Idiopathic Pulmonary Fibrosis Clinical Diagnostic Scoring System Validated

The Fairfax idiopathic pulmonary fibrosis (IPF) clinical score was an accurate tool for estimating probability of IPF in patients with interstitial lung disease (ILD), according to the results of a recent study published in the Annals of the American Thoracic Society.

Patients with ILD who were part of the Inova Fairfax ILD Program were assigned a clinical score. Factors that could affect the clinical score included age, sex, smoking history, ethnicity, ILD family history, exposures, presence of connective tissue disease signs or symptoms, and Velcro crackles. Using these scores, a multivariable model was created and validated by receiver operator curve characteristics.

Among the 844 patients with ILD, 347 were diagnosed with IPF and 497 were diagnosed with non-IPF. When the Fairfax IPF clinical score was calculated, scores ranged from 1 to 25. Among patients with a score of 10 or less, 4% had a final diagnosis of IPF; patients with a score between 16 and 20, 75% had IPF; and patients with a score of more than 20 (the highest quartile), 97% had IPF.

The clinical diagnostic score system was accurate in predicting IPF, as measured by the area under the curve (AUC, 0.88) in the derivation cohort with similar AUCs of 0.91, 0.81, and 0.71 in the respective validation cohorts.

“This model could ultimately be useful for increasing the degree of confidence in the final diagnosis and help to obviate the need for lung biopsy in cases of non-[usual interstitial pneumonia] patterns on [high-resolution computed tomography],” the study authors wrote.

Disclosure: Several study authors declared affiliations with the pharmaceutical industry. Please see the original reference for a full list of authors’ disclosures.

Reference

Pastre J, Barnett SD, Ksovreli I, et al. Development and validation of a clinical diagnostic scoring system for the diagnosis of IPF. Ann Am Thorac Soc. Published online April 12, 2021. doi:10.1513/AnnalsATS.202011-1430OC