patients was 6.779 (4.770-9.636), p<0.001. The OR of having secondary Sjögren's 2.447 (1.662-3.604), p<0.001, photosensitivity 2.184 (1.431-3.334), p<0.001, and oral ulcers 1.436 (1.005-2.051), p=0.047. CONCLUSIONS: Prevalence of FM in Caucasian SLE patients was high compared to the general population, and was significantly higher in those in later stages of disease. SLE patients with depression showed a strong risk of developing FM. Photosensitivity, oral ulcers and secondary Sjögren's were the only SLE-related factors associated with FM.

[Tumour necrosis factor alpha antagonists in established rheumatoid arthritis: effectiveness comparative study]. / Estudio comparativo de efectividad de los inhibidores del factor de necrosis tumoral alfa en la artritis reumatoide establecida.

BACKGROUND AND OBJECTIVE: Knowing the differences in the effectiveness between three tumour necrosis factor alpha antagonists (anti-TNF alpha) in rheumatoid arthritis (RA) has important clinical implications. The aim of this study was to assess anti-TNF alpha effectiveness and to study possible differences in outcomes between them. PATIENTS AND METHOD: We included all patients with rheumatoid arthritis (RA) attended in consulting room from Zaragoza Area II between May 2000 and December 2006 who completed a year with anti-TNF alpha treatment. Several demographic and clinical parameters at the beginning and after a year with three different agents were analysed and compared. RESULTS: 119 patients completed a year with anti-TNF alpha, 28 with infliximab, 44 with etanercept and 37 with adalimumab. After a year with treatment, DAS 28 descended 1,82 (1,42) points and HAQ 0,3 (0,58) (p<0,05). Comparing the clinical parameters after a year DAS 28 was 3,8 in the three groups. HAQ was 1,2 for patients in treatment with infliximab and 0,9 for patients with etanercept and adalimumab. There were no significant differences in effectiveness between the 3 drugs. CONCLUSIONS: Anti-TNF alpha drugs are effective to treat RA and the effectiveness is similar in all them.

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.

Incidence, associated factors and clinical impact of severe infections in a large, multicentric cohort of patients with systemic lupus erythematosus.

OBJECTIVES: To estimate the incidence of severe infection and investigate the associated factors and clinical impact in a large systemic lupus erythematosus (SLE) retrospective cohort. METHODS: All patients in the Spanish Rheumatology Society Lupus Registry (RELESSER) who meet ≥4 ACR-97 SLE criteria were retrospectively investigated for severe infections. Patients with and without infections were compared in terms of SLE severity, damage, comorbidities, and demographic characteristics. A multivariable Cox regression model was built to calculate hazard ratios (HRs) for the first infection. RESULTS: A total of 3658 SLE patients were included: 90% female, median age 32.9 years (DQ 9.7), and mean follow-up (months) 120.2 (±87.6). A total of 705 (19.3%) patients suffered ≥1 severe infection. Total severe infections recorded in these patients numbered 1227. The incidence rate was 29.2 (95% CI: 27.6-30.9) infections per 1000 patient years. Time from first infection to second infection was significantly shorter than time from diagnosis to first infection (p < 0.000). Although respiratory infections were the most common (35.5%), bloodstream infections were the most frequent cause of mortality by infection (42.0%). In the Cox regression analysis, the following were all associated with infection: age at diagnosis (HR = 1.016, 95% CI: 1.009-1.023), Latin-American (Amerindian-Mestizo) ethnicity (HR = 2.151, 95% CI: 1.539-3.005), corticosteroids (≥10mg/day) (HR = 1.271, 95% CI: 1.034-1.561), immunosuppressors (HR = 1.348, 95% CI: 1.079-1.684), hospitalization by SLE (HR = 2.567, 95% CI: 1.905-3.459), Katz severity index (HR = 1.160, 95% CI: 1.105-1.217), SLICC/ACR damage index (HR = 1.069, 95% CI: 1.031-1.108), and smoking (HR = 1.332, 95% CI: 1.121-1.583). Duration of antimalarial use (months) proved protective (HR = 0.998, 95% CI: 0.997-0.999). CONCLUSIONS: Severe infection constitutes a predictor of poor prognosis in SLE patients, is more common in Latin-Americans and is associated with age, previous infection, and smoking. Antimalarials exerted a protective effect.

