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Minerva Pneumologica 2017 March;56(1):25-32

DOI: 10.23736/S0026-4954.17.01776-X

Copyright © 2016 EDIZIONI MINERVA MEDICA

language: English

Prevalence, characteristics, and outcomes of patients with unclassifiable interstitial lung disease

Sabina A. GULER 1, 2, Christopher J. RYERSON 1

1 Department of Medicine and Centre for Heart Lung Innovation, University of British Columbia, Vancouver, Canada; 2 Department of Pulmonary Medicine, University Hospital and University of Bern, Bern, Switzerland


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Interstitial lung disease (ILD) is a group of disorders that are often characterized by fibrosis of the lung parenchyma, typically causing increased dyspnea, functional limitation, and early mortality. Approximately 5-25% of ILD patients cannot be provided with a confident diagnosis despite an exhaustive search for potential etiologies and a multidisciplinary review. This can occur due to an incomplete acquisition of diagnostic studies, conflicting or overlapping features, or non-classifiable histological findings. The clinical features of unclassifiable ILD have been described in several recent studies, showing that this population includes a heterogeneous collection of patients that have similar characteristics compared to mixed ILD cohorts. Pharmacotherapy for patients with unclassifiable ILD is typically guided by a multidisciplinary review that considers the most likely diagnosis and anticipated disease behaviour. The objective of this review is to summarize recent studies related to unclassifiable ILD, with a focus on its prevalence, characteristics, and outcomes. Limitations in the literature are identified, including the inconsistent terminology and lack of a precise definition of unclassifiable ILD.


KEY WORDS: Idiopathic interstitial pneumonias - Idiopathic pulmonary fibrosis - Diagnosis - Disease management

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