Pediatric systemic lupus erythematosus linkedin slideshare. Pathogenesis 20 and clinical features 1 introduction systemic lupus erythematosus sle is the prototypic multisystem autoimmune disorder with a broad spectrum of clinical presentations encompassing almost all organs and tissues. Systemic lupus erythematosus is an autoimmune disease that affects many systems, including the skin, musculoskel etal, renal, neuropsychiatric, hematologic, cardiovascular, pulmonary. Sle affects children in the same ways that it affects adults except the disease can be more severe and involve more organs. Systemic means that it affects many organs of the body. In this chapter we provide an overview of the clinical symptoms, laboratory findings and treatment for the following inflammatory diseases. Sle has been classically described as a prototypic autoimmune disease with a wide array of clinical manifestations and characterized by the production of autoantibodies to components of the cell nucleus. Demographic data, clinical features and disease activity were recorded.
Keywords pediatric systemic lupus erythematosus, systemic lupus. Multiple organ damage can ensue with renal and neurological involvement carrying the worse prognosis. The clinical presentation of pediatric systemic lupus erythematosus is highly variable because of the systemic nature of the disease. Patients with sle present in many different ways and therefore may first encounter the medical system in a number of different clinics, including dermatology, nephrology, neurology, haematology or rheumatology, in both adult and paediatric care settings. Disparities in outcomes among adults with systemic lupus erythematosus sle have been documented. Although it has the potential to affect any organ, clinically significant hepatic involvement is considered to be uncommon and liver dysfunction is not a diagnostic criteria. The disease can affect, for example, the kidneys, skin, blood cells, and nervous system. The word lupus is derived from the latin word for wolf and it refers to the characteristic butterflylike rash on the face, which is similar to the white markings on a wolfs face. Pediatric systemic lupus erythematosus in siriraj hospital. Discuss the treatment and adverse effects of medications used to treat sle. Systemic lupus erythematosus sle is a lifethreatening multisystem autoimmune disease that is more severe in patients of african ancestry and children, yet pediatric sle on the african continent has been understudied. Systemic lupus erythematosus in children pediatrics. We investigated associations between sociodemographic factors and volume of annual inpatient hospital admissions with hospitalization characteristics and poor outcomes among patients with childhoodonset sle. Bernstein, in comprehensive pediatric hospital medicine, 2007.
Attention is drawn to the difficulties that may be encountered in the positive identification and classification of many patients suspected of suffering from systemic lupus erythematosus. American college of rheumatology provisional criteria for. The wide acceptance and popularity of the first eular recommendations for its management, published in 2008,1 prompted the subsequent development of specific recommendations regarding monitoring, neuropsychiatric and renal disease, as well as for pregnancy and womens health in lupus. The management of pediatric systemic lupus erythematosus. Sle is an autoimmune condition marked by abnormalities of b and t lymphocytes and the. Share single hub and access point for paediatric rheumatology in europe was launched to optimise and disseminate management regimens for. Much of this is due to a lack of specific criteria, either clinical or pathologic, for the diagnosis of the disease. Systemic lupus erythematosus an overview sciencedirect.
Pediatric onset systemic lupus erythematosus sle is not uncommon and female to male ratio varies. The prevalence of pediatric systemic lupus erythematosus psle varies in different ethnic populations, with estimates of 0. Systemic lupus erythematosus sle is an autoimmune disease that affects various part of body. Autoimmune hemolytic anemia in systemic lupus erythematosus. Pediatric onset sle psle is associated with greater mortality and a higher rate of major organ involvement in. Childhoodonset sle is associated with higher morbidity and mortality than adultonset sle. To determine the flare rate and the change in safety of estrogens in lupus erythematosus. Systemic lupus erythematosus is a systemic autoimmune disease that can manifest an array of cardiac features including pericarditis, myocarditis, ischemic heart disease, valvular dysfunction, as well as cerebrovascular disease, vasculopathies, and thromboembolic disease 1,2,3. This podcast is an introduction to systemic lupus erythematosus. Systemic lupus erythematous sle is a systemic and complex disease that can involve multiple organs. We present below a seventeenyearold female patient, one month postpartum, who had findings consistent with an acute infectious pneumonia whom upon further query and passage of time was diagnosed with severe. Sle can be a mild disease of main skin or joint manifestations or might be a fatal condition. Today we will be discussing systemic lupus erythematosus, sle or simply lupus.
