IPF is characterized as a progressive, highly life-threatening interstitial lung disease with unknown etiology. The median survival of patients with IPF is only 2 to 3 years. It occurs primarily in elders, limited to the lungs, and is defined by the histopathologic and/or radiologic pattern of UIP. It should be considered in all adult patients with unexplained chronic exertional dyspnea, cough, bibasilar inspiratory crackles, and/or digital clubbing that occur without constitutional or other symptoms that suggest a multisystem disease. Patients with IPF may present with an acute exacerbation as an initial manifestation. The disease course is unpredictable and various, so early IPF detection and immediate treatment are crucial to the preservation of patients’ lung function.
Beyond IPF, the presentation, diagnosis, and clinical course of Progressive-Fibrosing ILD (PF-ILD) have shown the similarity with IPF. This approach entails evaluation of image quality, precise assessment of specific disease features by use of standard terminology, and the determination of distribution and extent. High-quality CT images are essential. Optimal quality CT requires thin sections (<2 mm) and high spatial resolution reconstruction. Images should be obtained at full inspiration to total lung capacity. Inadequate inspiration increases lung attenuation, potentially leading to misinterpretation of key findings (eg, ground glass opacity and fine reticulation). Volumetric CT acquisition is preferred to non-contiguous imaging because it improves the characterization of patchy disease and delineation of disease extent, clarifies disease distribution, allows identification of ancillary findings, facilitates differentiation between honeycombing and traction bronchiectasis, and optimizes comparison with follow-up images to assess progression or improvement.
PF-ILD usually is associated with autoimmune disease (e.g., SSc-ILD, RA-ILD, DM-ILD, Sjogren syndrome associated ILD), fibrotic chronic hypersensitivity pneumonitis, sarcoidosis or related to occupational environment exposure. In this meeting, we will invite domestic and foreign ILD experts to present the latest IPF/PF-ILD clinical treatment information and differential diagnosis for ILD via multiple disciplinary discussions.