Interstitial lung disease in connective tissue diseases: evolving concepts of pathogenesis and management |
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Authors: | Flavia V Castelino John Varga |
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Institution: | (1) Division of Rheumatology, Bulfinch-165, Massachusetts General Hospital, Harvard Medical School, 55 Fruit St, Boston, MA 02114, USA;(2) Division of Rheumatology, Northwestern University Feinberg School of Medicine, McGaw 2300, 240 East Huron Street, Chicago, IL 60611, USA |
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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. |
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