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Feasibility and Outcomes of a Standardized Management Protocol for Acute Exacerbation of Interstitial Lung Disease

  • INTERSTITIAL LUNG DISEASE
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Abstract

Background and Objective

Despite the high mortality of acute exacerbations of interstitial lung disease (AE-ILD), there is minimal evidence to guide management decisions. We aimed to assess the feasibility and outcomes of a standardized management protocol for AE-ILD.

Methods

We performed a retrospective cohort study of patients with AE-ILD admitted to hospital between January 2015 and August 2019. Patients were managed with a standardized protocol including chest computed tomography (CT) at diagnosis, pulse corticosteroid treatment, and a follow-up CT 7 days after corticosteroid pulse. The association between idiopathic pulmonary fibrosis (IPF) versus non-IPF diagnosis and transplant-free survival within 1-year of AE-ILD was assessed using adjusted Cox proportional hazards regression survival analysis. Associations with CT chest improvement 7 days after corticosteroid pulse were secondarily assessed.

Results

89 patients with AE-ILD were identified. 1-year transplant-free and overall survival were 20.2 and 51.7%, respectively. Protocol adherence to pulse corticosteroids was high (95.5%). A diagnosis of IPF was associated with higher risk of death or transplant at 1-year versus a non-IPF diagnosis [hazard ratio (HR) 2.23, 95% CI 1.19–4.17, p = 0.012]. There were no significant associations with 7-day CT improvement; however, CT improvement was associated with higher transplant-free survival (p = 0.02) and a lower risk of in-hospital mortality (χ2 = 7.06, p = 0.01) on unadjusted analysis.

Conclusions

IPF is associated with a higher risk of death or transplant at 1-year as compared to a non-IPF diagnosis in patients with AE-ILD managed using a standardized protocol. Improvement on CT chest 7 days after corticosteroid pulse is associated with better survival.

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Data Availability

The data that support the findings of this study are available from the corresponding author upon reasonable request and approval by applicable research ethics board.

Abbreviations

AE:

Acute exacerbation

CCI:

Charlson comorbidity index

CHP:

Chronic hypersensitivity pneumonitis

CI:

Confidence interval

CT:

Computed tomography

CTD:

Connective tissue disease

DLco:

Diffusing capacity of carbon monoxide

FVC:

Forced vital capacity

GVHD:

Graft versus host disease

HR:

Hazard ratio

ILD:

Interstitial lung disease

IPAF:

Idiopathic pneumonia with autoimmune features

IPF:

Idiopathic pulmonary fibrosis

IQR:

Interquartile range

MDD:

Multidisciplinary discussion

NSIP:

Non-specific interstitial pneumonia

PPI:

Proton pump inhibitor

6MWD:

6-Minute walk test distance

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Acknowledgements

No funding was required for this study.

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Authors and Affiliations

  1. Division of Respirology, Department of Medicine, University of Toronto, Toronto, ON, Canada

    Colin J. Adams, Dmitry Rozenberg, Shane Shapera & Jolene H. Fisher

  2. Faculty of Medicine, University of Toronto, Toronto, ON, Canada

    Karan Chohan

  3. Toronto General Hospital Research Institute, Lung Transplant Program, University Health Network, Toronto, ON, Canada

    Dmitry Rozenberg

  4. Department of Medical Imaging, University of Toronto, Toronto, ON, Canada

    John Kavanagh & Gerhard Greyling

  5. University Health Network, 9N-945 585 University, Avenue, Toronto, ON, M5G 2N2, Canada

    Jolene H. Fisher

Authors
  1. Colin J. Adams
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  2. Karan Chohan
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  4. John Kavanagh
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  7. Jolene H. Fisher
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Correspondence to Jolene H. Fisher.

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Conflict of interest

CJA, KC, JK and GG report no conflicts of interest. DR reports Salary Support from the Sandra Faire and Ivan Fecan Professorship in Rehabilitation Medicine. SS reports grants and personal fees from Boehringer-Ingelheim and Hoffman-La Roche Ltd. and personal fees from AstraZeneca, outside the submitted work. JHF reports grants from the Canadian Pulmonary Fibrosis Foundation and personal fees Boehringer-Ingelheim and AstraZeneca, outside the submitted work.

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Adams, C.J., Chohan, K., Rozenberg, D. et al. Feasibility and Outcomes of a Standardized Management Protocol for Acute Exacerbation of Interstitial Lung Disease. Lung 199, 379–387 (2021). https://doi.org/10.1007/s00408-021-00463-5

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  • DOI: https://doi.org/10.1007/s00408-021-00463-5

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