Abstract
Interstitial lung disease (ILD) is a challenging clinical entity associated with multiple connective tissue diseases, and is a significant cause of morbidity and mortality. Effective therapies for connective tissue disease-associated interstitial lung disease (CTD-ILD) are still lacking. Multidisciplinary clinics dedicated to the early diagnosis and improved management of patients with CTD-ILD are now being established. There is rapid progress in understanding and identifying the effector cells, the proinflammatory and profibrotic mediators, and the pathways involved in the pathogenesis of CTD-ILD. Serum biomarkers may provide new insights as risk factors for pulmonary fibrosis and as measures of disease progression. Despite these recent advances, the management of patients with CTD-ILD remains suboptimal. Further studies are therefore urgently needed to better understand these conditions, and to develop effective therapeutic interventions.
Highlights
The term interstitial lung disease (ILD) is used to describe a heterogeneous group of parenchymal lung disorders that share common radiologic, pathologic, and clinical manifestations
ILD is subdivided into idiopathic interstitial pneumonia, of which idiopathic pulmonary fibrosis (IPF) is
Multidisciplinary combined connective tissue diseaseassociated interstitial lung disease (CTD-ILD) clinics with rheumatologists and respiratory specialists are being established at many academic medical centers
Summary
The term interstitial lung disease (ILD) is used to describe a heterogeneous group of parenchymal lung disorders that share common radiologic, pathologic, and clinical manifestations. ILD is subdivided into idiopathic interstitial pneumonia, of which idiopathic pulmonary fibrosis (IPF) is. Multidisciplinary combined connective tissue diseaseassociated interstitial lung disease (CTD-ILD) clinics with rheumatologists and respiratory specialists are being established at many academic medical centers. The underlying pathology in CTD-ILD is dominated by inflammation or fibrosis, or a combination of both with distinct radiologic and histopathologic patterns. These patterns are nonspecific interstitial pneumonia (NSIP), usual interstitial pneumonia (UIP), desquamative interstitial pneumonia, cryptogenic organizing pneumonia, diffuse alveolar damage, acute interstitial pneumonia,
Sign-in/Register to view highlights & summary Sign-in/Register to access full text options 
Talk to us
Join us for a 30 min session where you can share your feedback and ask us any queries you have
Schedule a callDisclaimer: All third-party content on this website/platform is and will remain the property of their respective owners and is provided on "as is" basis without any warranties, express or implied. Use of third-party content does not indicate any affiliation, sponsorship with or endorsement by them. Any references to third-party content is to identify the corresponding services and shall be considered fair use under The CopyrightLaw.
