Interstitial Lung Diseases

Interstitial lung diseases (ILDs) comprise a heterogeneous group of complex chronic lung diseases. Patients with ILD present with progressive dyspnea, cough, and impaired pulmonary function resulting in quality of life issues. Mortality in patients with ILD can be as high as in some types of cancer. Patients with idiopathic pulmonary fibrosis (IPF), the most common of the idiopathic ILDs, have a median life expectancy of only 3–5 years from the time of diagnosis. Various ILD subtypes show overlap in pathophysiology and imaging appearance, which can confound ILD classification. Many experts conditionally agree that the reference standard for diagnostic decision-making and diagnosis of ILDs is through a multidisciplinary discussion including pulmonologist, radiologist, and pathologist. Multidisciplinary discussion of suspected IPF cases results in better interobserver agreement, better congruence, and higher confidence in the final diagnoses, and in certain cases, changes the impression to a non-IPF diagnosis. Multidisciplinary discussion also aids in making a decision to pursue invasive diagnostic procedures such as transbronchial biopsy, bronchoalveolar lavage, and surgical lung biopsy, especially when the risks of such procedures are high because of disease severity, advanced age, and comorbidities.