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Chapter 91 : Immunodiagnosis of Scleroderma

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Immunodiagnosis of Scleroderma, Page 1 of 2

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Abstract:

Systemic sclerosis (SSc) or scleroderma is a connective tissue disease characterized by excessive fibrosis, microangiopathy, and the presence of circulating autoantibodies to various cellular components (1). Clinical presentation is highly heterogeneous in patients with SSc: some have only Raynaud's phenomenon and sclerodactyly without any symptomatic organ involvement for >10 years, but others have progressive functional impairment in lungs, heart, kidneys, or gastrointestinal tract, leading to death. Distinct specificities of antinuclear antibodies (ANAs) are selectively detected in SSc patients and are associated with unique disease manifestations (2). The detection of individual SSc-related ANAs is useful in the diagnosis, disease subgrouping, and prediction of future organ involvement and prognosis. Therefore, SSc-related ANAs are important biomarkers in routine rheumatology practice.

Citation: Kuwana M. 2016. Immunodiagnosis of Scleroderma, p 888-896. In Detrick B, Schmitz J, Hamilton R (ed), Manual of Molecular and Clinical Laboratory Immunology, Eighth Edition. ASM Press, Washington, DC. doi: 10.1128/9781555818722.ch91
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Figures

Image of FIGURE 1
FIGURE 1

Diagram showing SSc subsets stratified by autoantibodies. Areas of individual circles represent approximate proportions in the entire SSc patients. dcSSc: diffuse cutaneous systemic sclerosis, lcSSc: limited cutaneous systemic sclerosis.

Citation: Kuwana M. 2016. Immunodiagnosis of Scleroderma, p 888-896. In Detrick B, Schmitz J, Hamilton R (ed), Manual of Molecular and Clinical Laboratory Immunology, Eighth Edition. ASM Press, Washington, DC. doi: 10.1128/9781555818722.ch91
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Image of FIGURE 2
FIGURE 2

An outline flowchart for identification of SSc-related ANAs including minor ones.

Citation: Kuwana M. 2016. Immunodiagnosis of Scleroderma, p 888-896. In Detrick B, Schmitz J, Hamilton R (ed), Manual of Molecular and Clinical Laboratory Immunology, Eighth Edition. ASM Press, Washington, DC. doi: 10.1128/9781555818722.ch91
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References

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Tables

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TABLE 1

Structure and function of molecules targeted by SSc-related ANAs

Citation: Kuwana M. 2016. Immunodiagnosis of Scleroderma, p 888-896. In Detrick B, Schmitz J, Hamilton R (ed), Manual of Molecular and Clinical Laboratory Immunology, Eighth Edition. ASM Press, Washington, DC. doi: 10.1128/9781555818722.ch91
Generic image for table
TABLE 2

Methods for detection and clinical associations with SSc-related ANAs

Citation: Kuwana M. 2016. Immunodiagnosis of Scleroderma, p 888-896. In Detrick B, Schmitz J, Hamilton R (ed), Manual of Molecular and Clinical Laboratory Immunology, Eighth Edition. ASM Press, Washington, DC. doi: 10.1128/9781555818722.ch91
Generic image for table
TABLE 3

Commercially available kits for detection of anti-Scl-70, ACA, anti-RNAP III, and anti-U1RNP antibodies

Citation: Kuwana M. 2016. Immunodiagnosis of Scleroderma, p 888-896. In Detrick B, Schmitz J, Hamilton R (ed), Manual of Molecular and Clinical Laboratory Immunology, Eighth Edition. ASM Press, Washington, DC. doi: 10.1128/9781555818722.ch91
Generic image for table
TABLE 4

The American College of Rheumatology/European League Against Rheumatism criteria for the classification of SSc

Citation: Kuwana M. 2016. Immunodiagnosis of Scleroderma, p 888-896. In Detrick B, Schmitz J, Hamilton R (ed), Manual of Molecular and Clinical Laboratory Immunology, Eighth Edition. ASM Press, Washington, DC. doi: 10.1128/9781555818722.ch91

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