Unclassifiable interstitial lung disease: from phenotyping to possible treatments.

Guler, Sabina; Ryerson, Christopher J (2018). Unclassifiable interstitial lung disease: from phenotyping to possible treatments. Current opinion in pulmonary medicine, 24(5), pp. 461-468. Wolters Kluwer 10.1097/MCP.0000000000000509

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PURPOSE OF REVIEW Accurate diagnosis of interstitial lung diseases (ILDs) can be challenging, and a substantial percentage of ILD patients remain unclassifiable even after thorough assessment by an experienced multidisciplinary team. In this review, we summarize the recent literature on the definition, prevalence, diagnosis, treatment, and prognosis of unclassifiable ILD, and also discuss important current issues and provide future perspectives on the classification of ILD. RECENT FINDINGS Approximately 12% of patients with ILD are considered unclassifiable, with large variability across previous studies that is in part secondary to inconsistent definitions of unclassifiable ILD and other ILD subtypes. A recent International Working Group suggested that unclassifiable ILD should be defined by the absence of a leading diagnosis that is considered more likely than not after multidisciplinary discussion of all available information. Clinical features and outcomes of unclassifiable ILD are intermediate between idiopathic pulmonary fibrosis and nonidiopathic pulmonary fibrosis ILD cohorts, and choices for pharmacotherapy should be considered on a case-by-case basis. SUMMARY Recent studies have provided additional data on the clinical features and prognosis of unclassifiable ILD, but also highlight the many uncertainties that still exist in ILD diagnosis and classification. New tools are needed to more accurately characterize patients with unclassifiable ILD.

Item Type:

Journal Article (Review Article)


04 Faculty of Medicine > Department of Gastro-intestinal, Liver and Lung Disorders (DMLL) > Clinic of Pneumology

UniBE Contributor:

Guler, Sabina


600 Technology > 610 Medicine & health




Wolters Kluwer




Rahel Holderegger

Date Deposited:

06 Feb 2019 15:24

Last Modified:

06 Feb 2019 15:27

Publisher DOI:


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