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Etiology, Risk Factors, and Biomarkers in Systemic Sclerosis with Interstitial Lung Disease
Systemic sclerosis (SSc) is a complex, multiorgan, autoimmune disease. Lung fibrosis occurs in ∼80% of patients with SSc; 25% to 30% develop progressive interstitial lung disease (ILD). The pathogenesis of fibrosis in SSc-associated ILD (SSc-ILD) involves cellular injury, activation/differentiation...
Autores principales: | , , , , , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
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American Thoracic Society
2020
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7068837/ https://www.ncbi.nlm.nih.gov/pubmed/31841044 http://dx.doi.org/10.1164/rccm.201903-0563CI |
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author | Khanna, Dinesh Tashkin, Donald P. Denton, Christopher P. Renzoni, Elisabetta A. Desai, Sujal R. Varga, John |
author_facet | Khanna, Dinesh Tashkin, Donald P. Denton, Christopher P. Renzoni, Elisabetta A. Desai, Sujal R. Varga, John |
author_sort | Khanna, Dinesh |
collection | PubMed |
description | Systemic sclerosis (SSc) is a complex, multiorgan, autoimmune disease. Lung fibrosis occurs in ∼80% of patients with SSc; 25% to 30% develop progressive interstitial lung disease (ILD). The pathogenesis of fibrosis in SSc-associated ILD (SSc-ILD) involves cellular injury, activation/differentiation of mesenchymal cells, and morphological/biological changes in epithelial/endothelial cells. Risk factors for progressive SSc-ILD include older age, male sex, degree of lung involvement on baseline high-resolution computed tomography imaging, reduced Dl(CO), and reduced FVC. SSc-ILD does not share the genetic risk architecture observed in idiopathic pulmonary fibrosis (IPF), with key risk factors yet to be identified. Presence of anti–Scl-70 antibodies and absence of anti-centromere antibodies indicate increased likelihood of progressive ILD. Elevated levels of serum Krebs von den Lungen-6 and C-reactive protein are both associated with SSc-ILD severity and predict SSc-ILD progression. A promising prognostic indicator is serum chemokine (C-C motif) ligand 18. SSc-ILD shares similarities with IPF, although clear differences exist. Histologically, a nonspecific interstitial pneumonia pattern is commonly observed in SSc-ILD, whereas IPF is defined by usual interstitial pneumonia. The course of SSc-ILD is variable, ranging from minor, stable disease to a progressive course, whereas all patients with IPF experience progression of disease. Although appropriately treated patients with SSc-ILD have better chances of stabilization and survival, a relentlessly progressive course, akin to IPF, is seen in a minority. Better understanding of cellular and molecular pathogenesis, genetic risk, and distinctive features of SSc-ILD and identification of robust prognostic biomarkers are needed for optimal disease management. |
format | Online Article Text |
id | pubmed-7068837 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2020 |
publisher | American Thoracic Society |
record_format | MEDLINE/PubMed |
spelling | pubmed-70688372020-03-15 Etiology, Risk Factors, and Biomarkers in Systemic Sclerosis with Interstitial Lung Disease Khanna, Dinesh Tashkin, Donald P. Denton, Christopher P. Renzoni, Elisabetta A. Desai, Sujal R. Varga, John Am J Respir Crit Care Med Concise Clinical Review Systemic sclerosis (SSc) is a complex, multiorgan, autoimmune disease. Lung fibrosis occurs in ∼80% of patients with SSc; 25% to 30% develop progressive interstitial lung disease (ILD). The pathogenesis of fibrosis in SSc-associated ILD (SSc-ILD) involves cellular injury, activation/differentiation of mesenchymal cells, and morphological/biological changes in epithelial/endothelial cells. Risk factors for progressive SSc-ILD include older age, male sex, degree of lung involvement on baseline high-resolution computed tomography imaging, reduced Dl(CO), and reduced FVC. SSc-ILD does not share the genetic risk architecture observed in idiopathic pulmonary fibrosis (IPF), with key risk factors yet to be identified. Presence of anti–Scl-70 antibodies and absence of anti-centromere antibodies indicate increased likelihood of progressive ILD. Elevated levels of serum Krebs von den Lungen-6 and C-reactive protein are both associated with SSc-ILD severity and predict SSc-ILD progression. A promising prognostic indicator is serum chemokine (C-C motif) ligand 18. SSc-ILD shares similarities with IPF, although clear differences exist. Histologically, a nonspecific interstitial pneumonia pattern is commonly observed in SSc-ILD, whereas IPF is defined by usual interstitial pneumonia. The course of SSc-ILD is variable, ranging from minor, stable disease to a progressive course, whereas all patients with IPF experience progression of disease. Although appropriately treated patients with SSc-ILD have better chances of stabilization and survival, a relentlessly progressive course, akin to IPF, is seen in a minority. Better understanding of cellular and molecular pathogenesis, genetic risk, and distinctive features of SSc-ILD and identification of robust prognostic biomarkers are needed for optimal disease management. American Thoracic Society 2020-03-15 2020-03-15 /pmc/articles/PMC7068837/ /pubmed/31841044 http://dx.doi.org/10.1164/rccm.201903-0563CI Text en Copyright © 2020 by the American Thoracic Society https://creativecommons.org/licenses/by-nc-nd/4.0/This article is open access and distributed under the terms of the Creative Commons Attribution Non-Commercial No Derivatives License 4.0 (http://creativecommons.org/licenses/by-nc-nd/4.0/ (https://creativecommons.org/licenses/by-nc-nd/4.0/) ). For commercial usage and reprints, please contact Diane Gern (dgern@thoracic.org). |
spellingShingle | Concise Clinical Review Khanna, Dinesh Tashkin, Donald P. Denton, Christopher P. Renzoni, Elisabetta A. Desai, Sujal R. Varga, John Etiology, Risk Factors, and Biomarkers in Systemic Sclerosis with Interstitial Lung Disease |
title | Etiology, Risk Factors, and Biomarkers in Systemic Sclerosis with Interstitial Lung Disease |
title_full | Etiology, Risk Factors, and Biomarkers in Systemic Sclerosis with Interstitial Lung Disease |
title_fullStr | Etiology, Risk Factors, and Biomarkers in Systemic Sclerosis with Interstitial Lung Disease |
title_full_unstemmed | Etiology, Risk Factors, and Biomarkers in Systemic Sclerosis with Interstitial Lung Disease |
title_short | Etiology, Risk Factors, and Biomarkers in Systemic Sclerosis with Interstitial Lung Disease |
title_sort | etiology, risk factors, and biomarkers in systemic sclerosis with interstitial lung disease |
topic | Concise Clinical Review |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7068837/ https://www.ncbi.nlm.nih.gov/pubmed/31841044 http://dx.doi.org/10.1164/rccm.201903-0563CI |
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