Acute exacerbation in interstitial lung disease.

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Date: April-June 2021
From: Annals of Thoracic Medicine(Vol. 16, Issue 2)
Publisher: Medknow Publications and Media Pvt. Ltd.
Document Type: Article
Length: 4,709 words
Lexile Measure: 1690L

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Byline: Esam. Alhamad, Joseph. Cal, Nuha. Alrajhi, Ahmad. AlBoukai

BACKGROUND: Information regarding acute exacerbation (AE) in patients with interstitial lung disease (ILD) is limited. OBJECTIVES: The objective of the study was to elucidate the clinical features and outcome of AE among ILD patients. METHODS: We retrospectively analyzed the data of 667 consecutive ILD (nonidiopathic pulmonary fibrosis [IPF] ILD, n = 463; IPF, n = 204) patients. ILD patients meeting the 2016 definition of AE-IPF were identified. Information analyzed included pulmonary function tests, 6-min walk tests, and right heart catheterization data, among others. Cox regression models were used to identify independent predictors of survival. RESULTS: AE was identified in non-IPF ILD (n = 113) and IPF (n = 74). Compared with AE-IPF patients, non-IPF ILD patients with AE were of younger age, predominantly women, and primarily nonsmokers (all, P < 0.0001). The estimated survival probabilities at 1, 3, and 5 years were 88%, 75%, and 70%, respectively, in the ILD without AE group; 80%, 57%, and 50%, respectively, in the non-IPF ILD with AE group; and 53%, 38%, and 28%, respectively, in the AE-IPF group (P < 0.0001 by log-rank analysis). Age, body mass index, IPF diagnosis, AE, diffusion capacity of the lung for carbon monoxide <35% predicted, 6-min walk distance <300 meters, and cardiac index were independent predictors of survival in the ILD cohort. CONCLUSIONS: Non-IPF ILD patients with AE have distinct clinical features compared to AE-IPF patients. Importantly, AE is one of many independent risk factors associated with worsened outcomes regardless of the underlying ILD type.

Interstitial lung disease (ILD) comprises a large group of disorders characterized by repetitive injury to the lung parenchyma with variable degrees of inflammation and scarring depending on the underlying ILD type. Idiopathic pulmonary fibrosis (IPF), connective tissue disease (CTD)-associated ILD, chronic hypersensitivity pneumonitis, and sarcoidosis are the most common ILD subtypes seen in ILD clinics. The natural course of fibrotic ILD involves progressive worsening of underlying lung fibrosis that occurs over many years; however, a subset of ILD patients may develop acute exacerbation (AE), defined as acute deterioration of respiratory symptoms of <1 month in duration with new ground-glass opacity and/or consolidation based on computed tomography and in the absence of heart failure or fluid overload.[1]

Although the term AE was originally described in IPF patients, accumulating evidence has shown that AE can also occur in other forms of fibrotic ILDs such as idiopathic nonspecific interstitial pneumonia (NSIP), CTD-ILD, chronic hypersensitivity pneumonitis, and sarcoidosis.[2],[3],[4],[5],[6],[7] As such, it is now generally accepted that the definition of AE in IPF can also be applied in other types of fibrotic ILDs.[8] Although some studies[9],[10],[11],[12] have shown that IPF with AE is associated with poor survival compared with other ILDs, other studies[13],[14],[15],[16] found no significant difference in overall survival between AE in IPF and AE in fibrotic ILDs. Nonetheless, when AE occurs, regardless of the underlying ILD diagnosis, it creates significant challenges for patients, families, clinicians, and the health-care system. As such, identifying the factors that predict...

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Gale Document Number: GALE|A660154804