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

Frequent internal organ complications characterize autoimmune diseases (ADs), such as rheumatoid arthritis, systemic sclerosis or inflammatory myopathies. Of these, interstitial lung disease (ILD) is the most important one regarding overall mortality. Currently, however, it is not known which patients will develop ILD in the context of their autoimmune disease, an association to the presence of specific antibodies, such as anti-CCP, anti-Scl70 or anti-tRNA-Synthetase antibodies has been described. The underlying pathophysiologic mechanisms are not fully understood as of yet. While various treatments, such as immunosuppressive or anti-fibrotic therapies, have been proposed for ILD, these are of limited or uncertain efficacy in patients with systemic disease. Since mortality from ILD is high, interest in clinical applications and clinical trials has been increasing, and it is an area of active research. Much has to be learned from different cohorts and phenotypes to stratify patients correctly and improve treatments.

With this research topic, we, therefore, aim for an improved understanding of the pathophysiology of ILD in various autoimmune diseases and are interested in the outcome of different treatment modalities. We welcome authors from different subspecialties, such as pulmonary medicine, rheumatology, neurology, or others to contribute their original clinical or experimental research data, review articles or exceptional case reports that shed light on the pathophysiology, diagnosis or treatment on ILD in autoimmune diseases with the ultimate goal of improving the care of affected patients.