Discoid rash raised erythematous patches with keratotic scaling, follicular plugging, and atrophic scarring 3. This study aims to compare the performance among the american college of rheumatology acr 1997, the systemic lupus international collaborating clinics criteria slicc and the new european league against rheumatism eularacr criteria, in a csle. Systemic lupus erythematosus sle is a severe and chronic inflammatory autoimmune disorder that has multisystem affection. The slicc 2012 criteria may allow patients to be classified as having sle earlier in the disease course. Indeed clinical trials have had to add the requirement for the presence of a sle autoantibody when recruiting patients to optimize the likelihood of response to immunosuppressive therapy 14. Hydroxychloroquine is the cornerstone of treatment because it reduces disease flares and other constitutional symptoms. Systemic lupus erythematosus diagnosis and management bernard thong. It was felt that important control groups, including chronic cutaneous lupus, needed to be included in a. Validation of the 2017 weighted criteria compared to the. Toward new criteria for systemic lupus erythematosusa. Iraj salehiabari 2015, 2015 acrslicc revised criteria for diagnosis of systemic lupus erythematosus.
Systemic lupus erythematosus sle presents with nonspecific signs and symptoms that are also found in other conditions. Acr and the 2012 systemic lupus international cooperating clinics slicc sets of classi. Derivation and validation of the systemic lupus international. Listing a study does not mean it has been evaluated by the u. The disease can affect, for example, the kidneys, skin, blood cells, and nervous system. Application of the 2012 systemic lupus international collaborating. The systemic lupus international cooperating clinics slicc 2012 criteria increased sensitivity, but at the price of reduced specificity. Neuropsychiatric systemic lupus erythematosus npsle is a complex neurological disorder characterized by neuropsychological dysfunction. The diagnostic accuracies of the 2012 slicc criteria and the proposed eularacr criteria for systemic lupus erythematosus classification are comparable. 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. An epigeneticsbased hypothesis of autoantigen development in systemic lupus erythematosus author. Systemic lupus erythematosus sle is a chronic disease that causes inflammation in connective tissues, such as cartilage and the lining of blood vessels, which provide strength and flexibility to structures throughout the body. Diagnostic criteria for systemic lupus erythematosus.
With rare exception, the unifying laboratory abnormality is. Indeed, it is the diversity of presentation, accumulation of manifestations over time, and undulating disease course that challenge the most astute of clinicians. Systemic lupus erythematosus is a remarkable and challenging disorder. Our objective was to update the eular recommendations for the management of systemic lupus erythematosus sle, based on emerging new evidence. To date there are no specific classification criteria for childhoodonset systemic lupus erythematosus csle. The systemic lupus international collaborating clinics slicc group revised and validated the american college of rheumatology acr systemic lupus erythematosus sle classification criteria in order to improve clinical relevance, meet stringent methodology requirements, and incorporate new knowledge regarding the immunology of sle. All patients with lupus should receive education, counseling, and support. Its diversity of clinical features is matched by the complexity of the factors genetic, hormonal, and environmental that cause it, and the array of autoantibodies with which it is associated. Performance of the proposed acr eular classification. The most recent addendum to the classification criteria for sle dates from 2012, when the systemic lupus international collaborating clinics slicc group published a revision and validation of the acr criteria 5.
Correspondence to professor andrea doria, department of medicine, division of rheumatology, university of padua, padova 35128, italy. Comparison among acr1997, slicc and the new eularacr. In the validation cohort, the new criteria had a sensitivity of 96. How do patients with newly diagnosed systemic lupus.
Acr endorsed criteria american college of rheumatology. Systemic lupus erythematosus sle is a chronic autoimmune disease characterized by multisystemic involvement and diverse manifestations. If a patient meets at least four criteria, lupus can be diagnosed with 95% specificity and 85% sensitivity. In 2012, the slicc criteria were published in an attempt to improve clinical relevance of sle criteria. Higher interferon score and normal complement levels may. Thus, slicc12 criteria were applied to confirmed sle cases in our. Derivation and validation of systemic lupus international. The thorough heterogeneity among patients with systemic lupus erythematosus sle causes problems regarding diagnostic accuracy in clinical. Systemic lupus erythematosus, a manual, is a useful reference for specialists in the diagnosis and management of patients with sle, a tool for measurement of clinical activity for pharmaceutical development and basic research of the disease and a reference work for hospital libraries.
Systemic lupus erythematosus sle, is the most common type of lupus. Pediatric systemic lupus erythematosus american academy. The common manifestations are in the form of arthritis, skin rash, renal and hematological involvement along with fever and constitutional symptoms. Systemic lupus erythematosus sle is a complex autoim mune disease. Childhoodonset systemic lupus erythematosus clinically relevant improvement in children and adolescents. The slicc group is an international group of investigators dedicated to clinical research on sle. New therapeutic strategies in systemic lupus erythematosus.
