Acute exacerbation of interstitial lung disease associated with rheumatic disease.


Journal

Nature reviews. Rheumatology
ISSN: 1759-4804
Titre abrégé: Nat Rev Rheumatol
Pays: United States
ID NLM: 101500080

Informations de publication

Date de publication:
02 2022
Historique:
accepted: 10 11 2021
pubmed: 9 12 2021
medline: 23 3 2022
entrez: 8 12 2021
Statut: ppublish

Résumé

Interstitial lung disease (ILD) is a cause of morbidity and mortality in patients with rheumatic diseases, such as connective-tissue diseases, rheumatoid arthritis and systemic vasculitis. Some patients with ILD secondary to rheumatic disease (RD-ILD) experience acute exacerbations, with sudden ILD progression and high mortality during or immediately after the exacerbation, and a very low 1-year survival rate. In the ILD subtype idiopathic pulmonary fibrosis (IPF), an acute exacerbation is defined as acute worsening or development of dyspnoea associated with new bilateral ground-glass opacities and/or consolidations at high-resolution CT, superimposed on a background pattern consistent with fibrosing ILD. However, acute exacerbation in RD-ILD (AE-RD-ILD) currently has no specific definition. The aetiology and pathogenesis of AE-RD-ILD remain unclear, but distinct triggers might include infection, mechanical stress, microaspiration and DMARD treatment. At this time, no effective evidence-based therapeutic strategies for AE-RD-ILD are available. In clinical practice, AE-RD-ILD is often empirically treated with high-dose systemic steroids and antibiotics, with or without immunosuppressive drugs. In this Review, we summarize the clinical features, diagnosis, management and prognosis of AE-RD-ILD, enabling the similarities and differences with acute exacerbation in IPF to be critically assessed.

Identifiants

pubmed: 34876670
doi: 10.1038/s41584-021-00721-z
pii: 10.1038/s41584-021-00721-z
doi:

Types de publication

Journal Article Research Support, Non-U.S. Gov't Review

Langues

eng

Sous-ensembles de citation

IM

Pagination

85-96

Informations de copyright

© 2021. Springer Nature Limited.

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Auteurs

Fabrizio Luppi (F)

Respiratory Diseases Unit, University of Milano-Bicocca, S. Gerardo Hospital, ASST Monza, Monza, Italy. fabrizio.luppi@unimib.it.

Marco Sebastiani (M)

Rheumatology Unit, University of Modena and Reggio Emilia, Azienda Ospedaliero-Universitaria Policlinico di Modena, Modena, Italy.

Carlo Salvarani (C)

Rheumatology Unit, University of Modena and Reggio Emilia, Azienda Ospedaliero-Universitaria Policlinico di Modena, Modena, Italy.
Rheumatology Unit, Dipartimento Medicina Interna e Specialità Mediche, Azienda Unità Sanitaria Locale di Reggio Emilia-Istituto di Ricerca e Cura a Carattere Scientifico, Reggio Emilia, Italy.

Elisabeth Bendstrup (E)

Center for Rare Lung Disease, Department of Respiratory Medicine and Allergy, Aarhus University Hospital, Aarhus, Denmark.

Andreina Manfredi (A)

Rheumatology Unit, University of Modena and Reggio Emilia, Azienda Ospedaliero-Universitaria Policlinico di Modena, Modena, Italy.

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