Budget impact analysis of Subcutaneous Belimumab in patients with systemic Lupus Erythematosus in Spain

Objective: To evaluate the budget impact and the direct costs of the introduction of subcutaneous belimumab (SC) into the Spanish National Health Service (NHS) for patients with systemic lupus erythematosus (SLE) in Spain. Methods: This study was conducted from the Spanish NHS perspective with a time horizon of 3 years. The budget impact analysis compared the cost difference between two scenarios: current market (standard therapy (ST) and intravenous belimumab (IV)) and other market in which patients switched from belimumab IV to belimumab SC until reaching 17% of the total market share. The eligible population was calculated to receive treatment with belimumab, applying the EPISER (study of the prevalence of rheumatic diseases in adult population in Spain) prevalence (91 per 100,000 inhabitants), Autoimmune Systemic Diseases Study Group (GEAS) incidence (2 per 100,000 inhabitants), and the risk of annual mortality to the Spanish adult population. Patients with severe active lupus nephritis and with severe active CNS lupus were excluded. Patients' characteristics, flare rates and severity, and healthcare resource consumption were evaluated based on data from the literature and interviews with an expert panel. A sensitivity analysis was performed. Results: Currently, there is an estimated 34,697 adult patients with SLE in Spain and 3849 patients who are eligible to be treated with belimumab. The introduction of belimumab SC into the Spanish NHS could generate savings in direct healthcare costs of 6 million euros over the 3 years. Conclusion: The introduction of belimumab SC shows direct savings for the Spanish NHS. These savings could contribute to sustainability and decision-making

Insights into the procoagulant profile of patients with systemic lupus erythematosus without antiphospholipid antibodies

We aimed to identify the key players in the prothrombotic profile of patients with systemic lupus erythematosus (SLE) not mediated by antiphospholipid antibodies, as well as the potential utility of global coagulation tests to characterize hemostasis in these patients. Patients with SLE without antiphospholipid antibodies and without signs of thrombosis were included. The kinetics of clot formation were determined by ROTEM®. Platelet activation markers were determined by flow cytometry. Thrombin generation associated with Neutrophil Extracellular Traps (NETs) and microparticles (MPs) was measured by calibrated automated thrombogram (CAT). The plasma levels of PAI-1 were also determined. ROTEM® showed a procoagulant profile in SLE patients. SLE patients had activated platelets and more leukocyte/platelet aggregates at basal conditions. The plasma PAI-1 and platelet aggregates correlated with several ROTEM® parameters. The thrombin generation associated withthe tissue factor (TF) content of MPs and with NETs was increased. Our results suggest the utility of global tests for studying hemostasis in SLE patients because they detect their procoagulant profile, despite having had neither antiphospholipid antibodies nor any previous thrombotic event. A global appraisal of hemostasis should, if possible, be incorporated into clinical practice to detect the risk of a thrombotic event in patients with SLE and to consequently act to prevent its occurrenceThis work was supported by grant from the FIS-FONDOS FEDER (PI19/00772, NVB). E.M.M. holds a predoctoral fellowship from Fundación Española de Trombosis y Hemostasia (FETH-SETH

Current clinical spectrum of common variable immunodeficiency in Spain: The multicentric nationwide GTEM-SEMI-CVID registry

Common variable immunodeficiency (CVID) constitutes a heterogenic group of primary immunodeficiency disorders with a wide-ranging clinical spectrum. CVID-associated non-infectious morbidity constitutes a major challenge requiring a full understanding of its pathophysiology and its clinical importance and global variability, especially considering the broad clinical, genetic, and regional heterogeneity of CVID disorders. This work aimed to develop a nationwide, multicenter, retrospective study over a 3-year period describing epidemiological, clinical, laboratory, therapeutic, and prognostic features of 250 CVID patients in Spain. The mean diagnostic delay was around 10 years and most patients initially presented with infectious complications followed by non-infectious immune disorders. However, infectious diseases were not the main cause of morbimortality. Non-infectious lung disease was extraordinarily frequent in our registry affecting approximately 60% of the patients. More than one-third of the patients in our cohort showed lymphadenopathies and splenomegaly in their follow-up, and more than 33% presented immune cytopenias, especially Evans' syndrome. Gastrointestinal disease was observed in more than 40% of the patients. Among biopsied organs in our cohort, benign lymphoproliferation was the principal histopathological alteration. Reaching 15.26%, the global prevalence of cancer in our registry was one of the highest reported to date, with non-Hodgkin B lymphoma being the most frequent. These data emphasize the importance of basic and translational research delving into the pathophysiological pathways involved in immune dysregulation and diffuse lymphocytic infiltration. This would reveal new tailored strategies to reduce immune complications, and the associated healthcare burden, and ensure a better quality of life for CVID patients

IgG4-related disease: results from a multicenter Spanish registry

IgG4-related disease (IgG4-RD) is a rare entity consisting of inflammation and fibrosis that has been described in multiple organs. Concrete diagnostic criteria have been established recently and there is a lack of large series of patients.To describe the clinical presentation, histopathological characteristics, treatment and evolution of a series of IgG4-RD Spanish patients.A retrospective multicenter study was performed. Twelve hospitals across Spain included patients meeting the current 2012 consensus criteria on IgG4-RD diagnosis.Fifty-five patients were included in the study, 38 of whom (69.1%) were male. Median age at diagnosis was 53 years. Thirty (54.5%) patients were included in the Histologically Highly Suggestive IgG4-RD group and 25 (45.5%) in the probable IgG4-RD group. Twenty-six (47.3%) patients had more than 1 organ affected at presentation. The most frequently affected organs were: retroperitoneum, orbital pseudotumor, pancreas, salivary and lachrymal glands, and maxillary sinuses.Corticosteroids were the mainstay of treatment (46 patients, 83.6%). Eighteen patients (32.7%) required additional immunosuppressive agents. Twenty-four (43.6%) patients achieved a complete response and 26 (43.7%) presented a partial response (<50% of regression) after 22 months of follow-up. No deaths were attributed directly to IgG4-RD and malignancy was infrequent.This is the largest IgG4-RD series reported in Europe. Patients were middle-aged males, with histologically probable IgG4-RD. The systemic form of the disease was frequent, involving mainly sites of the head and abdomen. Corticosteroids were an effective first line treatment, sometimes combined with immunosuppressive agents. Neither fatalities nor malignancies were attributed to IgG4-RD

