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Pulmonary fibrosis: patterns and perpetrators
Paul W. Noble, … , Christina E. Barkauskas, Dianhua Jiang
Paul W. Noble, … , Christina E. Barkauskas, Dianhua Jiang
Published August 1, 2012
Citation Information: J Clin Invest. 2012;122(8):2756-2762. https://doi.org/10.1172/JCI60323.
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Review Series

Pulmonary fibrosis: patterns and perpetrators

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Abstract

Pulmonary fibrosis occurs in a variety of clinical settings, constitutes a major cause of morbidity and mortality, and represents an enormous unmet medical need. However, the disease is heterogeneous, and the failure to accurately discern between forms of fibrosing lung diseases leads to inaccurate treatments. Pulmonary fibrosis occurring in the context of connective tissue diseases is often characterized by a distinct pattern of tissue pathology and may be amenable to immunosuppressive therapies. In contrast, idiopathic pulmonary fibrosis (IPF) is a progressive and lethal form of fibrosing lung disease that is recalcitrant to therapies that target the immune system. Although animal models of fibrosis imperfectly recapitulate IPF, they have yielded numerous targets for therapeutic intervention. Understanding the heterogeneity of these diseases and elucidating the final common pathways of fibrogenesis are critical for the development of efficacious therapies for severe fibrosing lung diseases.

Authors

Paul W. Noble, Christina E. Barkauskas, Dianhua Jiang

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Figure 1

Fibrotic NSIP.

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Fibrotic NSIP.
(A) Chest CAT scan from a 60-year-old woman with 6 months...
(A) Chest CAT scan from a 60-year-old woman with 6 months of cough, exertional breathlessness, and fatigue. (B) Surgical lung biopsy shows a homogenous pattern of fibrosis and chronic inflammation with a paucity of normal lung. Magnification, ×40. (C) Chest CAT after treatment with a combination of prednisone and mycophenolate mofetil.

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