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Semin Respir Crit Care Med 2016; 37(03): 468-476
DOI: 10.1055/s-0036-1580689
Thieme Medical Publishers 333 Seventh Avenue, New York, NY 10001, USA.

Connective Tissue Disease-Associated Interstitial Lung Diseases: Unresolved Issues

Irene Jarana Aparicio
1   Department of Respiratory Medicine, Gregorio Marañon Hospital, Madrid, Spain
,
Joyce S. Lee
2   Division of Pulmonary Sciences and Critical Care Medicine, Department of Medicine, University of Colorado Denver, Aurora, Colorado
› Author Affiliations
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Publication History

Publication Date:
27 May 2016 (online)

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Abstract

Interstitial lung disease (ILD) complicating connective tissue disorders, such as scleroderma and rheumatoid arthritis, is associated with significant morbidity and mortality. Progress has been made in our understanding of these collective diseases; however, there are still many unanswered questions. In this review, we describe the current views on epidemiology, clinical presentation, treatment, and prognosis in patients with connective tissue disease (CTD)-associated ILD. We also highlight several areas that remain unresolved and in need of further investigation, including interstitial pneumonia with autoimmune features, histopathologic phenotype, and pharmacologic management. A multidisciplinary and multidimensional approach to diagnosis, management, and investigation of CTD-associated ILD patients is essential to advance our understanding of the epidemiology and pathobiology of this challenging group of diseases.

Keywords

connective tissue disease - interstitial lung disease - autoimmunity - interstitial pneumonia
 

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