Autoantibodies in pathogenesis

Kimberly Doering, Antony Rosen

Research output: Chapter in Book/Report/Conference proceedingChapter

Abstract

Scleroderma or systemic sclerosis (SSc) is a systemic autoimmune disease characterized by a distinct, proliferative vasculopathy associated with skin thickening and fibrosis within internal organs and is associated with a unique autoimmune response. While vascular damage is almost universal in scleroderma, disease expression is heterogeneous in terms of phenotypic characteristics and disease course. Nevertheless, clear clinical subtypes can be discerned. The limited cutaneous form of scleroderma (lcSSc) is characterized by skin thickening on the distal limbs, face and neck, and an increased prevalence of isolated pulmonary hypertension and ischemic digital loss. In contrast, the diffuse form of the disease also involves more proximal skin, and affects visceral organs, including the lung, heart, gastrointestinal tract, skeletal muscle and kidneys, with attendant negative effects on mortality. There are striking associations between specific autoantibodies and the distinct phenotypic subsets of scleroderma. These associations are clinically useful, suggesting, at a minimum, that the different autoantibodies are reporting on specific circumstances which underlie disease propagation in different tissues. It is also possible that specific autoantibodies play a direct pathogenic role in the propagation and amplification of autoimmune reactions and pathology in SSc. This chapter reviews some of the interesting insights which autoantibodies and their specificity provide into scleroderma pathogenesis.

Original languageEnglish (US)
Title of host publicationScleroderma
Subtitle of host publicationFrom Pathogenesis to Comprehensive Management
PublisherSpringer US
Pages199-208
Number of pages10
ISBN (Electronic)9781441957740
ISBN (Print)9781441957733
DOIs
StatePublished - Jan 1 2012

Fingerprint

Autoantibodies
Skin
Systemic Scleroderma
Autoimmunity
Pulmonary Hypertension
Autoimmune Diseases
Blood Vessels
Gastrointestinal Tract
Skeletal Muscle
Fibrosis
Neck
Extremities
Pathology
Kidney
Lung
Mortality

Keywords

  • ANA
  • Anticentromere
  • Autoantibodies
  • Humoral immunity in ssc
  • Pathogenesis
  • Rna polymerase III
  • Scl-70

ASJC Scopus subject areas

  • Medicine(all)

Cite this

Doering, K., & Rosen, A. (2012). Autoantibodies in pathogenesis. In Scleroderma: From Pathogenesis to Comprehensive Management (pp. 199-208). Springer US. https://doi.org/10.1007/978-1-4419-5774-0_17

Autoantibodies in pathogenesis. / Doering, Kimberly; Rosen, Antony.

Scleroderma: From Pathogenesis to Comprehensive Management. Springer US, 2012. p. 199-208.

Research output: Chapter in Book/Report/Conference proceedingChapter

Doering, K & Rosen, A 2012, Autoantibodies in pathogenesis. in Scleroderma: From Pathogenesis to Comprehensive Management. Springer US, pp. 199-208. https://doi.org/10.1007/978-1-4419-5774-0_17
Doering K, Rosen A. Autoantibodies in pathogenesis. In Scleroderma: From Pathogenesis to Comprehensive Management. Springer US. 2012. p. 199-208 https://doi.org/10.1007/978-1-4419-5774-0_17
Doering, Kimberly ; Rosen, Antony. / Autoantibodies in pathogenesis. Scleroderma: From Pathogenesis to Comprehensive Management. Springer US, 2012. pp. 199-208
@inbook{82495564069b4e1fb7974c6cd6367618,
title = "Autoantibodies in pathogenesis",
abstract = "Scleroderma or systemic sclerosis (SSc) is a systemic autoimmune disease characterized by a distinct, proliferative vasculopathy associated with skin thickening and fibrosis within internal organs and is associated with a unique autoimmune response. While vascular damage is almost universal in scleroderma, disease expression is heterogeneous in terms of phenotypic characteristics and disease course. Nevertheless, clear clinical subtypes can be discerned. The limited cutaneous form of scleroderma (lcSSc) is characterized by skin thickening on the distal limbs, face and neck, and an increased prevalence of isolated pulmonary hypertension and ischemic digital loss. In contrast, the diffuse form of the disease also involves more proximal skin, and affects visceral organs, including the lung, heart, gastrointestinal tract, skeletal muscle and kidneys, with attendant negative effects on mortality. There are striking associations between specific autoantibodies and the distinct phenotypic subsets of scleroderma. These associations are clinically useful, suggesting, at a minimum, that the different autoantibodies are reporting on specific circumstances which underlie disease propagation in different tissues. It is also possible that specific autoantibodies play a direct pathogenic role in the propagation and amplification of autoimmune reactions and pathology in SSc. This chapter reviews some of the interesting insights which autoantibodies and their specificity provide into scleroderma pathogenesis.",
keywords = "ANA, Anticentromere, Autoantibodies, Humoral immunity in ssc, Pathogenesis, Rna polymerase III, Scl-70",
author = "Kimberly Doering and Antony Rosen",
year = "2012",
month = "1",
day = "1",
doi = "10.1007/978-1-4419-5774-0_17",
language = "English (US)",
isbn = "9781441957733",
pages = "199--208",
booktitle = "Scleroderma",
publisher = "Springer US",

