Assessment of lupus: where are we now? 1993

E Hay, and C Gordon, and P Emery
Epidemiology Unit, University of Manchester, United Kingdom.

Decisions about treatment for patients with SLE are based on numerous criteria, including the rate of change of clinical features and disease markers (especially antibodies to dsDNA and markers of complement turnover), which organ systems are affected, the severity of manifestations, and the presence of pre-existing damage (which may reduce the reserve capacity of the organ system). Most of the currently available organ systems calculate a single overall score, at a single point in time, and take few of these considerations into account. SLAM is the only index to consider directly the scoring of severity as well as activity, though this concept is probably inherent in most of the other indices because of various methods for weighting the scores. Preliminary studies have indicated that four of the scales (BILAG, SLAM, LAI, and SLEDAI) are sensitive to change. Few of the indices have been tested longitudinally, hence their role in clinical trials remains to be established. None of the indices considers the impact of damage, indeed this is not their remit, but this concept is being considered by an international working group. Outcome in SLE has been shown to be determined by, among other things, the number of exacerbations and the presence of renal or neurological disease. It would seem, therefore, important to measure disease activity in designated organ systems, which most of the indices fail to do. The inclusion of immunological tests in some scales (for example, SIS, SLEDAI, LAI) makes them unsuitable for use as instruments to validate immunological or other scatological markers. Furthermore, given the heterogeneity of disease manifestations in SLE, and evidence linking scatological abnormalities with specific clinical manifestations, it is perhaps naive to expect a new scatological test to correlate strongly with overall disease activity. Three of the currently available activity scales have been shown to be reliable, both between and within raters (BILAG, SLAM, SLEDAI). The lack of a 'gold standard' for measuring disease activity in SLE makes it difficult to be sure that these scales are actually measuring what they are supposed to be measuring (criterion validity), but they do correlate strongly with each other in cross sectional studies, suggesting that they are, at least, all measuring the same thing (convergent validity). Convergent validity for these instruments used longitudinally remains to be established, particularly for patients with very active disease. In conclusion, measurement of disease activity in SLE is central to patient care, and a number of instruments are available fro this purpose. Although none is perfect, most are reliable and valid, and are suitable for classifying and monitoring groups of patients in the research setting. In reality, the indices are used with the additional benefit of laboratory markers and, as yet, no one has found the instruments sufficiently sensitive and specific to rely entirely upon them. The exact choice of instrument should be governed by the purpose for which it is required in clinical practice. Disease activity scales are unlikely to be appropriate for dictating treatment decisions in individual cases. An instrument which would be comprehensive and flexible enough for this purpose would necessarily prove too complicated and cumbersome for widespread use.

UI MeSH Term Description Entries
D008178 Lupus Erythematosus, Cutaneous A form of lupus erythematosus in which the skin may be the only organ involved or in which skin involvement precedes the spread into other body systems. It has been classified into three forms - acute ( Lupus Erythematosus, Cutaneous, Subacute,Lupus Erythematosus, Subacute Cutaneous,Cutaneous Lupus Erythematosus
D008180 Lupus Erythematosus, Systemic A chronic, relapsing, inflammatory, and often febrile multisystemic disorder of connective tissue, characterized principally by involvement of the skin, joints, kidneys, and serosal membranes. It is of unknown etiology, but is thought to represent a failure of the regulatory mechanisms of the autoimmune system. The disease is marked by a wide range of system dysfunctions, an elevated erythrocyte sedimentation rate, and the formation of LE cells in the blood or bone marrow. Libman-Sacks Disease,Lupus Erythematosus Disseminatus,Systemic Lupus Erythematosus,Disease, Libman-Sacks,Libman Sacks Disease
D008181 Lupus Nephritis Glomerulonephritis associated with autoimmune disease SYSTEMIC LUPUS ERYTHEMATOSUS. Lupus nephritis is histologically classified into 6 classes: class I - normal glomeruli, class II - pure mesangial alterations, class III - focal segmental glomerulonephritis, class IV - diffuse glomerulonephritis, class V - diffuse membranous glomerulonephritis, and class VI - advanced sclerosing glomerulonephritis (The World Health Organization classification 1982). Glomerulonephritis, Lupus,Lupus Glomerulonephritis,Nephritis, Lupus,Glomerulonephritides, Lupus,Lupus Glomerulonephritides,Lupus Nephritides,Nephritides, Lupus
D010808 Physical Examination Systematic and thorough inspection of the patient for physical signs of disease or abnormality. Physical Exam,Examination, Physical,Physical Examinations and Diagnoses,Exam, Physical,Examinations, Physical,Exams, Physical,Physical Examinations,Physical Exams
D012044 Regression Analysis Procedures for finding the mathematical function which best describes the relationship between a dependent variable and one or more independent variables. In linear regression (see LINEAR MODELS) the relationship is constrained to be a straight line and LEAST-SQUARES ANALYSIS is used to determine the best fit. In logistic regression (see LOGISTIC MODELS) the dependent variable is qualitative rather than continuously variable and LIKELIHOOD FUNCTIONS are used to find the best relationship. In multiple regression, the dependent variable is considered to depend on more than a single independent variable. Regression Diagnostics,Statistical Regression,Analysis, Regression,Analyses, Regression,Diagnostics, Regression,Regression Analyses,Regression, Statistical,Regressions, Statistical,Statistical Regressions
D006801 Humans Members of the species Homo sapiens. Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D000208 Acute Disease Disease having a short and relatively severe course. Acute Diseases,Disease, Acute,Diseases, Acute
D012720 Severity of Illness Index Levels within a diagnostic group which are established by various measurement criteria applied to the seriousness of a patient's disorder. Illness Index Severities,Illness Index Severity
D015415 Biomarkers Measurable and quantifiable biological parameters (e.g., specific enzyme concentration, specific hormone concentration, specific gene phenotype distribution in a population, presence of biological substances) which serve as indices for health- and physiology-related assessments, such as disease risk, psychiatric disorders, ENVIRONMENTAL EXPOSURE and its effects, disease diagnosis; METABOLIC PROCESSES; SUBSTANCE ABUSE; PREGNANCY; cell line development; EPIDEMIOLOGIC STUDIES; etc. Biochemical Markers,Biological Markers,Biomarker,Clinical Markers,Immunologic Markers,Laboratory Markers,Markers, Biochemical,Markers, Biological,Markers, Clinical,Markers, Immunologic,Markers, Laboratory,Markers, Serum,Markers, Surrogate,Markers, Viral,Serum Markers,Surrogate Markers,Viral Markers,Biochemical Marker,Biologic Marker,Biologic Markers,Clinical Marker,Immune Marker,Immune Markers,Immunologic Marker,Laboratory Marker,Marker, Biochemical,Marker, Biological,Marker, Clinical,Marker, Immunologic,Marker, Laboratory,Marker, Serum,Marker, Surrogate,Serum Marker,Surrogate End Point,Surrogate End Points,Surrogate Endpoint,Surrogate Endpoints,Surrogate Marker,Viral Marker,Biological Marker,End Point, Surrogate,End Points, Surrogate,Endpoint, Surrogate,Endpoints, Surrogate,Marker, Biologic,Marker, Immune,Marker, Viral,Markers, Biologic,Markers, Immune

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