Unclassifiable, or simply unclassified interstitial lung disease?

Unclassifiable interstitial lung disease (ILD) comprises a subset of ILDs which cannot be classified according to the current diagnostic framework. This is a likely a heterogeneous group of diseases rather than a single entity and it is poorly defined and hence problematic for prognosis and therapy.

With increased treatment options for progressive fibrosing ILD it is increasingly relevant to correctly categorise ILD.

This review article will summarise the definition and reasons for a diagnosis of unclassifiable ILD, the current management options and possible future approaches to improve diagnosis and differentiation within this broad subset. Finally, we will describe the implications of the labelling of unclassifiable ILD in clinical practice and research and whether the term 'unclassified' should be used, implying a less definitive diagnosis.