Inducción de endometriosis en rata Wistar, mediante autotransplante de fragmento uterino, sobre cicatriz previamente producida en el cuerno uterino izquierdo / Induction of endometriosis in Wistar rats through autologous transplantation of a uterine fragment over a previous scar in the left uterine horn

Objetivo. Estudiar la posibilidad de inducir endometriosis interna/externa en rata wistar, a través de un nuevo modelo microquirúrgico, que denominamos PGR 1. Sujetos y métodos. Se han utilizado 11 ratas Wistar, a las que se ha realizado un autotransplante de fragmento uterino, sobre la cicatriz resultante de la cirugía necesaria para su obtención. Después de 8 semanas se les practicó una nueva intervención, para la inspección de la cavidad abdominal y la toma de muestras relevantes para su estudio anatomopatológico. Resultados. Macroscópicos: adherencias que afectaban fundamentalmente a la zona del implante; vesículas amarillentas. Microscópicos: vesículas/quistes; fenómenos inflamatorios; neovascularización; endometriosis interna en un animal. Conclusiones. Este nuevo modelo de implante nos puede ayudar a comprender mejor los mecanismos involucrados en la aparición de endometriosis, tanto interna como externa (AU)

Objective. To study the possibility of inducing internal or external endometriosis in Wistar rats through a new microsurgical model known as PGR 1. Subjects and methods. Eleven Wistar rats underwent autologous transplantation of a uterine fragment over a deliberately created scar. After 8 weeks, a further intervention was carried out to inspect the abdominal cavity and take samples for pathological analysis. Results. Macroscopic results consisted of adhesions mainly affecting the implant and yellow vesicles. Microscopic findings consisted of vesicles/cysts, inflammatory phenomena, neovascularization, and internal endometriosis in one animal. Conclusions. This new implant model could enhance our understanding of the mechanisms involved in the development of endometriosis, both internal and external (AU)

Estudio comparativo de efectividad de los inhibidores del factor de necrosis tumoral alfa en la artritis reumatoide establecida / Tumour necrosis factor alpha antagonists in established rheumatoid arthritis: Effectiveness comparative study

Fundamento y objetivo: El conocimiento de las posibles diferencias de efectividad de los 3 inhibidores del factor de necrosis tumoral alfa (anti-TNF-α) en la artritis reumatoide (AR) tiene importantes implicaciones en la práctica clínica. El objetivo del estudio fue determinar la efectividad de los 3 anti-TNF-α en la AR y estudiar posibles diferencias de resultados entre ellos. Pacientes y método: Se incluyó a todos los pacientes con AR atendidos en consultas del Sector Sanitario II de Zaragoza entre mayo de 2000 y diciembre de 2006 que completaron un año de tratamiento con anti-TNF-α. Se analizaron diferentes parámetros demográficos y clínicos al inicio y al año de tratamiento, y se compararon entre los 3 grupos. Resultados: Un total de 119 pacientes completaron un año de tratamiento con los anti-TNF-α: 38 pacientes con infliximab, 44 pacientes con etanercept y 37 pacientes con adalimumab. Después de un año de tratamiento hubo un descenso medio (desviación estándar) del Disease Activity Score 28 (DAS 28) de 1,82 puntos (1,42) y de 0,3 puntos (0,58) del Health Assessment Questionnaire (HAQ) (p<0,05). Al comparar las variables clínicas de los 3 fármacos al año de tratamiento, el DAS 28 medio fue de 3,8 puntos en los 3 grupos. El valor medio del HAQ fue de 1,2 para los pacientes que estuvieron en tratamiento con infliximab y de 0,9 para los pacientes que estuvieron en tratamiento con etanercept y adalimumab. No se encontraron diferencias significativas en efectividad entre los 3 fármacos. Conclusiones: Los anti-TNF-α son fármacos efectivos para el tratamiento de la AR y se observa una efectividad similar para todos ellos (AU)

Background and objective: Knowing the differences in the effectiveness between three tumour necrosis factor alpha antagonists (anti-TNF alpha) in rheumatoid arthritis (RA) has important clinical implications. The aim of this study was to assess anti-TNF alpha effectiveness and to study possible differences in outcomes between them. Patients and method: We included all patients with rheumatoid arthritis (RA) attended in consulting room from Zaragoza Area II between May 2000 and December 2006 who completed a year with anti-TNF alpha treatment. Several demographic and clinical parameters at the beginning and after a year with three different agents were analysed and compared. Results: 119 patients completed a year with anti-TNF alpha, 28 with infliximab, 44 with etanercept and 37 with adalimumab. After a year with treatment, DAS 28 descended 1,82 (1,42) points and HAQ 0,3 (0,58) (p<0,05). Comparing the clinical parameters after a year DAS 28 was 3,8 in the three groups. HAQ was 1,2 for patients in treatment with infliximab and 0,9 for patients with etanercept and adalimumab. There were no significant differences in effectiveness between the 3 drugs. Conclusions: Anti-TNF alpha drugs are effective to treat RA and the effectiveness is similar in all them (AU)

Artritis erosiva seronegativa asociada a gammapatía monoclonal / Erosive seronegative arthritis associated with monoclonal gammopathy

No disponible