Clinical characteristics, BALF composition and Follow-up Data of Patients with Unclassifiable ILD; Results from a single-center study.

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Background: Unclassifiable interstitial lung disease (uILD) is a heterogeneous group of lung diseases, in which a definite diagnosis cannot be made, even after a multi-disciplinary team discussion. Unclassifiable disease may be found in up to 20% of ILD patients representing a challenging diagnosis, while follow-up evaluation and optimal management remains unclear. In this study, we investigated the clinical features, bronchoalveolar lavage fluid (BALF) composition and disease course of patients with uILD. Methods: This was a single-center study performed at the ILD outpatient clinic of the University Hospital of Crete, and 31 patients were included. Baseline clinical features, pulmonary function, BALF cellular composition, and the presence of MUC5B minor allele were evaluated. The median follow-up period was 3 years. Results: The median age of the included patients was 71 years old, and 19 were females(61%).During follow-up, 4 patients were re-evaluated and started antifibrotic treatment with a diagnosis of IPF. Four patients were classified as ILD associated with Connective Tissue Disease. Paired analysis showed FVC decline in the first year, while 12(39%) patients progressed based on the current definition. No significant differences in regards to baseline characteristics were found between progressors and non-progressors. The percentage of BALF neutrophils and eosinophils was correlated with pulmonary function. Conclusion: Unclassifiable ILD has a heterogeneous clinical course, which is difficult to be predicted. Larger studies are crucial for a thorough evaluation uILD patients and for the detection of potential biomarkers to predict disease course.

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