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Aim: To study the influence of prednisone dose during the first month after systemic lupus erythematosus (SLE) diagnosis (prednisone-1) on glucocorticoid burden during the subsequent 11 months (prednisone-2-12). Methods: 223 patients from the Registro Espanol de Lupus Eritematoso Sisternico inception cohort were studied. The cumulative dose of prednisone-1 and prednisone-2-12 were calculated and recoded into a four-level categorical variable: no prednisone, low dose (up to 7.5 mg/day), medium dose (up to 30 mg/day) and high dose (over 30 mg/day). The association between the cumulative prednisone-1 and prednisone-2-12 doses was tested. We analysed whether the four level prednisone-1 categorical variable was an independent predictor of an average dose >7.5 mg/day of prednisone-2-12. Adjusting variables included age, immunosuppressives, antimalarials, methyl-prednisolone pulses, lupus nephritis and baseline SLE Disease Activity Index (SLEDAI). Results: Within the first month, 113 patients (51%) did not receive any prednisone, 24 patients (11%) received average low doses, 46 patients (21%) received medium doses and 40 patients (18%) received high doses. There was a strong association between prednisone-1 and prednisone-2-12 dose categories (p7.5 mg/day, while patients receiving low dose prednisone-1 were not (adjusted OR 1.4, 95% CI 0. 0.38 to 5.2). If the analysis was restricted to the 158 patients with a baseline SLEDAI of >= 6, the model did not change. Conclusion: The dose of prednisone during the first month after the diagnosis of SLE is an independent predictor of prednisone burden during the following 11 months., This study was supported by the Spanish Society of Internal Medicine.

Registro RELES-Grupo de Enfermedades Autoinmunes Sistémicas (GEAS)., [ES] Introducción: Los registros de pacientes son herramientas útiles para evaluar enfermedades poco frecuentes. Nuestro objetivo es presentar el «Registro español de pacientes con lupus eritematoso sistémico» (RELES)., Pacientes y métodos: RELES se inició en 2008, como un registro multicéntrico de cohortes, observacional, prospectivo, incluyendo a pacientes desde el momento del diagnóstico, cuyo objetivo es analizar la incidencia y complicaciones no inflamatorias del lupus eritematoso sistémico (LES). Participan los servicios de Medicina Interna de 38 hospitales españoles., Resultados: Se incluyó a 298 pacientes con una edad media de 40,8 ± 15,7 años, de los que el 88,9% eran mujeres y el 85,6% de raza caucásica. En la primera visita, predominaron las manifestaciones articulares (74,5%). Ciento setenta y siete pacientes (59,4%) mostraban positividad para anti-DNA nativo, siendo superior en estos la frecuencia de nefritis lúpica (26,7% vs. 14%, p = 0,009; riesgo relativo [RR] 1,33), de anemia hemolítica (13,6% vs. 4,1%, p= 0,07; RR 1,46) y linfopenia (55,4% vs. 43,8%, p = 0,05; RR 1,21). La mediana del Systemic Lupus Erythematosus Disease Activity Index 2000 (SLEDAI 2K) fue de 9,64 puntos (rango intercuartílico 4-13). Los tratados con antipalúdicos antes del diagnóstico de LES tenían una mediana de SLEDAI en la primera visita de 5, frente a 8 en los que no los tomaban (p = 0,02)., Conclusiones: RELES constituye la primera cohorte de pacientes con LES recogidos desde el momento del diagnóstico en España. La presencia de anti-DNA se ha relacionado con manifestaciones graves como nefritis y anemia hemolítica. El tratamiento con antipalúdicos antes del diagnóstico se asoció con una enfermedad menos activa al comienzo., [Abstract] Introduction: Patient registries are useful tools for assessing rare diseases. Our objective is to present the Spanish registry of patients with systemic lupus erythematosus (Registro español de pacientes con lupus eritematoso sistémico, RELES)., Patients and methods: RELES was started in 2008 as an observational, prospective, multicentre cohort registry that included patients from the time they were diagnosed. The registry's objective is to analyse the incidence and noninflammatory complications of systemic lupus erythematosus (SLE). The departments of internal medicine of 38 Spanish hospitals participate in this registry., Results: A total of 298 patients with a mean age of 40.8 ± 15.7 years were included, 88.9% of whom were women and 85.6% of whom were white. In the first visit, there was a predominance of joint manifestations (74.5%). One hundred and seventy-seven patients (59.4%) were positive for anti-native DNA. In these patients, there was a higher rate of lupus nephritis (26.7% vs. 14%, p=.009; relative risk [RR], 1.33), haemolytic anaemia (13.6% vs. 4.1%, p=.07; RR, 1.46) and lymphopenia (55.4% vs. 43.8%, p=.05; RR, 1.21). The median Systemic Lupus Erythematosus Disease Activity Index 2000 (SLEDAI 2 K) score was 9.64 points (interquartile range, 4-13). The patients treated with antimalarial drugs before the diagnosis of SLE had a median SLEDAI score in the first visit of 5, compared with 8 for those who were not treated with these drugs (p=.02)., Conclusions: RELES constitutes the first Spanish patient cohort with SLE recorded from the time of the diagnosis. The presence of anti-DNA has been related to severe manifestations such as nephritis and haemolytic anaemia. Treatment with antimalarial drugs before the diagnosis was associated with less active disease at the initial presentation.

