Preventing organ damage in systemic lupus erythematosus: the impact of early biological treatment.

INTRODUCTION: One of the most important aims in the management of systemic lupus erythematosus (SLE) is to avoid or delay the accumulation of organ damage. The first five years after diagnosis are crucial for prognosis. AREAS COVERED: This manuscript reviews available data on organ damage accrual in SLE and early therapeutic intervention as a possible strategy to prevent its long-term accrual. EXPERT OPINION: Organ damage can be minimized by controlling disease activity and risk of flares, reducing the dose of glucocorticoids, and ensuring a proper therapeutic intervention with an early introduction of the right therapies. The current standard treatment cannot provide clinical remission in all patients with SLE. Therefore, there is a clinical need for introducing new therapeutic strategies able to achieve the main therapeutic objectives. The addition of biologic and other therapeutic agents to the standard of care is effective for controlling disease activity and for preventing severe flares, enabling a reduced use of glucocorticoids, and presumably reducing organ damage progression. Considering its efficacy and safety, early inclusion of biologic agents in the first lines of the treatment algorithm, at least in certain patients, could be considered as an innovative treatment approach to decrease disease burden in SLE patients.

Recommendations for prevention of infection in systemic autoimmune rheumatic diseases.

OBJECTIVES: To develop recommendations for the prevention of infection in adult patients with systemic autoimmune rheumatic diseases (SARD). METHODS: Clinical research questions relevant to the objective of the document were identified by a panel of experts selected based on their experience in the field. Systematic reviews of the available evidence were conducted, and evidence was graded according to the Scottish Intercollegiate Guidelines Network criteria. Specific recommendations were made. RESULTS: Five questions were selected, referring to prevention of infection by Pneumocystis jirovecii with trimethoprim/sulfamethoxazole, primary and secondary prophylactic measures against hepatitis B virus, vaccination against human papillomavirus, vaccination against Streptococcus pneumoniae and vaccination against influenza virus, making a total of 18 recommendations, structured by question, based on the evidence found for the different SARD and/or expert consensus. CONCLUSIONS: There is enough evidence on the safety and efficacy of vaccinations and other prophylactic measures against the microorganisms reviewed in this document to specifically recommend them for patients with SARD.

Recommendations for prevention of infection in systemic autoimmune rheumatic diseases. / Recomendaciones SER sobre prevención de infección en enfermedades reumáticas autoinmunes sistémicas.

OBJECTIVES: To develop recommendations for the prevention of infection in adult patients with systemic autoimmune rheumatic diseases (SARD). METHODS: Clinical research questions relevant to the objective of the document were identified by a panel of experts selected based on their experience in the field. Systematic reviews of the available evidence were conducted, and evidence was graded according to the Scottish Intercollegiate Guidelines Network criteria. Specific recommendations were made. RESULTS: Five questions were selected, referring to prevention of infection by Pneumocystis jirovecii with trimethoprim/sulfamethoxazole, primary and secondary prophylactic measures against hepatitis B virus, vaccination against human papillomavirus, vaccination against Streptococcus pneumoniae and vaccination against influenza virus, making a total of 18 recommendations, structured by question, based on the evidence found for the different SARD and/or expert consensus. CONCLUSIONS: There is enough evidence on the safety and efficacy of vaccinations and other prophylactic measures against the microorganisms reviewed in this document to specifically recommend them for patients with SARD.

Comorbidities (excluding lymphoma) in Sjögren's syndrome.

The information about comorbidities (excluding lymphoma) in primary Sjögren's syndrome (pSS) is relatively scarce. Cardiovascular disease, infections, musculoskeletal conditions or malignancy are likely the most relevant comorbid conditions in pSS. Different infections (particularly oral candidal infections) and fibromyalgia are extremely frequent in the daily clinical practice. On the other hand, the incidence of cardiovascular events and cancer in pSS is low, so information about them comes from large epidemiological studies or meta-analysis. For this reason, preclinical vascular disease is investigated by different techniques, demonstrating the presence of early atherosclerosis in pSS patients. Coronary events could be slightly more frequent in pSS than in the general population. The overall risk of malignancy in pSS patients seems to be slightly increased, likely due to excess occurrence of lymphoma. An association between pSS and thyroid cancer might exist, although it should be confirmed by further investigations.

Contributions of the lupus register of the Spanish Society of Rheumatology (RELESSER) to the knowledge of systemic lupus erythematosus in Spain. / Aportaciones del registro de lupus de la Sociedad Española de Reumatología (RELESSER) al conocimiento del lupus eritematoso sistémico en España.

