Connective tissue disease-associated interstitial lung disease

ABSTRACT Connective tissue disease-associated interstitial lung disease (CTD-ILD) represents a group of systemic autoimmune disorders characterized by immune-mediated organ dysfunction. Systemic sclerosis, rheumatoid arthritis, idiopathic inflammatory myositis, and Sjögren’s syndrome are the most common CTDs that present with pulmonary involvement, as well as with interstitial pneumonia with autoimmune features. The frequency of CTD-ILD varies according to the type of CTD, but the overall incidence is 15%, causing an important impact on morbidity and mortality. The decision of which CTD patient should be investigated for ILD is unclear for many CTDs. Besides that, the clinical spectrum can range from asymptomatic findings on imaging to respiratory failure and death. A significant proportion of patients will present with a more severe and progressive disease, and, for those, immunosuppression with corticosteroids and cytotoxic medications are the mainstay of pharmacological treatment. In this review, we summarized the approach to diagnosis and treatment of CTD-ILD, highlighting recent advances in therapeutics for the various forms of CTD..

Medienart:

E-Artikel

Erscheinungsjahr:

2024

Erschienen:

2024

Enthalten in:

Zur Gesamtaufnahme - volume:50

Enthalten in:

Jornal Brasileiro de Pneumologia - 50(2024), 1

Sprache:

Englisch ; Portugiesisch

Beteiligte Personen:

Karin Mueller Storrer [VerfasserIn]
Carolina de Souza Müller [VerfasserIn]
Maxwell Cássio de Albuquerque Pessoa [VerfasserIn]
Carlos Alberto de Castro Pereira [VerfasserIn]

Links:

doi.org [kostenfrei]
doaj.org [kostenfrei]
www.scielo.br [kostenfrei]
www.scielo.br [kostenfrei]
Journal toc [kostenfrei]

Themen:

Arthritis, rheumatoid
Collagen diseases
Diseases of the respiratory system
Lung diseases, interstitial
Myositis
Scleroderma, systemic
Therapeutics

doi:

10.36416/1806-3756/e20230132

funding:

Förderinstitution / Projekttitel:

PPN (Katalog-ID):

DOAJ09141279X