}

TY - CHAP

T1 - Autoantibodies in pathogenesis

AU - Doering, Kimberly

AU - Rosen, Antony

PY - 2012/1/1

Y1 - 2012/1/1

N2 - Scleroderma or systemic sclerosis (SSc) is a systemic autoimmune disease characterized by a distinct, proliferative vasculopathy associated with skin thickening and fibrosis within internal organs and is associated with a unique autoimmune response. While vascular damage is almost universal in scleroderma, disease expression is heterogeneous in terms of phenotypic characteristics and disease course. Nevertheless, clear clinical subtypes can be discerned. The limited cutaneous form of scleroderma (lcSSc) is characterized by skin thickening on the distal limbs, face and neck, and an increased prevalence of isolated pulmonary hypertension and ischemic digital loss. In contrast, the diffuse form of the disease also involves more proximal skin, and affects visceral organs, including the lung, heart, gastrointestinal tract, skeletal muscle and kidneys, with attendant negative effects on mortality. There are striking associations between specific autoantibodies and the distinct phenotypic subsets of scleroderma. These associations are clinically useful, suggesting, at a minimum, that the different autoantibodies are reporting on specific circumstances which underlie disease propagation in different tissues. It is also possible that specific autoantibodies play a direct pathogenic role in the propagation and amplification of autoimmune reactions and pathology in SSc. This chapter reviews some of the interesting insights which autoantibodies and their specificity provide into scleroderma pathogenesis.

AB - Scleroderma or systemic sclerosis (SSc) is a systemic autoimmune disease characterized by a distinct, proliferative vasculopathy associated with skin thickening and fibrosis within internal organs and is associated with a unique autoimmune response. While vascular damage is almost universal in scleroderma, disease expression is heterogeneous in terms of phenotypic characteristics and disease course. Nevertheless, clear clinical subtypes can be discerned. The limited cutaneous form of scleroderma (lcSSc) is characterized by skin thickening on the distal limbs, face and neck, and an increased prevalence of isolated pulmonary hypertension and ischemic digital loss. In contrast, the diffuse form of the disease also involves more proximal skin, and affects visceral organs, including the lung, heart, gastrointestinal tract, skeletal muscle and kidneys, with attendant negative effects on mortality. There are striking associations between specific autoantibodies and the distinct phenotypic subsets of scleroderma. These associations are clinically useful, suggesting, at a minimum, that the different autoantibodies are reporting on specific circumstances which underlie disease propagation in different tissues. It is also possible that specific autoantibodies play a direct pathogenic role in the propagation and amplification of autoimmune reactions and pathology in SSc. This chapter reviews some of the interesting insights which autoantibodies and their specificity provide into scleroderma pathogenesis.

KW - ANA

KW - Anticentromere

KW - Autoantibodies

KW - Humoral immunity in ssc

KW - Pathogenesis

KW - Rna polymerase III

KW - Scl-70

UR - http://www.scopus.com/inward/record.url?scp=84906327717&partnerID=8YFLogxK

UR - http://www.scopus.com/inward/citedby.url?scp=84906327717&partnerID=8YFLogxK

U2 - 10.1007/978-1-4419-5774-0_17

DO - 10.1007/978-1-4419-5774-0_17

M3 - Chapter

AN - SCOPUS:84906327717

SN - 9781441957733

SP - 199

EP - 208

BT - Scleroderma

PB - Springer US

ER -