The lupus register of the Spanish Society of Rheumatology (RELESSER) is a multicentre register of patients with systemic lupus erythematosus (SLE) under follow-up by Spanish Rheumatology Services. It contains data on a total of 4024 patients with SLE. So far, 14 studies have been published from the transversal phase of RELESSER. Here we report the more relevant contributions of those studies, according to the authors' perspective, concerning cumulative clinical characteristics, level of activity, treatments, refractory disease, damage and mortality. We also review the main results of the analysis regarding incomplete SLE, lupus nephritis, respiratory manifestations, cardiovascular disease, serious infection, malignancies, fibromyalgia, SLE in males, SLE in Hispanics and juvenile-onset SLE, comparing the main characteristics of each subgroup to the global cohort. RELESSER has become one of the most important clinical SLE registers around the world, with a high yield in terms of knowledge generation about the disease in Spain, also useful for the entire scientific community.

Guía de práctica clínica para el lupus eritematoso sistémico: recomendaciones para el abordaje clínico general / Clinical practice guidelines for systemic lupus erythematosus: Recommendations for general clinical management

El lupus eritematoso sistémico (LES) es una compleja enfermedad reumática multisistémica de origen autoinmune con una morbimortalidad importantes. Es una de las enfermedades autoinmunes más comunes, con una prevalencia estimada de 20-150/100.000 habitantes. El espectro clínico del LES es amplio y variable tanto en manifestaciones clínicas como en gravedad. Esto ha hecho que el Ministerio de Sanidad, Servicios Sociales e Igualdad haya promovido y financiado el desarrollo de una guía de práctica clínica (GPC) sobre el LES dentro del Programa de GPC en el Sistema Nacional de Salud que coordina GuiaSalud. Esta GPC pretende ser el instrumento de referencia en el Sistema Nacional de Salud para dar soporte a la gestión clínica integral de las personas con LES por parte de todos los profesionales sanitarios implicados, independientemente de la especialidad y el nivel asistencial, contribuyendo a homogeneizar y mejorar la calidad de las decisiones clínicas en nuestro entorno con el objetivo de mejorar los resultados de salud de las personas afectadas. En el presente artículo se presentan y discuten las recomendaciones formuladas en relación con el manejo general de la enfermedad, en concreto, el seguimiento clínico, el abordaje terapéutico general, los estilos de vida saludables, la fotoprotección y la formación para los pacientes. Recomendaciones basadas en la mejor evidencia científica disponible y en la discusión y consenso de grupos de expertos (AU)

Systemic lupus erythematosus (SLE) is a complex rheumatic multisystemic disease of autoimmune origin with significant potential morbidity and mortality. It is one of the most common autoimmune diseases with an estimated prevalence of 20-150 cases per 100,000 inhabitants. The clinical spectrum of SLE is wide and variable both in clinical manifestations and severity. This prompted the Spanish Ministry of Health, Social Services and Equality to promote and fund the development of a clinical practice guideline (CPG) for the clinical care of SLE patients within the Programme of CPG in the National Health System which coordinates GuiaSalud. This CPG is is intended as the reference tool in the Spanish National Health System in order to support the comprehensive clinical management of people with SLE by all health professionals involved, regardless of specialty and level of care, helping to standardize and improve the quality of clinical decisions in our context in order to improve the health outcomes of the people affected. The purpose of this document is to present and discuss the rationale of the recommendations on the general management of SLE, specifically, clinical follow-up, general therapeutic approach, healthy lifestyles, photoprotection, and training programmes for patients. These recommendationsare based on the best available scientific evidence, on discussion and the consensus of expert groups (AU)

[Clinical practice guidelines for systemic lupus erythematosus: Recommendations for general clinical management]. / Guía de práctica clínica para el lupus eritematoso sistémico: recomendaciones para el abordaje clínico general.

Systemic lupus erythematosus (SLE) is a complex rheumatic multisystemic disease of autoimmune origin with significant potential morbidity and mortality. It is one of the most common autoimmune diseases with an estimated prevalence of 20-150 cases per 100,000 inhabitants. The clinical spectrum of SLE is wide and variable both in clinical manifestations and severity. This prompted the Spanish Ministry of Health, Social Services and Equality to promote and fund the development of a clinical practice guideline (CPG) for the clinical care of SLE patients within the Programme of CPG in the National Health System which coordinates GuiaSalud. This CPG is is intended as the reference tool in the Spanish National Health System in order to support the comprehensive clinical management of people with SLE by all health professionals involved, regardless of specialty and level of care, helping to standardize and improve the quality of clinical decisions in our context in order to improve the health outcomes of the people affected. The purpose of this document is to present and discuss the rationale of the recommendations on the general management of SLE, specifically, clinical follow-up, general therapeutic approach, healthy lifestyles, photoprotection, and training programmes for patients. These recommendations are based on the best available scientific evidence, on discussion and the consensus of expert groups.