Aim: To study the influence of prednisone dose during the first month after systemic lupus erythematosus (SLE) diagnosis (prednisone-1) on glucocorticoid burden during the subsequent 11 months (prednisone-2–12). Methods: 223 patients from the Registro Español de Lupus Eritematoso Sistémico inception cohort were studied. The cumulative dose of prednisone-1 and prednisone-2–12 were calculated and recoded into a four-level categorical variable: no prednisone, low dose (up to 7.5 mg/day), medium dose (up to 30 mg/day) and high dose (over 30 mg/day). The association between the cumulative prednisone-1 and prednisone-2–12 doses was tested. We analysed whether the four-level prednisone-1 categorical variable was an independent predictor of an average dose >7.5 mg/day of prednisone-2–12. Adjusting variables included age, immunosuppressives, antimalarials, methyl-prednisolone pulses, lupus nephritis and baseline SLE Disease Activity Index (SLEDAI). Results: Within the first month, 113 patients (51%) did not receive any prednisone, 24 patients (11%) received average low doses, 46 patients (21%) received medium doses and 40 patients (18%) received high doses. There was a strong association between prednisone-1 and prednisone-2–12 dose categories (p7.5 mg/day, while patients receiving low-dose prednisone-1 were not (adjusted OR 1.4, 95% CI 0. 0.38 to 5.2). If the analysis was restricted to the 158 patients with a baseline SLEDAI of =6, the model did not change. Conclusion: The dose of prednisone during the first month after the diagnosis of SLE is an independent predictor of prednisone burden during the following 11 months.

Shrinking lung syndrome (SLS) is a rare and less known complication mainly associated with systemic lupus erythematosus (SLE). In this study, we analyze the clinical features, investigation findings, approaches to management, and outcome in a case series of 9 adult patients with SLE and SLS diagnosed during a 35-year period in 3 referral tertiary care hospitals in Spain. Additionally, we reviewed 80 additional cases previously reported (PubMed 1965-2015). These 80 cases, together with our 9 patients, form the basis of the present analysis. The overall SLS prevalence in our SLE population was 1.1% (9/829). SLS may complicate SLE at any time over its course, and it usually occurs in patients without previous or concomitant major organ involvement. More than half of the patients had inactive lupus according to SELENA-systemic lupus erythematosus disease activity index (SLEDAI) scores. Typically, it presents with progressive exertional dyspnea of variable severity, accompanied by pleuritic chest pain in 76% of the cases. An important diagnostic delay is common. The diagnostic tools that showed better yield for SLS detection are the imaging techniques (chest x-ray and high-resolution computed tomography) along with pulmonary and diaphragmatic function tests. Evaluation of diaphragm dome motion by M-mode ultrasonography and phrenic nerve conduction studies are less useful. There are no standardized guidelines for the treatment of SLS in SLE. The majority of patients were treated with medium or high doses of glucocorticoids. Several immunosuppressive agents have been used in conjunction with steroids either if the patient fails to improve or since the beginning of the treatment. Theophylline and beta-agonists, alone or in combination with glucocorticoids, have been suggested with the intent to increase diaphragmatic strength. The overall long-term prognosis was good. The great majority of patients had significant clinical improvement and stabilization, or mild to moderate improvement on pulmonary function tests. The mortality rate was very low.

Behçet disease is a systemic form of vasculitis which presents with neurological symptoms with a frequency varying between 16 and 40%. Involvement of the parenchyma has been found to worsen the prognosis in patients with neuroBehçet (NB).To review the clinical features and course of patients with NB involving the parenchyma of the central nervous system (CNS).Seven patients with Behçet disease and neurological localizing signs were seen in our hospital between 1989 and 1996. The initial diagnosis was of ischemic ictus in five of the seven patients. Both neuroimaging studies and investigation of the cerebrospinal fluid were always pathological in all cases. Vascular studies (arteriography and echo-Doppler of the supra-aortic trunks) were normal. One patient died. Four patients had serious sequelae following treatment.NB should be included in the different diagnosis of ictus. Involvement of the parenchyma of the CNS was accompanied by lymphocytic meningitis, perhaps also leading to a worse functional prognosis.