Pediatric rheumatologist at the university of alberta and stollery childrens hospital in edmonton, ab, canada. Apr 29, 2019 systemic lupus erythematosus sle is a rheumatic disease characterized by autoantibodies directed against selfantigens, immune complex formation, and immune dysregulation, resulting in damage to essentially any organ. May 24, 2016 this podcast is an introduction to systemic lupus erythematosus. He also used the term lupus erythematosus and published the fi rst illustrations in his atlas of skin diseases in 1856. Apr 29, 2019 the most important tool in the medical care of the patient with systemic lupus erythematosus sle is careful and frequent clinical and laboratory evaluation to tailor the patients medical regimen and to provide prompt recognition and treatment of disease flare, which is the cornerstone of successful intervention. Clinical and immunological profile of systemic lupus erythematosus. The objectives include recognize the signs and symptoms suggestive of sle, list the 11 classification criteria for sle, elicit a focused history, demonstrate a pertinent physical exam, and discuss investigations and management for patient with sle.
Pediatric systemic lupus erythematosus american academy. Prior studies have described the racialethnic disparities of sle. American college of rheumatologyarhp scientific meeting. Shrinking lung syndrome complicating pediatric systemic. Zhu j, wu f, huang x 20 agerelated differences in the clinical characteristics of systemic lupus erythematosus in children. Jul 18, 2019 the clinical presentation of pediatric systemic lupus erythematosus is highly variable because of the systemic nature of the disease.
Pediatric systemic lupus erythematosus psle is a rare condition, representing approximately. Ruperto n, ravelli a, cuttica r, espada g, ozen s, porras o, et al. Severe disease presentation and poor outcomes among. Systemic lupus erythematosus sle is an autoimmune disease causing inflammatory tissue damage. Pediatric sle patients have more severe disease at onset. The problem has been made more difficult by the recognition of a number of other syndromes. Pediatric systemic lupus erythematosus sle diagnostic. Sle is a challenging condition that presents unique issues in diagnosis and management. Race, ethnicity, and gender affect the severity of renal outcomes in patients with pediatric systemic lupus erythematosus. European evidencebased recommendations for diagnosis and. Outcomes in hospitalized pediatric patients with systemic. Acrendorsed criteria for rheumatic diseases below are a number of acrapproved criteria sets. Systemic lupus erythematosus an overview sciencedirect topics.
Most children and adolescents with systemic lupus erythematosus sle now survive into adulthood, leading the pediatric rheumatology community to focus on preventing longterm complications of sle. A healthy immune system protects your child from infection by attacking invaders such as viruses or other pathogens. Neuropsychiatric involvement in pediatric systemic lupus erythematosus. Pdf pediatric systemic lupus erythematosus in siriraj.
Juvenile systemic lupus erythematosus presenting as. Pediatric systemic lupus erythematosus childrens health. Nature vs nurture, hispanic and african american patients were found to have greater disease activity at an earlier age of onset compared with caucasians. This retrospective study included 25 patients diagnosed with psle and followed at the university of pisa. An update on treatment and management of pediatric systemic.
This retrospective study evaluated the medical charts of 336 childhoodonset systemic lupus erythematosus csle and 1830 adult sle asle patients followed in the same tertiary hospital. Severe disease presentation and poor outcomes among pediatric. The aim of this study was to identify variables to improve the diagnostic awareness and management of psle patients. Shrinking lung syndrome complicating pediatric systemic lupus.
Patterns and influence of familial autoimmunity in pediatric systemic. See information about the purpose of criteria sets, their development and validation, and the role of the acr in adopting them. A high prevalence of autoimmune disease ad has been documented in relatives of adult patients with systemic lupus erythematosus sle. Article information, pdf download for childonset systemic lupus. Pdf systemic lupus erythematosus in children and adolescents. Juvenile systemic lupus erythematosus jsle is defined as systemic lupus erythematosus sle with onset before 18 years of age and accounts for 15% of sle patients. Karim my, miranda lc, tench cm et al 2002 presentation and prognosis of the shrinking lung syndrome in systemic lupus erythematosus.