Lo mejor del año en lupus eritematoso sistémico / What is new in systemic lupus erythematosus

El lupus eritematoso sistémico es una enfermedad reumática sistémica enormemente heterogénea, con múltiples posibles manifestaciones de patogenia diversa, como se ilustra en esta revisión sobre las novedades más relevantes concernientes a esta compleja enfermedad autoinmune. Se revisan aspectos como la patogenia de la anemia crónica asociada al lupus eritematoso sistémico, la estimación del riesgo cardiovascular, el síndrome antifosfolipídico, la predicción del daño acumulado y los avances más recientes en el tratamiento, incluyendo los tolerógenos y las terapias biológicas. También se revisan las contribuciones más relevantes en torno a las terapias clásicas, como la optimización del uso de los glucocorticoides y los antipalúdicos, así como el papel que pueda desempeñar la vitamina D (AU)

Systemic lupus erythematosus is a heterogeneous rheumatic systemic disease with extremely varied clinical manifestations and a diverse pathogenesis, as illustrated in this review on the most relevant new knowledge related to the disease. Topics such as anemia, pathogenesis, cardiovascular risk assessment, antiphospholipid syndrome, prediction of damage and recent advances in treatment, including tolerogenic and biological agents, are discussed. Relevant contributions regarding classical therapies such as corticosteroid and antimalarials and their optimal use, as well as the roll of vitamin D, are also referred (AU)

What is new in systemic lupus erythematosus.

Systemic lupus erythematosus is a heterogeneous rheumatic systemic disease with extremely varied clinical manifestations and a diverse pathogenesis, as illustrated in this review on the most relevant new knowledge related to the disease. Topics such as anemia, pathogenesis, cardiovascular risk assessment, antiphospholipid syndrome, prediction of damage and recent advances in treatment, including tolerogenic and biological agents, are discussed. Relevant contributions regarding classical therapies such as corticosteroid and antimalarials and their optimal use, as well as the roll of vitamin D, are also referred.

Tratamiento de las vasculitis sistémicas asociadas a ANCA / Treatment of ANCA-associated systemic vasculitis

El tratamiento de las vasculitis sistémicas ha experimentado cambios sustanciales en los últimos años. La ciclofosfamida sigue teniendo un papel crucial en la inducción de remisión en formas severas, reduciendo considerablemente la mortalidad. Sin embargo, su empleo conlleva una importante toxicidad a largo plazo y el acúmulo de morbilidad derivada de un control subóptimo del proceso. Se han desarrollado estrategias para limitar la exposición al fármaco y minimizar su toxicidad, como son el uso de pulsos endovenosos como alternativa a la vía oral y la estrategia secuencial. Tanto para inducir remisión en casos no severos como para el mantenimiento de remisión se preconiza el empleo de inmunosupresores alternativos, como son el metotrexate, la azatioprina o la leflunomide. En determinadas situaciones con compromiso vital puede recurrirse a opciones como la plasmaféresis o las inmunoglobulinas endovenosas. Las terapias biológicas suponen una alternativa prometedora, si bien su empleo actual debe restringirse a los casos refractarios (AU)

The treatment of systemic vasculitis has undergone important changes in recent years. Cyclophosphamide still plays a crucial role in the induction of remission in severe forms, reducing the mortality. However, its use entails a significant long-term toxicity and the accumulation of damage resulting from a sub-optimal control of the process. Strategies has been developed to limit exposure to the drug and minimize its toxicity, such as using intravenous pulses as an alternative to oral administration and a sequential strategy. Both induce remission in less severe cases and work also for the maintenance of remission; the use of alternative immunosuppressants, such as methotrexate, azathioprine or leflunomide has been advocated. In life-threatening situations, options such as plasmapheresis or intravenous inmunoglobulins are available. Biologic therapies are a promising alternative, but their use must be limited for now to refractory cases (AU)

[Treatment of ANCA-associated systemic vasculitis]. / Tratamiento de las vasculitis sistémicas asociadas a ANCA.

The treatment of systemic vasculitis has undergone important changes in recent years. Cyclophosphamide still plays a crucial role in the induction of remission in severe forms, reducing the mortality. However, its use entails a significant long-term toxicity and the accumulation of damage resulting from a sub-optimal control of the process. Strategies has been developed to limit exposure to the drug and minimize its toxicity, such as using intravenous pulses as an alternative to oral administration and a sequential strategy. Both induce remission in less severe cases and work also for the maintenance of remission; the use of alternative immunosuppressants, such as methotrexate, azathioprine or leflunomide has been advocated. In life-threatening situations, options such as plasmapheresis or intravenous inmunoglobulins are available. Biologic therapies are a promising alternative, but their use must be limited for now to refractory cases.