Systemic lupus erythematosus is a multiorgan system autoimmune disease with clinical and serological heterogeneity. The acr 1997 criteria were criticized for their suboptimal sensitivity. The 2012 systemic lupus international collaborating clinics slicc classification criteria addressed many of these issues 9. Herein, we present the first independent evaluation of these criteria in comparison. Systemic lupus erythematosus sle is an autoimmune disorder characterized by antibodies to nuclear and cytoplasmic antigens, multisystem inflammation, protean clinical manifestations, and a relapsing and remitting course. More than 90% of cases of sle occur in women, frequently starting at childbearing age. Background in a joint effort, the european league against rheumatism eular and the american college of rheumatology acr recently proposed new criteria for the classification of systemic lupus erythematosus sle with the overarching goal to identify potential participants for clinical studies. The current treatment approach for systemic lupus erythematosus sle, as outlined in the recommendations by international medical associations including eular and the acr, is. Derivation and validation of systemic lupus international collaborating clinics classification criteria for systemic lupus erythematosus. Mucocutaneous and neuropsychiatric manifestations were added, as were hypocomplementemia and new antiphospholipid. Effect of curcumin on systemic lupus erythematosus the safety and scientific validity of this study is the responsibility of the study sponsor and investigators. This study aimed to evaluate manifestations at disease onset and to compare early sle manifestations. Systemic lupus erythematosus sle is an autoimmune disorder characterized by antibodies to nuclear and cytoplasmic antigens, multisystem inflammation, protean clinical manifestations, and a. Symptoms vary between people and may be mild to severe.
The diagnostic accuracies of the 2012 slicc criteria and. Risk factors for cataracts in a cohort of egyptian. Derivation and validation of the systemic lupus international collaborating clinics classification criteria for systemic lupus erythematosus. In 2012, the systemic lupus international collaborating clinics slicc developed and validated new sle classification criteria.
In this seminar we reflect on changes in its classification criteria. Systemic lupus erythematosus sle is a multiorgan autoimmune disease of unknown etiology with many clinical manifestations. Clinical criteria acute cutaneous lupus or subacute cutaneous lupus. 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. Different classification criteria for systemic lupus erythematosus sle have been proposed over many years. Systemic lupus erythematosus, the most common form of lupus, is a chronic autoimmune disease that can cause severe fatigue and joint pain. The diagnostic accuracies of the 2012 slicc criteria and the.
American college of rheumatology classification criteria for. Systemic lupus erythematosus diagnosis and management. The american college of rheumatology acr established 11 criteria as a classificatory instrument to operationalise the definition of sle in clinical trials. Cutaneous manifestations of systemic lupus erythematosus. The 2012 revised slicc criteria for classification of. The slicc criteria for sle classification requires. Lupus, technically known as systemic lupus erythematosus sle, is an autoimmune disease in which the bodys immune system mistakenly attacks healthy tissue in many parts of the body. Systemic lupus erythematosus sle is a prototypic autoimmune disease. It can affect the joints, skin, brain, lungs, kidneys, and blood vessels.
Npsle is associated with increased morbidity and mortality. Slicc revision of the acr classification criteria clinical criteria 1. The most widely used and accepted criteria has been the 1997 acr criteria. The signs and symptoms of sle vary among affected individuals, and can involve many organs and systems, including the skin, joints, kidneys, lungs, central nervous. Criteria are cumulative and need not be present concurrently. Systemic lupus erythematosus, interferon score, complement, autoinflammation, autoimmunity, patients stratification introduction systemic lupus erythematosus systemic lupus erythematosus sle is a multisystem inflammatory and autoimmune disorder and it is characterized by a wide spectrum of clinical manifestations.
Systemic lupus erythematosus sle is a systemic autoimmune disease that predominantly affects the females female to male ratio is 9. Correct classification of patients with systemic lupus erythematosus sle is critical for clinical trials and clinical and translational science. Treatment in sle aims at remission or low disease activity and. Currently, we have a limited understanding of mechanisms leading to systemic lupus erythematosus, but we know that genetics, environmental factors, and epigenetics contribute to. Systemic lupus erythematosus genetics home reference nih. Performance of the 2012 systemic lupus international collaborating clinics classification criteria versus the 1997 american college of rheumatology classification criteria in adult and juvenile systemic lupus erythematosus. Effect of curcumin on systemic lupus erythematosus full. There have been two rcts of rituximab in sle, the lunar. Sle is an autoimmune disease in which the immune system attacks its own tissues, causing widespread inflammation and tissue damage in the affected organs. What are eularacr diagnostic criteria for systemic lupus. The skin is one of the target organs most variably affected by the disease. Classification criteria for systemic lupus erythematosus. Table 1 1982 revised criteria for classification of sle the criteria were revised again in 1997 with changes to the immunologic disorder criterion that involve deleting a positive lupus erythematosus preparation and adding a positive finding of antiphospholipid antibodies based on 1 abnormal immunoglobulin g or.
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