Pulmonary fibrosis: patterns and perpetrators.
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.
Duke Scholars
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Related Subject Headings
- Respiratory Mucosa
- Pulmonary Fibrosis
- Pulmonary Alveoli
- Models, Biological
- Mesoderm
- Immunology
- Idiopathic Pulmonary Fibrosis
- Humans
- Genetic Predisposition to Disease
- Animals
Citation
Published In
DOI
EISSN
Publication Date
Volume
Issue
Start / End Page
Location
Related Subject Headings
- Respiratory Mucosa
- Pulmonary Fibrosis
- Pulmonary Alveoli
- Models, Biological
- Mesoderm
- Immunology
- Idiopathic Pulmonary Fibrosis
- Humans
- Genetic Predisposition to Disease
- Animals