Importancia de la asociación entre la enfermedad de Kikuchi y el lupus erimatoso sistémico

Necrotizing histiocytic lymphadenopathy or Kikuchi's Disease (KD) is a rare clinical entity that is occasionally associated with systemic lupus erythematosus (SLE). The histologic features of the disorder have been considered indistinguishable to lymphadenitis in patients with SLE, and the clinical symptoms of the two disorders share common features. This article presents a clinical case of a 23-year-old woman with a history of KD who later developed SLE.La linfadenopatía histiocítica necrotizante o enfermedad de Kikuchi (EK) es una entidad clínica que se observa con poca frecuencia y que en ocasiones se asocia con lupus eritematoso sistémico (LES). Se ha considerado que las características histológicas del trastorno son indistinguibles de las de la linfadenitis en sujetos con LES, y los síntomas clínicos de los dos trastornos comparten características comunes. En este artículo se presenta un caso clínico de una mujer de 23 años con antecedente de EK que posteriormente desarrolla LES.

Biological sequential therapy with rituximab-belimumab in patients with recurrent lupus nephritis

Sin financiaciónNo data JCR 2021UE

Consenso de expertos sobre el diagnóstico integrado de la enfermedad de Castleman idiopática multicéntrica

[EN]: Idiopathic multicentric Castleman disease (iMCD) is rare. The differential diagnosis includes inflammatory, autoimmune and neoplastic disease. The identification of the histopathological features of Castleman disease in the lymph node is the main diagnostic criterion. Fifty-three experts from three medical societies (SEMI, SEHH and SEAP) have created a multi-disciplinary consensus document in order to standardise the diagnosis of Castleman disease. Using the Delphi method, specific recommendations for the initial clinical, laboratory and imaging studies have been made for an integrated diagnosis of iMCD as well as for the best way to obtain samples for histopathological confirmation, correct laboratory procedure and interpretation and reporting of results.[ES]: La enfermedad de Castleman multicéntrica idiopática (ECMi) es una patología infrecuente. El diagnóstico diferencial incluye patología inflamatoria, autoinmune y neoplásica. El estudio anatomopatológico del ganglio linfático y la identificación de las características histopatológicas de la enfermedad de Castleman constituyen un criterio principal para el diagnóstico. Con el objetivo de estandarizar el proceso diagnóstico de esta patología, se ha desarrollado un documento de consenso multidisciplinario con la participación de 53 expertos de tres sociedades médicas (Sociedad Española de Medicina Interna [SEMI], Sociedad Española de Hematología y Hemoterapia [SEHH] y Sociedad Española de Anatomía Patológica [SEAP]). Mediante el método Delphi se han validado las recomendaciones específicas para el diagnóstico integrado de la ECMi con respecto a los estudios clínicos, de laboratorio y de imagen necesarios en el abordaje inicial del paciente y las recomendaciones acerca de la mejor obtención de muestras para confirmación histopatológica, así como procedimientos de laboratorio para el estudio de las muestras, interpretación de resultados y emisión de un informe anatomopatológico.EUSA Pharma supported this work

Prevalence, risk factors and echocardiographic predictors of pulmonary hypertension in systemic lupus erythematosus: towards a screening protocol

Background Systemic lupus erythematosus (SLE) significantly affects the lungs and heart, and pulmonary hypertension (PH) is a severe manifestation that leads to considerable morbidity and mortality.Objectives We aimed to determine the prevalence and risk factors of probable SLE-PH, assess the main echocardiographic predictors and develop a potential screening strategy.Methods A prospective single-centre study was conducted on 201 patients with SLE who underwent transthoracic echocardiography. Patients meeting PH criteria were referred for right heart catheterisation (RHC).Results Among patients, 88.56% were women, 85.57% were of Spanish origin and 43.78% had structural heart disease. Out of these, 16 (7.96%) had intermediate or high probability criteria for PH according to European Society of Cardiology (ESC) 2022. Six RHCs confirmed PH with a prevalence of 2.99% for SLE-PH and 1.99% for SLE-pulmonary arterial hypertension (PAH).Key risk factors Key risk factors included age, cardiorespiratory symptoms, serositis, anti-Ro, cardiac biomarkers and altered pulmonary function tests (PFTs). PH was linked to a higher Systemic Lupus International Collaborative Clinics/American College of Rheumatology Damage Index (SDI) (mean SDI 4.75 vs 2.05, p&lt;0.001) and increased mortality risk in a 2-year follow-up (12.50% vs 1.08%, p=0.002).Conclusion In our cohort, 7.96% of patients with SLE had an intermediate or high PH probability. By RHC, six patients (2.99%) met the ESC/European Respiratory Society criteria for PH and four (1.99%) for PAH. The main risk factors were older age, cardiorespiratory symptoms, serositis, anti-Ro, cardiac biomarkers and altered PFTs. PH was a severe SLE complication, suggesting the need for earlier diagnosis through data-driven screening to reduce associated morbidity and mortality