Interstitial lung disease in connective tissue diseases: evolving concepts of pathogenesis and management.
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.
Castelino
Flavia V
FV
Division of Rheumatology, Massachusetts General Hospital, Harvard Medical School, Boston, MA 02114, USA. fcastelino@partners.org
Varga
John
J
D015415:Biomarkers; D003240:Connective Tissue Diseases / Q000209:etiology / Q000401:mortality / Q000628:therapy; D006801:Humans; D017563:Lung Diseases, Interstitial / Q000209:etiology / Q000401:mortality / Q000628:therapy; D009017:Morbidity; D012307:Risk Factors
D015415:Biomarkers