Biomaterial Database

Autoimmunity in scleroderma: the origin, pathogenetic role, and clinical significance of autoantibodies. 2003

Michelle L Harris, and Antony Rosen

Department of Medicine, Johns Hopkins University School of Medicine, Baltimore, Maryland 21205, USA.

OBJECTIVE Systemic sclerosis, or scleroderma, is a systemic autoimmune disease manifest by vascular damage and fibrosis within the skin and visceral organs. Whereas scleroderma is a heterogenous disorder in terms of disease symptoms and clinical course, scleroderma-specific autoantibody profiles associate strongly with distinct clinical phenotypes, making serologic testing of great diagnostic aid. This review will focus on the clinical significance and the potential pathogenic role of autoantibodies in scleroderma. RESULTS Novel autoantibody and phenotype associations discovered within the past year underscore the clinical utility of systemic sclerosis-associated autoantibodies. Whereas autoantibodies are generally believed to indicate the presence of ongoing tissue damage, some research suggests that the humoral immune response may play a role in generating such damage. CONCLUSIONS Improvements in multiplex autoantibody assays will aid in the diagnosis and prognosis of the complications associated with systemic sclerosis. Continued research into autoantibody/phenotype associations could also yield critical insights into the pathogenesis of, and suggest novel therapeutic targets for, this chronic, debilitating disease.

UI	MeSH Term	Description	Entries
D008297	Male		Males
D011379	Prognosis	A prediction of the probable outcome of a disease based on a individual's condition and the usual course of the disease as seen in similar situations.	Prognostic Factor,Prognostic Factors,Factor, Prognostic,Factors, Prognostic,Prognoses
D005260	Female		Females
D006801	Humans	Members of the species Homo sapiens.	Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D001323	Autoantibodies	Antibodies that react with self-antigens (AUTOANTIGENS) of the organism that produced them.	Autoantibody
D012595	Scleroderma, Systemic	A chronic multi-system disorder of CONNECTIVE TISSUE. It is characterized by SCLEROSIS in the SKIN, the LUNGS, the HEART, the GASTROINTESTINAL TRACT, the KIDNEYS, and the MUSCULOSKELETAL SYSTEM. Other important features include diseased small BLOOD VESSELS and AUTOANTIBODIES. The disorder is named for its most prominent feature (hard skin), and classified into subsets by the extent of skin thickening: LIMITED SCLERODERMA and DIFFUSE SCLERODERMA.	Sclerosis, Systemic,Systemic Scleroderma,Systemic Sclerosis
D012680	Sensitivity and Specificity	Binary classification measures to assess test results. Sensitivity or recall rate is the proportion of true positives. Specificity is the probability of correctly determining the absence of a condition. (From Last, Dictionary of Epidemiology, 2d ed)	Specificity,Sensitivity,Specificity and Sensitivity
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
D015551	Autoimmunity	Process whereby the immune system reacts against the body's own tissues. Autoimmunity may produce or be caused by AUTOIMMUNE DISEASES.	Autoimmune Response,Autoimmune Responses,Autoimmunities
D018570	Risk Assessment	The qualitative or quantitative estimation of the likelihood of adverse effects that may result from exposure to specified health hazards or from the absence of beneficial influences. (Last, Dictionary of Epidemiology, 1988)	Assessment, Risk,Benefit-Risk Assessment,Risk Analysis,Risk-Benefit Assessment,Health Risk Assessment,Risks and Benefits,Analysis, Risk,Assessment, Benefit-Risk,Assessment, Health Risk,Assessment, Risk-Benefit,Benefit Risk Assessment,Benefit-Risk Assessments,Benefits and Risks,Health Risk Assessments,Risk Analyses,Risk Assessment, Health,Risk Assessments,Risk Benefit Assessment,Risk-Benefit Assessments