Abstract
Background: Interstitial lung diseases (ILDs) represent a heterogeneous group of more than 200 distinct entities. Despite a thorough assessment and multidisciplinary discussion (MDD), about 12% of ILD patients remain unclassifiable (uILD).
Aims and Objectives: To determine whether a significant proportion of patients classified as uILD at time of first MDD are later given a specific diagnosis through either disease progression or availability of further data.
Methods: Medical notes for patients diagnosed with uILD of idiopathic pulmonary fibrosis (IPF) by MDD between 1 January 2015 and 30 June 2018 and completing at least 1 year of follow up before 30 June 2019 were retrospectively reviewed to assess any change in diagnosis.
Results: 93 patients were diagnosed with IPF and 90 with uILD, 35 of whom had surgical biopsy. 3 patients with uILD (3.3%) had a change in diagnosis during follow up; 1 to dermatomyositis and 2 to hypersensitivity pneumonitis. 66.6% of uILD patients had had at least one course of corticosteroids or immunosuppressive drugs. 23.3% with uILD and 84% with IPF received antifibrotic drugs. The median delay between the first disease-related manifestation and the MDD for uILD was 3.23 ±4.35 years [0-28 years]. Patients with uILD had worse outcome than patients with IPF (mean survival from the date of MDD to last follow-up was 36.1 months [31.6- 40.5] versus 48.6 months [39.8-45.7] respectively, p< 0.0001).
Conclusions: uILD may represent a distinct diagnostic category rather than a provisional diagnosis, and is characterized by a considerable delay to diagnosis and a poorer outcome.
Footnotes
Cite this article as: European Respiratory Journal 2020; 56: Suppl. 64, 3408.
This abstract was presented at the 2020 ERS International Congress, in session “Respiratory viruses in the "pre COVID-19" era”.
This is an ERS International Congress abstract. No full-text version is available. Further material to accompany this abstract may be available at www.ers-education.org (ERS member access only).
- Copyright ©the authors 2020