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Natural history and screening of interstitial lung disease in systemic autoimmune rheumatic disorders
Interstitial lung disease (ILD) is a relatively frequent manifestation of systemic autoimmune rheumatic disorders (SARDs), including systemic sclerosis (SSc), rheumatoid arthritis (RA), idiopathic inflammatory myopathies (IIM), systemic lupus erythematosus (SLE), primary Sjögren’s syndrome (pSS), an...
Autores principales: | , , , , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
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SAGE Publications
2021
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Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8404673/ https://www.ncbi.nlm.nih.gov/pubmed/34471427 http://dx.doi.org/10.1177/1759720X211037519 |
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author | Panagopoulos, Panagiotis Goules, Andreas Hoffmann-Vold, Anna Maria Matteson, Eric L. Tzioufas, Athanasios |
author_facet | Panagopoulos, Panagiotis Goules, Andreas Hoffmann-Vold, Anna Maria Matteson, Eric L. Tzioufas, Athanasios |
author_sort | Panagopoulos, Panagiotis |
collection | PubMed |
description | Interstitial lung disease (ILD) is a relatively frequent manifestation of systemic autoimmune rheumatic disorders (SARDs), including systemic sclerosis (SSc), rheumatoid arthritis (RA), idiopathic inflammatory myopathies (IIM), systemic lupus erythematosus (SLE), primary Sjögren’s syndrome (pSS), and anti-neutrophil cytoplasmic antibody (ANCA) associated vasculitis. Interstitial pneumonia with autoimmune features (IPAF) has been proposed to describe patients with ILD who have clinical or serological findings compatible with SARDs but they are not sufficient for a definite diagnosis. ILD may present with different patterns among patients with SARDs, but most commonly as nonspecific interstitial pneumonia (NSIP), with the exception of RA and ANCA vasculitis that more often present with usual interstitial pneumonia (UIP). The natural history of ILD is quite variable, even among patients with the same SARD. It may present with subclinical features following a slow progressively course or with acute manifestations and clinically significant rapid progression leading to severe deterioration of pulmonary function and respiratory failure. The radiographic pattern of ILD, the extent of the disease, the baseline pulmonary function, the pulmonary function deterioration rate over time and clinical variables related to the primary SARD, such as age, sex and the clinical phenotype, are considered prognostic factors for SARDs-ILD associated with adverse outcomes and increased mortality. Different modalities can be employed for ILD detection including clinical evaluation, pulmonary function tests, high resolution computed tomography and novel techniques such as lung ultrasound and serum biomarkers. ILD may determine the clinical outcome of SARDs, since it is associated with significant morbidity and mortality and therefore screening of patients with SARDs for ILD is of great clinical importance. |
format | Online Article Text |
id | pubmed-8404673 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2021 |
publisher | SAGE Publications |
record_format | MEDLINE/PubMed |
spelling | pubmed-84046732021-08-31 Natural history and screening of interstitial lung disease in systemic autoimmune rheumatic disorders Panagopoulos, Panagiotis Goules, Andreas Hoffmann-Vold, Anna Maria Matteson, Eric L. Tzioufas, Athanasios Ther Adv Musculoskelet Dis Interstitial Lung Disease in Autoimmune Rheumatic Disorders Interstitial lung disease (ILD) is a relatively frequent manifestation of systemic autoimmune rheumatic disorders (SARDs), including systemic sclerosis (SSc), rheumatoid arthritis (RA), idiopathic inflammatory myopathies (IIM), systemic lupus erythematosus (SLE), primary Sjögren’s syndrome (pSS), and anti-neutrophil cytoplasmic antibody (ANCA) associated vasculitis. Interstitial pneumonia with autoimmune features (IPAF) has been proposed to describe patients with ILD who have clinical or serological findings compatible with SARDs but they are not sufficient for a definite diagnosis. ILD may present with different patterns among patients with SARDs, but most commonly as nonspecific interstitial pneumonia (NSIP), with the exception of RA and ANCA vasculitis that more often present with usual interstitial pneumonia (UIP). The natural history of ILD is quite variable, even among patients with the same SARD. It may present with subclinical features following a slow progressively course or with acute manifestations and clinically significant rapid progression leading to severe deterioration of pulmonary function and respiratory failure. The radiographic pattern of ILD, the extent of the disease, the baseline pulmonary function, the pulmonary function deterioration rate over time and clinical variables related to the primary SARD, such as age, sex and the clinical phenotype, are considered prognostic factors for SARDs-ILD associated with adverse outcomes and increased mortality. Different modalities can be employed for ILD detection including clinical evaluation, pulmonary function tests, high resolution computed tomography and novel techniques such as lung ultrasound and serum biomarkers. ILD may determine the clinical outcome of SARDs, since it is associated with significant morbidity and mortality and therefore screening of patients with SARDs for ILD is of great clinical importance. SAGE Publications 2021-08-28 /pmc/articles/PMC8404673/ /pubmed/34471427 http://dx.doi.org/10.1177/1759720X211037519 Text en © The Author(s), 2021 https://creativecommons.org/licenses/by-nc/4.0/This article is distributed under the terms of the Creative Commons Attribution-NonCommercial 4.0 License (https://creativecommons.org/licenses/by-nc/4.0/) which permits non-commercial use, reproduction and distribution of the work without further permission provided the original work is attributed as specified on the SAGE and Open Access page (https://us.sagepub.com/en-us/nam/open-access-at-sage). |
spellingShingle | Interstitial Lung Disease in Autoimmune Rheumatic Disorders Panagopoulos, Panagiotis Goules, Andreas Hoffmann-Vold, Anna Maria Matteson, Eric L. Tzioufas, Athanasios Natural history and screening of interstitial lung disease in systemic autoimmune rheumatic disorders |
title | Natural history and screening of interstitial lung disease in systemic autoimmune rheumatic disorders |
title_full | Natural history and screening of interstitial lung disease in systemic autoimmune rheumatic disorders |
title_fullStr | Natural history and screening of interstitial lung disease in systemic autoimmune rheumatic disorders |
title_full_unstemmed | Natural history and screening of interstitial lung disease in systemic autoimmune rheumatic disorders |
title_short | Natural history and screening of interstitial lung disease in systemic autoimmune rheumatic disorders |
title_sort | natural history and screening of interstitial lung disease in systemic autoimmune rheumatic disorders |
topic | Interstitial Lung Disease in Autoimmune Rheumatic Disorders |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8404673/ https://www.ncbi.nlm.nih.gov/pubmed/34471427 http://dx.doi.org/10.1177/1759720X211037519 |
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