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Highly Accessed Review

Interstitial lung disease in connective tissue diseases: evolving concepts of pathogenesis and management

Flavia V Castelino1* and John Varga2

Author Affiliations

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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Arthritis Research & Therapy 2010, 12:213  doi:10.1186/ar3097

Published: 23 August 2010

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.