Figures
- FIGURE 1
Case of clinical evolution from interstitial lung abnormalities (ILA), early idiopathic pulmonary fibrosis (IPF) (subclinical), mild IPF and to advanced IPF. A 70-year-old man without risk factors for interstitial lung disease (ILD). He presented to the Forlì ILD Clinic, V. Poletti, for the incidental finding of ILA, was reclassified to early IPF after multidisciplinary revision of radiological and pathological findings on lung biopsy and during the follow-up evolved to mild IPF, advanced IPF and ultimately died of the disease. a) ILA. Incidental finding of reticular subpleural abnormalities with traction bronchiectasis. Pulmonary function tests: forced vital capacity (FVC) 129%, diffusing capacity of the lung for carbon monoxide (DLCO) 82%. No symptoms. b) Early IPF. The lung biopsy showed a definite usual interstitial pneumonia pattern and the case was reclassified as early IPF. c) Mild IPF. After 2 years of follow-up the DLCO dropped to 73%. The patient remained asymptomatic, with FVC around 120%. d) Advanced IPF. After 7 years of follow-up the FVC dropped to 60%, DLCO to 40% and the patient became symptomatic. The patient died after 10 years of follow-up.
- FIGURE 4
Proposed algorithm for diagnosis and management of interstitial lung abnormalities (ILA), early interstitial lung disease (ILD) (both subclinical and pre-clinical) and mild ILD. BAL: bronchoalveolar lavage; CT: computed tomography; HRCT: high-resolution computed tomography; IPF: idiopathic pulmonary fibrosis; PFT: pulmonary function test; UIP: usual interstitial pneumonia.
Tables
- TABLE 1
Simplified definitions
Entity Population Diagnostic criteria Definition ILA Only individuals without known or suspected ILD# Clinical-radiological entity Incidental finding of CT abnormalities affecting more than 5% of any lung zone Early ILD Pre-clinical ILD Individuals at risk for ILD Clinical-radiological-pathological entity Any ILD in asymptomatic patients with preserved lung function Subclinical ILD Individuals NOT at risk for ILD Mild ILD All individuals Clinical-radiological-pathological entity Any clinically significant ILD with minor symptoms and/or trivial PFT abnormalities ILA: interstitial lung abnormalities; ILD: interstitial lung disease; CT: computed tomography; PFT: pulmonary function test. #: abnormalities identified during screening for ILD in high-risk groups (e.g. those with rheumatoid arthritis, systemic sclerosis or familial ILD) are not considered as ILA because they are not incidental.