The aims of disease management with early immunosuppression are to decrease disease activity and improve quality of life. Cryptococcal meningitis in pediatric systemic lupus. Systemic lupus erythematosus sle in children pediatrics. Pdf pediatric systemic lupus erythematosus in siriraj hospital. A descriptive study of one hundred and one pediatric patients with systemic lupus erythematosus treated between july 1985 and march 2003 in department of pediatrics, faculty of medicine siriraj. Clinical and immunological aspects and outcome of a brazilian cohort of 414 patients with systemic lupus erythematosus sle. Fda approves first drug for pediatric patients with systemic. Childonset systemic lupus erythematosus is associated with a. Acr endorsed criteria american college of rheumatology. However, mortality rates and causes of death are under reported in the pediatric population, so that most of our understanding derived from. Lupus was fi rst recognised as a systemic disease with visceral manifestations by moriz kaposi 18371902. Median systemic lupus erythematosus disease activity index 2000 sledai2 k was comparable in csle and asle p 0. Systemic lupus erythematosus sle is a chronic, multisystem, autoimmune disease characterized by periods of increased disease activity caused by inflammation of blood vessels and connective tissue. Systemic lupus erythematosus sle is a rheumatic disease characterized by autoantibodies directed against selfantigens, immune complex formation, and immune dysregulation, resulting in damage to essentially any organ.
In the large multiethnic adult sle cohort of lupus in minority populations. Systemic lupus erythematosus sle is a chronic multisystem autoimmune disease associated with the production of autoantibodies. Understand the special needs of a pediatric patient who has sle. List the most common presenting symptoms of systemic lupus erythematosus sle in the pediatric population. The pulmonary manifestations of systemic lupus erythematosus can range in severity from mild to life threatening and can be particularly marked in women who are recently postpartum. The name systemic lupus erythematosus dates back to the early 20th century. Systemic lupus erythematosus in children and adolescents.
In this case report we present a 10yearold african american girl who presented with abnormal choreiform movements, headache, weight loss, and fatigue. Although regarded as a disease of adulthood, sle is also seen in children, and is associated with an increased risk for aggressive clinical course and major organ damage in comparison to adults. Systemic lupus erythematosus sle is a chronic autoimmune disease that can involve any organ system with a wide range of disease manifestations, and can lead to significant morbidity and even mortality. Pediatric systemic lupus erythematosus american academy of. The clinical presentation includes distinctive cutaneous lesions resembling those seen in systemic lupus erythematosus, hepatobiliary disease, and cytopenias, which disappear with the clearance of maternal autoantibodies. Vaccinations in juvenile chronic inflammatory diseases. Cryptococcal meningitis is an uncommon but often fatal complication of systemic lupus erythematosus sle. Juvenile systemic lupus erythematosus presenting as pancarditis. National assessment systemic lupus erythematosus disease activity index selena sledai score with disease flare in pediatric systemic lupus erythematosus psle.
Systemic lupus erythematosus is an autoimmune disease that affects many systems, including the skin, musculoskel etal, renal, neuropsychiatric, hematologic, cardiovascular, pulmonary, and. An unusual presentation of lupus in a pediatric patient. Pdf on oct 1, 2006, beth s gottlieb and others published systemic lupus erythematosus in children and adolescents find, read and cite all. Comorbidities of pediatric systemic lupus erythematosus. Th e systemic form was further established by osler in baltimore and jadassohn in vienna.
Systemic lupus erythematosus sle is a chronic multisystem disease characterized by diffuse vasculitis and inflammation of connective tissues that may lead to widespread organ damage affecting the kidneys, brain, and joints. In lupus, the bodys immune system, which normally functions to. Recognize the signs and symptoms suggestive of sle 2. Juvenile sle is known to be associated with a higher incidence of arthritis, nephritis.
