How Do We Identify and Manage Progressive CTD-ILD?-Update in Treatment and Guideline

The lung is a frequent target of autoimmune-mediated injury in patients with rheumatic diseases. Not infrequently, respiratory involvement may be the presenting manifestation of connective tissue diseases (CTDs). The prevalence of interstitial lung disease (ILD) in rheumatoid arthritis is about 20 t...

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Main Authors: Oliver Distler, Toby Maher
Format: Article
Language:English
Published: World Scientific Publishing 2024-01-01
Series:Journal of Clinical Rheumatology and Immunology
Online Access:https://www.worldscientific.com/doi/10.1142/S2661341724740110
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author Oliver Distler
Toby Maher
author_facet Oliver Distler
Toby Maher
author_sort Oliver Distler
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description The lung is a frequent target of autoimmune-mediated injury in patients with rheumatic diseases. Not infrequently, respiratory involvement may be the presenting manifestation of connective tissue diseases (CTDs). The prevalence of interstitial lung disease (ILD) in rheumatoid arthritis is about 20 to 30% of patients, while ILD may occur in 20 to 50% of patients with idiopathic inflammatory myositis, 40-50% or more of patients with systemic sclerosis, 2 to 5% of patients with systemic lupus erythematosus, 20 to 60% of patients with mixed connective tissue disease, and up to 25% of patients with Sjögren’s syndrome. Rheumatic disease-associated lung involvement is a major cause of morbidity and mortality in these patients. It shows a considerable heterogeneity in incidence and prevalence, clinical course, and the involved lung structure depending on the underlying rheumatic disease. Each rheumatic disease is associated with a characteristic pattern of their lung disease. The standard therapy has traditionally been immunosuppressant. With the evolvement of treatment, anti-fibrotic is now indicated for chronic fibrosing ILDs with a progressive phenotype, including autoimmune disease-associated ILD. By targeting the underlying mechanism of pulmonary fibrosis, it is shown that the annual rate of FVC decline can be reduced by more than 50%. In this lecture, Prof. Oliver Distler and Prof. Toby Maher will review the importance of timely treatment, the management practices and treatment guidelines for CTD-ILD.
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spelling doaj-art-77059cf80ffb4965967d2d3abef01a162025-08-20T02:14:11ZengWorld Scientific PublishingJournal of Clinical Rheumatology and Immunology2661-34172661-34252024-01-0124supp01111110.1142/S2661341724740110How Do We Identify and Manage Progressive CTD-ILD?-Update in Treatment and GuidelineOliver Distler0Toby Maher1SwitzerlandUnited KingdomThe lung is a frequent target of autoimmune-mediated injury in patients with rheumatic diseases. Not infrequently, respiratory involvement may be the presenting manifestation of connective tissue diseases (CTDs). The prevalence of interstitial lung disease (ILD) in rheumatoid arthritis is about 20 to 30% of patients, while ILD may occur in 20 to 50% of patients with idiopathic inflammatory myositis, 40-50% or more of patients with systemic sclerosis, 2 to 5% of patients with systemic lupus erythematosus, 20 to 60% of patients with mixed connective tissue disease, and up to 25% of patients with Sjögren’s syndrome. Rheumatic disease-associated lung involvement is a major cause of morbidity and mortality in these patients. It shows a considerable heterogeneity in incidence and prevalence, clinical course, and the involved lung structure depending on the underlying rheumatic disease. Each rheumatic disease is associated with a characteristic pattern of their lung disease. The standard therapy has traditionally been immunosuppressant. With the evolvement of treatment, anti-fibrotic is now indicated for chronic fibrosing ILDs with a progressive phenotype, including autoimmune disease-associated ILD. By targeting the underlying mechanism of pulmonary fibrosis, it is shown that the annual rate of FVC decline can be reduced by more than 50%. In this lecture, Prof. Oliver Distler and Prof. Toby Maher will review the importance of timely treatment, the management practices and treatment guidelines for CTD-ILD.https://www.worldscientific.com/doi/10.1142/S2661341724740110
spellingShingle Oliver Distler
Toby Maher
How Do We Identify and Manage Progressive CTD-ILD?-Update in Treatment and Guideline
Journal of Clinical Rheumatology and Immunology
title How Do We Identify and Manage Progressive CTD-ILD?-Update in Treatment and Guideline
title_full How Do We Identify and Manage Progressive CTD-ILD?-Update in Treatment and Guideline
title_fullStr How Do We Identify and Manage Progressive CTD-ILD?-Update in Treatment and Guideline
title_full_unstemmed How Do We Identify and Manage Progressive CTD-ILD?-Update in Treatment and Guideline
title_short How Do We Identify and Manage Progressive CTD-ILD?-Update in Treatment and Guideline
title_sort how do we identify and manage progressive ctd ild update in treatment and guideline
url https://www.worldscientific.com/doi/10.1142/S2661341724740110
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