Editorial: Interstitial Lung Disease in the Context of Systemic Disease: Pathophysiology, Treatment and Outcomes

摘要

Interstitial lung disease (ILD) is an umbrella term for many different disease entities causing inflammation and fibrosis of the lung parenchyma. These can be broadly divided into five categories based on etiology (1): (1) ILD related to a distinct primary disease (e.g., sarcoidosis), (2) ILD related to environmental factors (e. g., hypersensitivity pneumonitis), (3) ILD induced by drugs or irradiation, (4) idiopathic interstitial pneumonias (e.g., idiopathic pulmonary fibrosis), and (5) ILD related to connective tissue diseases (CTD) (1). While all these entities require thorough and multidisciplinary assessment to ascertain a diagnosis, establish the need for diagnostic procedures, and recommend a patient-specific treatment plan, ILDs associated with systemic diseases are particularly challenging. In many cases, optimal treatment for involvement of other organ systems needs to be balanced with the choice of ILD-directed therapies.