Dec 27, 2019 fda approves first drug for pediatric patients with systemic lupus erythematosus from the food and drug administrations center for drug evaluation and research, division of pulmonary, allergy and rheumatology products, division of biometrics ii, division of pediatric and maternal health and office of pediatric therapeutics. Pediatric systemic lupus erythematosus psle is a rare condition, representing approximately 10% of sle cases. To clarify the risk of developing associated comorbidities after a diagnosis of sle in children, we used the national health insurance research database nhird in taiwan to investigate diseases experienced in these patients. Katarzyna gilekseibert, md abstract systemic lupus erythematosus sle is a chronic, compli cated and challenging disease to diagnose and treat. Evidencebased guidelines are sparse and management is often based on clinical expertise. Systemic lupus erythematosus diagnosis and management.
Pulmonary systemic lupus erythematosus mimicking a. Systemic lupus erythematosus sle or lupus is a disease in which the immune system is. Sep 30, 2016 systemic lupus erythematosus sle is a chronic multisystem autoimmune disorder in which 20 % of patients are diagnosed in childhood. Juvenile in spanish espanol download printfriendly pdf. Childhoodonset systemic lupus erythematosus csle is a severe, chronic, systemic autoimmune disease that has great impact on the child or young person affected. The disease activity was significantly increased and the damage index tended to increase in the josle cases compared to the jsle children fig. In children with lupus, the immune system mistakenly attacks the bodys own cells and tissue, causing. By using the pediatric health information system, we analyzed. The incidence and prevalence of neuropsychiatric syndromes in pediatric onset systemic lupus erythematosus. The disease is significantly more prevalent in women than men, with peak incidence in women of childbearing age. Systemic lupus erythematosus sle has variable presentation, course and prognosis. The pediatric rheumatology international trials organization criteria for the evaluation of response to therapy in juvenile systemic lupus. In children, it is fundamentally the same disease as in adults, with similar etiology, pathogenesis, clinical manifestations, and laboratory findings. Professor of pediatrics, albert einstein college of medicine.
An update on treatment and management of pediatric. Aug 17, 2017 diseases where ana is useful in diagnosis systemic lupus erythematosus 100% systemic sclerosis 6080% sjogrens syndrome 4070% dermatomyositis or polymyositis 3080% mixed connective tissue disease 100% autoimmune hepatitis 100%. Systemic lupus erythematosus lupus is a chronic autoimmune disease characterized by inflammation, pain and swelling. Pediatric systemic lupus erythematosus patients in south. The immune system attacks the body and creates inflammation in the skin, joints, kidneys, lungs, nervous system, and other organs of the body. Systemic lupus erythematosus sle or lupus is a disease in which the immune system is overactive and does not function properly.
Juvenile and juvenileonset systemic lupus erythematosus patients. The clinical presentation includes distinctive cutaneous lesions resembling those seen in systemic lupus erythematosus, hepatobiliary disease, and cytopenias, which disappear with the clearance of maternal. Basically, this is a multisystem inflammatory autoimmune disease thats caused by immunecomplex mediated damage. Chief, division of rheumatology, childrens hospital at montefiore, bronx, ny after completing this article, readers should be able to. Mortality related to pediatric systemic lupus erythematosus. Safety and immunogenicity of quadrivalent hpv vaccine in female systemic lupus erythematosus age 1226 years. Childhoodonset systemic lupus erythematosus csle is a rare, multisystem and potentially lifethreatening autoimmune disorder with significant associated morbidity. It affects multiple organs in different ways in different people and the cause is unknown. Fewer than 5% of patients who have isolated discoid sle will progress to sle. Systemic lupus erythematosus sle is a chronic multisystem autoimmune disorder in which 20 % of patients are diagnosed in childhood. Conclusions we identified that aiha was not a common condition in csle and asle, with distinct features characterized by a higher prevalenceseverity in children and concomitant constitutional symptoms in the majority of them. Neonatal lupus erythematosus is an uncommon syndrome, which is caused by transplacental passage of maternal autoantibodies to sjogrens syndrome a or b autoantigens. A closed renal biopsy is contraindicated in the presence of a prolonged bleeding time. Systemic lupus erythematosus sle is a multisystem autoimmune disorder characterized by widespread inflammation of the connective tissues and immune complexmediated vasculitis.
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