Introducción: la enfermedad de Behçet es una vasculitis sistémica que presenta clínica neurológica con una frecuencia que varía entre el 16 y el 40%. Se ha relacionado la afectación parenquimatosa con un peor pronóstico en los pacientes con neurobehçet (NB). Objetivo: revisar la clínica y evolución de los pacientes con NB y afectación parenquimatosa del sistema nervioso central (SNC). Casos clínicos. Siete pacientes con enfermedad de Behçet y focalidad neurológica fueron atendidos en nuestro centro entre 1989 y 1996. El diagnóstico inicial fue de ictus isquémico en cinco de los siete pacientes. Tanto los estudios de neuroimagen como los del líquido cefalorraquídeo resultaron siempre patológicos. Los estudios vasculares (arteriografía y eco-Doppler de troncos supraórticos) fueron normales. Un enfermo fue éxitus letalis. Cuatro pacientes presentaron secuelas tras el tratamiento. Conclusión: el NB ha de formar parte del diagnóstico diferencial del ictus. La afectación parenquimatosa del SNC se acompaña de meningitis linfocitaria y puede, además, condicionar un peor pronóstico funcional.

Objective To compare the main characteristics of two inception cohorts (Italian [ITC] and Spanish [SPC]) cohorts of patients with systemic lupus erythematosus (SLE) at the time of diagnosis and at one year of follow-up. Methods Demographic, clinical and immunological characteristics, and treatments at SLE diagnosis and at 12 months of follow-up of ITC and SPC were compared. Results One hundred and sixty-four patients in the ITC and 231 patients in the SPC were compared. the patients from ITC were younger at SLE diagnosis (41.1±15.0 years vs. 46.4±15.6 years; p

Because doctors are reluctant to diagnose systemic lupus erythematosus (SLE) in elderly patients, the initial diagnosis in this age group is usually tardy. Furthermore, the presenting manifestations in these patients are commonly atypical. We describe a 72-year-old woman in whom chronic ascites with antiphospholipid antibodies was the initial predominant manifestation of subsequently fatal SLE. Only 13 cases of chronic lupus ascites have been reported in the English and French literature. Our patient represents the first case reported in an elderly person.

16 p.-9 fig., C4b-binding protein (C4BP) is a well-known regulator of the complement system that holds additional and important activities unrelated to complement inhibition. Recently, we have described a novel immunomodulatory activity in the minor C4BP(β-) isoform directly acting over inflammatory phagocytes. Here we show that incorporation of the β-chain to the C4BP α-chain oligomer interferes with this immunomodulatory activity of C4BP. Moreover, an oligomeric form including only the complement control protein 6 (CCP6) domain of the C4BP α-chain (PRP6-HO7) is sufficient to “reprogram” monocyte-derived DCs (Mo-DCs) from a pro-inflammatory and immunogenic phenotype to an anti-inflammatory and tolerogenic state. PRP6-HO7 lacks complement regulatory activity but retains full immunomodulatory activity over inflammatory Mo-DCs induced by TLRs, characterized by downregulation of relevant surface markers such as CD83, HLA-DR, co-stimulatory molecules such as CD86, CD80 and CD40, and pro-inflammatory cytokines such as IL-12 and TNF-α. Furthermore, PRP6-HO7-treated Mo-DCs shows increased endocytosis, significantly reduced CCR7 expression and CCL21-mediated chemotaxis, and prevents T cell alloproliferation. Finally, PRP6-HO7 shows also full immunomodulatory activity over Mo-DCs isolated from lupus nephritis patients with active disease, even without further pro-inflammatory stimulation. Therefore PRP6-HO7, retaining the immunomodulatory activity of C4BP(β-) and lacking its complement regulatory activity, might represent a promising and novel alternative to treat autoimmune diseases., We thank CERCA Programme/Generalitat de Catalunya for institutional support. This work was supported by the Ministerio de Ciencia, Innovación y Universidades (Madrid, Spain) (grants FIS-ISCIII PI20/00464, PI16/00377 and DTS20/00016), and the “Departament de Recerca i Universitats de la Generalitat de Catalunya” (grants 2019PROD00081 and 2017SGR291), all co-funded by FEDER funds/European Regional Development Fund (ERDF)-a way to build Europe-. Dr. Vega is supported by the Spanish “Ministerio de Ciencia, Innovación y Universidades/FEDER” [RTI2018-102242-B-I00]. Dr. Rodriguez de Córdoba is supported by the Spanish “Ministerio de Economia y Competitividad/FEDER [SAF2015-66287-R]. Dr. Vega and Dr. Rodriguez de Córdoba are also funded by the Autonomous Region of Madrid [S2017/BMD-3673] and the European Commission – NextGenerationEU through CSIC’s Global Health Platform (“PTI Salud Global”) [SGL2103020].